Literature Review Of Management Of Pineal Region Tumour

Continuous Medical Continuous Medical EducationEducation

Department of Neurosurgery, HKL

8 November 2006

OVERVIEWOVERVIEW

Pineal region tumors are derived from Pineal region tumors are derived from cells located in and around the pineal cells located in and around the pineal gland. gland.

In their 1954 pineal tumor study, Ringertz In their 1954 pineal tumor study, Ringertz and colleagues defined the pineal region and colleagues defined the pineal region as being bound by the splenium of the as being bound by the splenium of the corpus callosum and tela choroidea corpus callosum and tela choroidea dorsally, the quadrigeminal plate and dorsally, the quadrigeminal plate and midbrain tectum ventrally, the posterior midbrain tectum ventrally, the posterior aspect of the third ventricle rostrally, and aspect of the third ventricle rostrally, and the cerebellar vermis caudally. the cerebellar vermis caudally.

OVERVIEWOVERVIEW

Pineal region tumors make up 0.4-1.0% of Pineal region tumors make up 0.4-1.0% of intracranial tumors in adults and 3.0-8.0% of intracranial tumors in adults and 3.0-8.0% of brain tumors in children. brain tumors in children.

Most children are aged 10-20 years at Most children are aged 10-20 years at presentation, with the average age at presentation, with the average age at presentation being 13 years. presentation being 13 years.

Adults typically are older than 30 years at Adults typically are older than 30 years at presentation. presentation.

A complete differential diagnosis for masses A complete differential diagnosis for masses in the pineal region also should include in the pineal region also should include vascular anomalies, as well as metastatic vascular anomalies, as well as metastatic tumor. tumor.

OVERVIEWOVERVIEW

Tumors of the pineal region have a varied Tumors of the pineal region have a varied histology that generally can be divided into histology that generally can be divided into germ cellgerm cell and and non–germ cell derivativesnon–germ cell derivatives. .

Most tumors are a result of displaced Most tumors are a result of displaced embryonic tissue, malignant transformation embryonic tissue, malignant transformation of of pineal parenchymal cellspineal parenchymal cells, or , or transformation of surrounding transformation of surrounding astrogliaastroglia. .

No specific genetic mutations have been No specific genetic mutations have been associated with sporadic pineal region associated with sporadic pineal region tumors. tumors.

Revolution in Pineal Revolution in Pineal SurgerySurgery

In the early part of this century pineal In the early part of this century pineal region surgery had poor outcomes, with region surgery had poor outcomes, with operative mortality rates approaching 90%. operative mortality rates approaching 90%.

From Horsley's initial attempt at removing a From Horsley's initial attempt at removing a pineal mass in 1910 and development of pineal mass in 1910 and development of the lateral transventricular approach in the lateral transventricular approach in 1931 by Van Wagenan, primitive anesthetic 1931 by Van Wagenan, primitive anesthetic technique and the lack of an operating technique and the lack of an operating microscope hindered pineal region surgery. microscope hindered pineal region surgery.


Torkildsen in 1948 abandoning aggressive Torkildsen in 1948 abandoning aggressive surgical resection in favor of cerebrospinal surgical resection in favor of cerebrospinal fluid (CSF) diversion followed by empiric fluid (CSF) diversion followed by empiric radiotherapy. radiotherapy.

If the patient did not respond to radiation, a If the patient did not respond to radiation, a surgical procedure to remove radioresistant surgical procedure to remove radioresistant tumor was performed. tumor was performed.

The algorithm of CSF diversion, radiation, The algorithm of CSF diversion, radiation, and observation sometimes was successful; and observation sometimes was successful; however, patients with however, patients with benign lesions were benign lesions were exposed to unnecessary and ineffective exposed to unnecessary and ineffective radiationradiation. .


Modification of this treatment strategy led to the Modification of this treatment strategy led to the radiation test by Japanese clinicians whose radiation test by Japanese clinicians whose patient population had high percentage of patient population had high percentage of radiosensitive germinomas. radiosensitive germinomas.

According to this protocol, patients were According to this protocol, patients were administered with small doses of radiation, and administered with small doses of radiation, and their cases were followed radiologically. their cases were followed radiologically.

If the pineal tumor decreased in size, it was If the pineal tumor decreased in size, it was presumed to be radiosensitive, and a full course presumed to be radiosensitive, and a full course of radiation was instituted. of radiation was instituted.

Patients not responding to radiotherapy Patients not responding to radiotherapy underwent surgical exploration. underwent surgical exploration.

Despite the low dose of radiation initially used, Despite the low dose of radiation initially used, significant long-term morbiditysignificant long-term morbidity remained remained associated with this strategy, particularly in associated with this strategy, particularly in children. children.


The advance of microsurgical The advance of microsurgical techniques and stereotactic procedures techniques and stereotactic procedures has change the practice of empiric has change the practice of empiric radiotherapy without tissue diagnosis. radiotherapy without tissue diagnosis.

Therapeutic decision-making now is Therapeutic decision-making now is based on based on tumor histologytumor histology rather than rather than radiation responsiveness. radiation responsiveness.

Currently, initial surgical management Currently, initial surgical management for tissue diagnosis, and for tissue diagnosis, and possible possible resectionresection, is the standard of care for , is the standard of care for most children with pineal region tumors most children with pineal region tumors

Pineal Tumour – Initial Pineal Tumour – Initial ManagementManagement

Initial management of patients with pineal region Initial management of patients with pineal region tumors should be directed at treating tumors should be directed at treating hydrocephalus hydrocephalus and establishing a diagnosis. and establishing a diagnosis.

Preoperative evaluation should include Preoperative evaluation should include (1)(1) high-resolution MRIhigh-resolution MRI of the head with gadolinium; of the head with gadolinium; (2)(2) measurement of measurement of serum and CSF markersserum and CSF markers, if available; , if available; (3)(3) cytologic examination of CSFcytologic examination of CSF, if available; , if available; (4)(4) evaluation of evaluation of pituitary functionpituitary function if endocrine if endocrine

abnormalities are suspected; and abnormalities are suspected; and (5)(5) visual field examinationvisual field examination if suprasellar extension of the if suprasellar extension of the

tumor is noted on MRI. tumor is noted on MRI. The ultimate management goal should be to The ultimate management goal should be to refine refine

adjuvant therapy based on tumor pathology. adjuvant therapy based on tumor pathology.

Indication for SurgeryIndication for Surgery

Indications for neurosurgical intervention Indications for neurosurgical intervention relate to the severity and chronicity of relate to the severity and chronicity of clinical presentation. clinical presentation.

The symptoms of pineal region tumors can The symptoms of pineal region tumors can be as varied as their diverse histology. be as varied as their diverse histology.

Patients presenting with signs and symptoms Patients presenting with signs and symptoms of raised intracranial pressure must receive a of raised intracranial pressure must receive a head CT scan or an MRI to assess the need head CT scan or an MRI to assess the need for emergent management. for emergent management.

Subsequent nonemergent workup of a Subsequent nonemergent workup of a patient with a pineal region tumor can be patient with a pineal region tumor can be divided into divided into radiologic and laboratory radiologic and laboratory studies. studies.

Lab StudiesLab Studies

Measurements of Measurements of serum and CSF tumor serum and CSF tumor markersmarkers are a valuable component of the are a valuable component of the preoperative evaluation. preoperative evaluation.

These results can be suggestive of tumor These results can be suggestive of tumor type but only occasionally provide the type but only occasionally provide the physician diagnostic information.physician diagnostic information.

Markers have been most helpful in the Markers have been most helpful in the workup of patients with workup of patients with germ cell tumorsgerm cell tumors. .

The expression of embryonic proteins, such The expression of embryonic proteins, such as alpha-fetoprotein (AFP) and bhCG, are as alpha-fetoprotein (AFP) and bhCG, are indicative of malignant germ cell elements. indicative of malignant germ cell elements.


Other biological markers for germ cell Other biological markers for germ cell tumors include lactate dehydrogenase tumors include lactate dehydrogenase isoenzymes and placental alkaline isoenzymes and placental alkaline phosphatase, although these are less phosphatase, although these are less specific. specific.

Serum and CSF measurements can be Serum and CSF measurements can be used for used for diagnosticdiagnostic purposes and for purposes and for monitoring a responsemonitoring a response to therapy. to therapy.

In general, In general, CSFCSF measurements are measurements are more sensitivemore sensitive than serum than serum measurements, and a CSF-to-serum measurements, and a CSF-to-serum gradient may be consistent with an gradient may be consistent with an intracranial lesion. intracranial lesion.


AFPAFP is a glycoprotein produced by fetal yolk is a glycoprotein produced by fetal yolk sac elements and is produced by a wide sac elements and is produced by a wide range of cancers, including gastric, liver, and range of cancers, including gastric, liver, and colon adenocarcinoma, as well as colon adenocarcinoma, as well as extracranial germ cell tumors. extracranial germ cell tumors.

Serum levels of AFP are greatest in newborns Serum levels of AFP are greatest in newborns and decline thereafter. and decline thereafter.

AFP is markedly elevated with endodermal AFP is markedly elevated with endodermal sinus tumors and elevated to a lesser degree sinus tumors and elevated to a lesser degree with embryonal cell carcinomas. with embryonal cell carcinomas.

While teratomas do not secrete AFP, the less-While teratomas do not secrete AFP, the less-differentiated immature teratomas can differentiated immature teratomas can produce detectable amounts produce detectable amounts


BhCGBhCG is a glycoprotein with a half-life of is a glycoprotein with a half-life of 15-20 hours and usually is produced by 15-20 hours and usually is produced by placental trophoblastic cells. placental trophoblastic cells.

Choriocarcinomas secrete large amounts Choriocarcinomas secrete large amounts of bhCG, and lesser elevations can occur of bhCG, and lesser elevations can occur in patients with embryonal cell in patients with embryonal cell carcinomas. carcinomas.

The presence of syncytiotrophoblastic The presence of syncytiotrophoblastic giant cells in mixed germinomas may giant cells in mixed germinomas may result in detectable levels of bhCG, but the result in detectable levels of bhCG, but the majority of germinomas are nonsecretory. majority of germinomas are nonsecretory.


Significant variability in expression of tumor Significant variability in expression of tumor markers is such that the markers is such that the absence of AFP or bhCG absence of AFP or bhCG does not rule out a mixed germ cell tumordoes not rule out a mixed germ cell tumor. .

Although recent studies suggest a less favorable Although recent studies suggest a less favorable prognosis for patients with germinomas secreting prognosis for patients with germinomas secreting bhCG, bhCG, nono established prognostic significance of established prognostic significance of tumor markerstumor markers exists. exists.

Determination of Determination of AFP and bhCG levels priorAFP and bhCG levels prior to to surgical resection is extremely important surgical resection is extremely important because it provides a because it provides a reference pointreference point that can be that can be used to assess recurrence during follow-up. used to assess recurrence during follow-up.


Pineal parenchymal cell tumorPineal parenchymal cell tumor markers markers are less well characterized than their are less well characterized than their germ cell counterparts and include germ cell counterparts and include melatoninmelatonin and the and the S antigenS antigen. .

Neither of these proteins has proven Neither of these proteins has proven valuable in the diagnosis of pineal valuable in the diagnosis of pineal parenchymal cell tumors. parenchymal cell tumors.

Some authors have reported using Some authors have reported using melatonin levels in follow-up for patients melatonin levels in follow-up for patients with pineocytoma after surgical with pineocytoma after surgical treatment. treatment.

Intracranial Germ Cell TumorsIntracranial Germ Cell TumorsRoger J. Packer, Bruce H. Cohen and Roger J. Packer, Bruce H. Cohen and Kathleen CooneyKathleen CooneyOncologist Oncologist 2000;5;312-3202000;5;312-320

Tumor markers have been used to diagnose Tumor markers have been used to diagnose the specific type of tumor present. the specific type of tumor present.

When these markers are elevated, especially When these markers are elevated, especially at high elevations, the diagnosis of a form of at high elevations, the diagnosis of a form of mixed germ cell tumormixed germ cell tumor is essentially confirmed. is essentially confirmed.

Similarly, Similarly, isolatedisolated high elevations of high elevations of β-HCGβ-HCG strongly suggest the presence of a strongly suggest the presence of a choriocarcinomachoriocarcinoma. .

Milder elevations of β-HCG are of less use, as Milder elevations of β-HCG are of less use, as they may be representative of a mixed germ they may be representative of a mixed germ cell tumor, choriocarcinoma, or possibly other cell tumor, choriocarcinoma, or possibly other forms of malignancy in the pineal region.forms of malignancy in the pineal region.

Imaging StudiesImaging Studies

High-resolution MRI with gadoliniumHigh-resolution MRI with gadolinium is necessary is necessary in the evaluation of pineal region lesions. in the evaluation of pineal region lesions.

Tumor characteristics, such as size, vascularity, Tumor characteristics, such as size, vascularity, and homogeneity, can be assessed, as well as the and homogeneity, can be assessed, as well as the anatomic relationship with surrounding structures. anatomic relationship with surrounding structures.

Irregular tumor bordersIrregular tumor borders can be suggestive of can be suggestive of tumor tumor invasiveness and associated histologic invasiveness and associated histologic malignancymalignancy. .

Although the type of tumor cannot be determined Although the type of tumor cannot be determined reliably from the radiographic characteristics reliably from the radiographic characteristics alone, some patterns are associated with specific alone, some patterns are associated with specific tumors. tumors.


Non–germ cell tumorsNon–germ cell tumors can derive from pineal can derive from pineal parenchymal cells, as well as from parenchymal cells, as well as from surrounding tissue. surrounding tissue.

PineocytomasPineocytomas and and pineoblastomaspineoblastomas typically typically are hypointense to isointense on T1-weighted are hypointense to isointense on T1-weighted images, have increased signal on T2, and images, have increased signal on T2, and demonstrate homogenous enhancement demonstrate homogenous enhancement after administration of gadolinium. after administration of gadolinium.

Pineoblastomas can be distinguished by their Pineoblastomas can be distinguished by their irregular shape and large size (ie, some >4.0 irregular shape and large size (ie, some >4.0 cm). cm).


AstrocytomasAstrocytomas, which can arise from , which can arise from the glial stroma of the pineal gland or the glial stroma of the pineal gland or surrounding tissue, also are surrounding tissue, also are hypointense on T1 and hyperintense hypointense on T1 and hyperintense on T2. on T2.

However, astrocytomas have variable However, astrocytomas have variable enhancement patterns. enhancement patterns.

Calcium may be present in either Calcium may be present in either pineal cell tumors or astrocytomas. pineal cell tumors or astrocytomas.


MeningiomasMeningiomas typically enhance typically enhance homogenously and have smooth, homogenously and have smooth, distinct borders. distinct borders.

Tentorial meningiomas can have Tentorial meningiomas can have an enhancing an enhancing dural taildural tail of origin of origin and are anatomically distinguished and are anatomically distinguished by their dorsal location relative to by their dorsal location relative to the deep venous system. the deep venous system.


GerminomasGerminomas are isointense on T1-weighted are isointense on T1-weighted MRI studies, are slightly hyperintense on T2, MRI studies, are slightly hyperintense on T2, and have strong homogenous enhancement. and have strong homogenous enhancement. Germinomas can have evidence of Germinomas can have evidence of calcification, which surrounds the pineal calcification, which surrounds the pineal gland as the germinoma grows. gland as the germinoma grows.

In contrast, In contrast, pineocytomaspineocytomas commonly have commonly have intratumoral calcium. Intratumoral cysts can intratumoral calcium. Intratumoral cysts can exist as well. exist as well.

Unlike germinomas, Unlike germinomas, teratomasteratomas typically have typically have heterogenous MRI signals because they can heterogenous MRI signals because they can contain tissue from all 3 germinal layers. contain tissue from all 3 germinal layers.


Teratomas are well-circumscribed benign tumors Teratomas are well-circumscribed benign tumors characterized by their heterogeneity, multilocularly, characterized by their heterogeneity, multilocularly, and irregular enhancement. and irregular enhancement.

These tumors also can demonstrate These tumors also can demonstrate ring ring enhancementenhancement. In some cases, a well-circumscribed . In some cases, a well-circumscribed teratoma has areas of low attenuation that correlate teratoma has areas of low attenuation that correlate with adipose tissue, which serves to further with adipose tissue, which serves to further distinguish it from other pineal region tumors. distinguish it from other pineal region tumors.

Malignant nongerminomatous germ cell tumorsMalignant nongerminomatous germ cell tumors also also can have a heterogeneous appearance due to a can have a heterogeneous appearance due to a mixture of benign and malignant germ cell mixture of benign and malignant germ cell components. components.

Areas of Areas of intratumoral hemorrhageintratumoral hemorrhage may distinguish may distinguish specific subtypes, such as specific subtypes, such as choriocarcinoma. choriocarcinoma.


The neuroimaging characteristics of germinomas and The neuroimaging characteristics of germinomas and nongerminomatous germ cell tumors are nongerminomatous germ cell tumors are similarsimilar enough to limit diagnostic certainty, and either enough to limit diagnostic certainty, and either tissue tissue confirmation or the measurement of specific tumor confirmation or the measurement of specific tumor markersmarkers are needed for diagnosis are needed for diagnosis

In addition, in the pineal region, germ cell tumors In addition, in the pineal region, germ cell tumors cannot be definitively separated on basis of cannot be definitively separated on basis of neuroimaging characteristics from other tumors, such neuroimaging characteristics from other tumors, such as pineoblastomas, pineocytomas, or gliomas. as pineoblastomas, pineocytomas, or gliomas.

In the suprasellar region, germinomas may be difficult In the suprasellar region, germinomas may be difficult to separate from other lesions, including germinomas to separate from other lesions, including germinomas which infiltrate the surrounding brain mimicking which infiltrate the surrounding brain mimicking gliomas and histiocytomas. gliomas and histiocytomas.

In general, mixed germ cell tumors tend to be more In general, mixed germ cell tumors tend to be more invasive than pure germinomas.invasive than pure germinomas.

Pineal teratomas can often be differentiated based on Pineal teratomas can often be differentiated based on more frequent areas of fat and large areas of more frequent areas of fat and large areas of calcification.calcification.


In addition to MRI, angiography In addition to MRI, angiography sometimes is used in cases of sometimes is used in cases of suspected suspected vascular anomaliesvascular anomalies. .

However, the anatomic and vascular However, the anatomic and vascular information provided by MRI has information provided by MRI has largely circumvented the need for largely circumvented the need for routine angiograms in the evaluation routine angiograms in the evaluation of pineal region neoplasms. of pineal region neoplasms.

Histologic FindingsHistologic Findings

Germ cellGerm cell tumors are the tumors are the most prevalentmost prevalent neoplasms of the pineal region in neoplasms of the pineal region in childrenchildren and are and are histologically indistinguishablehistologically indistinguishable from those found at from those found at extracranial locationsextracranial locations, , including the mediastinum and gonads. including the mediastinum and gonads.

These extracranial locations most These extracranial locations most commonly are midline. commonly are midline.

Intracranial germ cell tumors commonly Intracranial germ cell tumors commonly are divided into 2 categories: germinomas are divided into 2 categories: germinomas and tumors derived from totipotential and tumors derived from totipotential germ cells. germ cells.


Germinomas make up 60-70% of all pediatric Germinomas make up 60-70% of all pediatric germ cell tumors. germ cell tumors.

Nongerminomatous germ cell tumors fall Nongerminomatous germ cell tumors fall along a spectrum of differentiation. along a spectrum of differentiation.

The least differentiated is the The least differentiated is the embryonal cell embryonal cell carcinomacarcinoma, with further differentiation , with further differentiation described as either embryonic or described as either embryonic or extraembryonic. extraembryonic.

Immature and mature teratomasImmature and mature teratomas result from result from maturation along embryonic cell lines, maturation along embryonic cell lines, whereas the whereas the endodermal sinus tumor or yolk endodermal sinus tumor or yolk sacsac tumor and the tumor and the choriocarcinomachoriocarcinoma are a are a result of extraembryonic differentiation result of extraembryonic differentiation


Description and classification of a given Description and classification of a given lesion sometimes is confounded when lesion sometimes is confounded when more more than one typethan one type of germ cell component is of germ cell component is found in a surgical specimen. found in a surgical specimen.

Mixed germ cell tumorsMixed germ cell tumors are a result of are a result of simultaneous differentiation along more than simultaneous differentiation along more than one pathway such that, at presentation, 2 or one pathway such that, at presentation, 2 or more characterized components are more characterized components are recognized. recognized.

An example of this is the An example of this is the teratocarcinomateratocarcinoma, an , an embryonal carcinoma containing elements of embryonal carcinoma containing elements of an immature teratoma. an immature teratoma.

Histologic FindingsHistologic Findings The pineoblastoma is an aggressive The pineoblastoma is an aggressive

tumor and resembles the tumor and resembles the medulloblastoma with respect to age medulloblastoma with respect to age of presentation and its propensity to of presentation and its propensity to seed the subarachnoid space. seed the subarachnoid space.

The The pineocytomapineocytoma is significantly less is significantly less aggressive than the pineoblastoma. aggressive than the pineoblastoma.

It usually presents during adolescence It usually presents during adolescence and rarely seeds the subarachnoid and rarely seeds the subarachnoid space.space.

Histologic FindingsHistologic Findings Neoplasms derived from Neoplasms derived from glial cellsglial cells occur occur

second most frequently in children with second most frequently in children with pineal region tumors, exceeded only by pineal region tumors, exceeded only by germinomas in 2 large series that include germinomas in 2 large series that include children with pineal region tumors. children with pineal region tumors.

The histologic and macroscopic The histologic and macroscopic appearances are similar to malignant glial appearances are similar to malignant glial neoplasms found in other areas of the CNS.neoplasms found in other areas of the CNS.

Glial-derived neoplasms in the pineal Glial-derived neoplasms in the pineal region can include low-grade and high-region can include low-grade and high-grade lesions. grade lesions.

StagingStaging

Currently, no formal staging Currently, no formal staging designation is available for patients designation is available for patients with pineal region tumors. with pineal region tumors.

However, a postoperative MRI within 48 However, a postoperative MRI within 48 hours of surgery is an important means hours of surgery is an important means of assessing the degree of resection. of assessing the degree of resection.

In addition, surveillance scans of the In addition, surveillance scans of the spine are used to assess the presence spine are used to assess the presence of drop metastasis. of drop metastasis.

Medical therapy:Medical therapy:Radiation therapyRadiation therapy

Current treatment protocols for patients Current treatment protocols for patients older than 3 years who have malignant older than 3 years who have malignant pineal region tumors include pineal region tumors include radiotherapyradiotherapy. .

Early Early clinical trialsclinical trials of patients treated with of patients treated with radiotherapy reported radiotherapy reported significant mortalitysignificant mortality. .

Even low doses of radiation can have Even low doses of radiation can have significant long-term effects upon a significant long-term effects upon a child's child's cognitive developmentcognitive development. .

Radiation-induced deficits are an important Radiation-induced deficits are an important consideration because many children with consideration because many children with pineal region tumors enjoy prolonged pineal region tumors enjoy prolonged survival. survival.


Potential complications include Potential complications include hypothalamic and hypothalamic and endocrine dysfunctionendocrine dysfunction, cerebral necrosis, , cerebral necrosis, secondary tumorigenesis, and progression of secondary tumorigenesis, and progression of disease. disease.

Cases of radiation-induced meningioma have Cases of radiation-induced meningioma have been reported in children after radiotherapy for been reported in children after radiotherapy for pineal region tumors. pineal region tumors.

Standard radiotherapy protocols for children with Standard radiotherapy protocols for children with malignant pineal cell tumors use 4000 cGy of malignant pineal cell tumors use 4000 cGy of whole brain radiation followed by 1500 cGy to the whole brain radiation followed by 1500 cGy to the pineal region. pineal region.

The dose is administered in 180-cGy daily The dose is administered in 180-cGy daily fractions. fractions.


Whole brain radiationWhole brain radiation can cause significant can cause significant morbiditymorbidity in prepubescent patients, limiting in prepubescent patients, limiting the recommended initial extended field to the recommended initial extended field to 2500-3000 cGy. 2500-3000 cGy.

An additional dose directed at the tumor bed An additional dose directed at the tumor bed can be administered subsequently. can be administered subsequently.

Several studies demonstrate that patients Several studies demonstrate that patients receiving less than 5000 cGy are at receiving less than 5000 cGy are at risk for risk for recurrencerecurrence, strongly suggesting that this is the , strongly suggesting that this is the optimal total dose of radiation. optimal total dose of radiation.

For children with For children with malignant germ cellmalignant germ cell tumors, tumors, standard treatment is standard treatment is focal radiotherapyfocal radiotherapy followed by radiation to the followed by radiation to the ventricular fieldventricular field. .

The application of radiotherapy depends upon The application of radiotherapy depends upon the histology of the tumor being treated. the histology of the tumor being treated.


GerminomasGerminomas are among the are among the most radiosensitivemost radiosensitive tumors, with patient response rates and long-term tumors, with patient response rates and long-term tumor-free survival rates greater than 90% in most tumor-free survival rates greater than 90% in most published series. published series.

Nongerminomatous malignant germ cell tumorsNongerminomatous malignant germ cell tumors are are significantly less responsive to radiation, with a 5-year significantly less responsive to radiation, with a 5-year survival rate of survival rate of 30-40%30-40% using this treatment alone. using this treatment alone.

Patients with Patients with low-grade pineocytomaslow-grade pineocytomas can be can be observed cautiously after complete surgical resection observed cautiously after complete surgical resection without adjuvant radiationwithout adjuvant radiation because no clear evidence because no clear evidence shows that radiotherapy is beneficial. shows that radiotherapy is beneficial.

These patients' cases should be followed carefully These patients' cases should be followed carefully with serial MRIs to assess tumor recurrence or with serial MRIs to assess tumor recurrence or progression. progression.


In the past, a frequent means to determine the In the past, a frequent means to determine the histological type of pineal region tumors, without histological type of pineal region tumors, without surgery, was to diagnose the tumor on the basis of surgery, was to diagnose the tumor on the basis of its its response to radiotherapyresponse to radiotherapy

Patients with Patients with presumed germ cellpresumed germ cell tumors were given tumors were given 2,000 cGy of radiation to the area of abnormality and 2,000 cGy of radiation to the area of abnormality and if the tumor regressed after such treatment, a if the tumor regressed after such treatment, a diagnosis of germinoma was made. diagnosis of germinoma was made.

If there was minimal or no response, biopsy was If there was minimal or no response, biopsy was recommended. recommended.

Although it is true that germinomas will respond to Although it is true that germinomas will respond to radiotherapy, other germ cell tumors will also radiotherapy, other germ cell tumors will also respond, as will the pineoblastomas. respond, as will the pineoblastomas.

Given the differing types of treatment required for Given the differing types of treatment required for germ cell tumors, the use of germ cell tumors, the use of responsiveness to responsiveness to radiotherapy as a diagnostic tool is not appropriateradiotherapy as a diagnostic tool is not appropriate..


The use of The use of prophylactic spinal irradiationprophylactic spinal irradiation is is controversial. controversial.

Early recommendations for postoperative Early recommendations for postoperative spinal irradiation have been preempted by spinal irradiation have been preempted by reports showing the reports showing the incidence of drop incidence of drop metastasis into the spine to be relatively lowmetastasis into the spine to be relatively low. .

The propensity of a pineal region tumor to The propensity of a pineal region tumor to metastasize to the spine varies with tumor metastasize to the spine varies with tumor histology. histology.

Estimates of the incidence of spinal seeding Estimates of the incidence of spinal seeding with pineal cell tumors are in the range of with pineal cell tumors are in the range of 10-10-20%,20%, with significantly higher rates noted for with significantly higher rates noted for pineoblastoma as compared to pineocytoma. pineoblastoma as compared to pineocytoma.


The incidence of spinal metastasis for The incidence of spinal metastasis for germinomasgerminomas has been reported to be as high as 11%, and, for has been reported to be as high as 11%, and, for endodermal sinus tumorsendodermal sinus tumors, incidence is as high as 23%. , incidence is as high as 23%.

Craniospinal radiotherapyCraniospinal radiotherapy for nongerminomatous for nongerminomatous germ cell tumors is controversial but used routinely in germ cell tumors is controversial but used routinely in some countries. some countries.

For patients with For patients with pineoblastomaspineoblastomas, some authors , some authors suggest the use of preemptive suggest the use of preemptive spinal radiation spinal radiation therapytherapy even if the results of the postoperative even if the results of the postoperative surveillance MRI are negative. surveillance MRI are negative.

As modern improvements in surgical and adjuvant As modern improvements in surgical and adjuvant therapy are reflected in long-term survival, the rates therapy are reflected in long-term survival, the rates of spinal metastasis likely will drop significantly, of spinal metastasis likely will drop significantly, making the need for spinal irradiation obsolete. making the need for spinal irradiation obsolete.

Currently, a reasonable approach is to administer Currently, a reasonable approach is to administer spinal irradiation only for documented seeding. spinal irradiation only for documented seeding.

Medical therapy:Medical therapy:ChemotherapyChemotherapy

ChemotherapyChemotherapy has evolved as an attractive means of has evolved as an attractive means of minimizing the amount of radiation needed to minimizing the amount of radiation needed to effectively treat children with pineal region tumors. effectively treat children with pineal region tumors.

As with radiotherapy, the response to chemotherapy As with radiotherapy, the response to chemotherapy for patients with pineal region tumors varies according for patients with pineal region tumors varies according to tumor histology. to tumor histology.

Germ cell tumorsGerm cell tumors historically have been historically have been more more sensitivesensitive to chemotherapy than pineal cell tumors. to chemotherapy than pineal cell tumors.

Germinomas and nongerminomatous germ cell tumors Germinomas and nongerminomatous germ cell tumors have shown response rates ranging from have shown response rates ranging from 80-100% 80-100% with platinum-based regimens. with platinum-based regimens.

Patients with Patients with extracranial nongerminomatousextracranial nongerminomatous germ germ cell tumors respond well to treatment with a wide cell tumors respond well to treatment with a wide array of chemotherapeutic agents. array of chemotherapeutic agents.


Patients with Patients with intracranial nongerminomatous germ cellintracranial nongerminomatous germ cell tumors have demonstrated response rates as high as tumors have demonstrated response rates as high as 78%78% with some regimens. with some regimens.

The The Einhorn regimenEinhorn regimen, which includes cisplatin, vinblastine, , which includes cisplatin, vinblastine, and bleomycin, and later substituted VP-16 for vinblastine and bleomycin, and later substituted VP-16 for vinblastine and bleomycin, has been used with some success. and bleomycin, has been used with some success.

Several ongoing studies are aimed at determining the Several ongoing studies are aimed at determining the optimal sequence of adjuvant therapy for children with optimal sequence of adjuvant therapy for children with nongerminomatous germ cell tumors. nongerminomatous germ cell tumors.

Presently, these children undergo treatment with a course Presently, these children undergo treatment with a course of of chemotherapy prior to radiation.chemotherapy prior to radiation.

The dramatic success of radiotherapy in treating children The dramatic success of radiotherapy in treating children with germinomas has precluded extensive consideration of with germinomas has precluded extensive consideration of chemotherapy as a first-line treatment in older children. chemotherapy as a first-line treatment in older children.

Chemotherapy should be considered a Chemotherapy should be considered a first-line treatment first-line treatment only in very young children. only in very young children.

Some authors advocate treating children with Some authors advocate treating children with chemotherapy prior to radiation in an effort to reduce chemotherapy prior to radiation in an effort to reduce radiation exposure and its associated morbidity. radiation exposure and its associated morbidity.


Most clinicians currently advocate a derivative of the Most clinicians currently advocate a derivative of the Einhorn regimen as an alternative treatment for patients Einhorn regimen as an alternative treatment for patients with with recurrent or metastatic germinomasrecurrent or metastatic germinomas. Some . Some clinicians advocate the use of chemotherapy as well as clinicians advocate the use of chemotherapy as well as radiotherapy after diagnosis of nongerminomatous germ radiotherapy after diagnosis of nongerminomatous germ cell tumors. The impetus for adding chemotherapy cell tumors. The impetus for adding chemotherapy initially in these patients comes from the 5-year survival initially in these patients comes from the 5-year survival rate of 30-65% in children with nongerminomatous germ rate of 30-65% in children with nongerminomatous germ cell tumors treated with radiotherapy alone. cell tumors treated with radiotherapy alone.

The reported effectiveness of chemotherapeutic The reported effectiveness of chemotherapeutic regimens for children with pineal cell tumors is limited to regimens for children with pineal cell tumors is limited to anecdotal case reports and reported series involving anecdotal case reports and reported series involving small numbers of patients. No dominant agent has small numbers of patients. No dominant agent has evolved as the drug of choice, and treatment regimens evolved as the drug of choice, and treatment regimens have included various combinations of vincristine, have included various combinations of vincristine, lomustine, cisplatin, etoposide, cyclophosphamide, lomustine, cisplatin, etoposide, cyclophosphamide, actinomycin D, and methotrexate. actinomycin D, and methotrexate.


Recently, high-dose cyclophosphamide Recently, high-dose cyclophosphamide has been advocated as a has been advocated as a single-agent single-agent protocol in the treatment of children protocol in the treatment of children with pineoblastomaswith pineoblastomas. .

In their 1996 study, Ashley and In their 1996 study, Ashley and colleagues demonstrated that children colleagues demonstrated that children treated with high-dose treated with high-dose cyclophosphamide had stable or cyclophosphamide had stable or diminishing disease while on the diminishing disease while on the protocol. Impaired pulmonary function protocol. Impaired pulmonary function and thrombocytopenia were notable and thrombocytopenia were notable adverse effects. adverse effects.

RadiosurgeryRadiosurgery

Stereotactic radiation or radiosurgeryStereotactic radiation or radiosurgery increasingly is increasingly is being applied to patients with central nervous system being applied to patients with central nervous system disease. disease.

Currently, experience with radiosurgery in patients Currently, experience with radiosurgery in patients with pineal region tumors is limited; however, several with pineal region tumors is limited; however, several small studies have shown small studies have shown safety and some efficacysafety and some efficacy in in treating pineal region tumors over a range of treating pineal region tumors over a range of histologies. histologies.

Review of these studies does show that radiosurgery Review of these studies does show that radiosurgery is not a magic bullet for all pineal lesions with is not a magic bullet for all pineal lesions with treatment failures occurring with more malignant treatment failures occurring with more malignant lesionslesions. .

This demonstrates the importance of obtaining a This demonstrates the importance of obtaining a histological diagnosishistological diagnosis before embarking on a before embarking on a treatment plan and highlights the risk of treating a treatment plan and highlights the risk of treating a tumor of unknown histology with radiosurgery alone. tumor of unknown histology with radiosurgery alone.

Reyns, N., M. Hayashi, et al. (2006). The role of Reyns, N., M. Hayashi, et al. (2006). The role of Gamma Knife radiosurgery in the treatment of Gamma Knife radiosurgery in the treatment of pineal parenchymal tumours. pineal parenchymal tumours. Acta Neurochir Acta Neurochir (Wien)(Wien). . 148: 148: 5-11; discussion 11.5-11; discussion 11.

Retrospectively a series of 13 patients with PPT treated by Gamma Knife Retrospectively a series of 13 patients with PPT treated by Gamma Knife radiosurgery radiosurgery 8 patients had pineocytomas (61.5%)8 patients had pineocytomas (61.5%)5 had pineoblastomas (38.5%). 5 had pineoblastomas (38.5%).

Radiosurgery was performed alone in 6 cases, Radiosurgery was performed alone in 6 cases, after partial microsurgical resection in 3 cases, after partial microsurgical resection in 3 cases, in association with chemotherapy in 3 cases and in association with chemotherapy in 3 cases and following conventional fractionated radiotherapy in 1 case. following conventional fractionated radiotherapy in 1 case.

The marginal dose to these tumors ranged from 11 to 20 Gy (mean 15 Gy). The marginal dose to these tumors ranged from 11 to 20 Gy (mean 15 Gy).

RESULTSRESULTS: With a mean follow-up of 34 months (range 6 to 88), : With a mean follow-up of 34 months (range 6 to 88), all tumors responded to treatment and disappeared or ceased all tumors responded to treatment and disappeared or ceased growinggrowing. . At the end of the follow-up period, 10 out of 12 patients were alive. At the end of the follow-up period, 10 out of 12 patients were alive. No mortality or major morbidity related to radiosurgery. No mortality or major morbidity related to radiosurgery.

CONCLUSIONCONCLUSION: This study confirms that : This study confirms that radiosurgeryradiosurgery can be an can be an effective and effective and safe primary treatment modality for patients with pineocytomassafe primary treatment modality for patients with pineocytomas. . It should have a role in multimodality therapy which includes microsurgical It should have a role in multimodality therapy which includes microsurgical resection, fractionated radiotherapy and chemotherapy for the resection, fractionated radiotherapy and chemotherapy for the management of malignant pineal tumors.management of malignant pineal tumors.

Hasegawa, T., D. Kondziolka, et al. (2002). Hasegawa, T., D. Kondziolka, et al. (2002). "The role of radiosurgery for the "The role of radiosurgery for the treatment of pineal parenchymal tumors." treatment of pineal parenchymal tumors." NeurosurgeryNeurosurgery 5151(4): 880-9.(4): 880-9. Retrospectively evaluated 16 patients who had undergone Retrospectively evaluated 16 patients who had undergone

radiosurgery as the primary or adjuvant treatment for pineal radiosurgery as the primary or adjuvant treatment for pineal parenchymal tumors. parenchymal tumors.

Ten patients (62.5%) had pineocytomas, two (12.5%) had mixed Ten patients (62.5%) had pineocytomas, two (12.5%) had mixed pineocytoma and pineoblastoma, and four (25%) had pineocytoma and pineoblastoma, and four (25%) had pineoblastomas. pineoblastomas.

RESULTSRESULTS: The overall 2- and 5-year survival rates after diagnosis : The overall 2- and 5-year survival rates after diagnosis were 75.0 and 66.7%, respectively. In 14 patients who were were 75.0 and 66.7%, respectively. In 14 patients who were evaluated with imaging, 4 evaluated with imaging, 4 (29%) demonstrated complete remission(29%) demonstrated complete remission, , 8 (57%) had partial remission, 2 (14%) had no change, and no 8 (57%) had partial remission, 2 (14%) had no change, and no patient had local progression. patient had local progression. The local tumor control rateThe local tumor control rate (complete remission, partial remission, or no change) (complete remission, partial remission, or no change) was 100%.was 100%. Five patients died during follow-up. One patient with a pineocytoma Five patients died during follow-up. One patient with a pineocytoma and three patients with pineoblastomas died secondary to and three patients with pineoblastomas died secondary to leptomeningeal or extracranial spread tumor. No cause of death was leptomeningeal or extracranial spread tumor. No cause of death was established for one patient. Two patients developed adverse established for one patient. Two patients developed adverse radiation effects after radiosurgery. radiation effects after radiosurgery.

CONCLUSIONCONCLUSION: : Stereotactic radiosurgeryStereotactic radiosurgery is a valuable is a valuable primary primary management modality for patients with pineocytomasmanagement modality for patients with pineocytomas..– As adjuvant therapyAs adjuvant therapy, radiosurgery may be used to , radiosurgery may be used to boost local tumor doseboost local tumor dose

during multimodality management of malignant pineal parenchymal during multimodality management of malignant pineal parenchymal tumors.tumors.

RadiosurgeryRadiosurgery

In the pediatric population, radiosurgery is In the pediatric population, radiosurgery is an attractive potential an attractive potential first-line treatmentfirst-line treatment that merits further investigation. that merits further investigation.

Some authors have proposed using Some authors have proposed using radiosurgery in place of conventional radiosurgery in place of conventional radiotherapy in an effort to reduce or radiotherapy in an effort to reduce or eliminate the long-term sequelae of eliminate the long-term sequelae of radiotherapy in children. radiotherapy in children.

Radiosurgery is optimized for targets 3 cm Radiosurgery is optimized for targets 3 cm or less, which precludes treatment of or less, which precludes treatment of some patients with larger pineal region some patients with larger pineal region tumors. tumors.

Surgical therapySurgical therapy

The decision to perform a biopsy versus The decision to perform a biopsy versus an open procedure for the pineal region an open procedure for the pineal region tumor has been debated extensively in tumor has been debated extensively in the literature. the literature.

While the ultimate choice of procedure While the ultimate choice of procedure is based to some extent upon the is based to some extent upon the surgeon's personal bias and experience, surgeon's personal bias and experience, some distinct advantages and some distinct advantages and disadvantages exist for each of these disadvantages exist for each of these procedures. procedures.

Jeffrey N. Bruce, Alfred T. Ogden, Surgical Jeffrey N. Bruce, Alfred T. Ogden, Surgical Strategies for Treating Patients with Pineal Strategies for Treating Patients with Pineal Region Tumors, J Neurooncol, 69(1):221-236, Region Tumors, J Neurooncol, 69(1):221-236,

Aug-Sept 2004Aug-Sept 2004 Optimal management of pineal region tumors depends on Optimal management of pineal region tumors depends on

securing an securing an accurate histologic diagnosisaccurate histologic diagnosis to facilitate to facilitate management customized to the nuances of specific management customized to the nuances of specific pathologies. pathologies.

As an initial step, surgical intervention by either As an initial step, surgical intervention by either stereotactic stereotactic biopsy or open surgerybiopsy or open surgery is necessary to obtain tissue for is necessary to obtain tissue for pathologic examination. pathologic examination.

Stereotactic biopsyStereotactic biopsy has the benefit of relative ease and has the benefit of relative ease and minimal morbidity but is associated with greater likelihood of minimal morbidity but is associated with greater likelihood of diagnostic inaccuracy compared to open surgery where more diagnostic inaccuracy compared to open surgery where more extensive tissue sampling is possible.extensive tissue sampling is possible.

The role of The role of surgical debulkingsurgical debulking in the management of pineal in the management of pineal tumors is clearly defined for some tumors but is less evident tumors is clearly defined for some tumors but is less evident for others. for others.

Among the one third of pineal tumors that are Among the one third of pineal tumors that are benign or low benign or low gradegrade, , complete surgical resectioncomplete surgical resection is achievable and is achievable and constitutes optimal management with excellent long-term constitutes optimal management with excellent long-term recurrence-free survival. recurrence-free survival.

Jeffrey N. Bruce, Alfred T. Ogden, Surgical Jeffrey N. Bruce, Alfred T. Ogden, Surgical Strategies for Treating Patients with Pineal Strategies for Treating Patients with Pineal Region Tumors, J Neurooncol, 69(1):221-236, Region Tumors, J Neurooncol, 69(1):221-236,

Aug-Sept 2004Aug-Sept 2004 The benefits of The benefits of aggressive surgical resection among malignant aggressive surgical resection among malignant tumorstumors are less clear but several studies have correlated degree are less clear but several studies have correlated degree of tumor removal with improved outcome.of tumor removal with improved outcome.

Advances in technology, surgical technique, and post-operative Advances in technology, surgical technique, and post-operative care have minimized surgical complications, however all surgical care have minimized surgical complications, however all surgical procedures in the pineal region, including both stereotactic biopsy procedures in the pineal region, including both stereotactic biopsy and open surgery, are potentially hazardous. and open surgery, are potentially hazardous.

Advanced judgment, experience, and expertise are necessary to Advanced judgment, experience, and expertise are necessary to achieve rates of success sufficient to justify aggressive achieve rates of success sufficient to justify aggressive management. management.

Management strategies using stereotactic biopsy, endoscopy, and Management strategies using stereotactic biopsy, endoscopy, and radiosurgery can also provide favorable outcomes in some cases. radiosurgery can also provide favorable outcomes in some cases.

Selective incorporation of these innovations can be expected to Selective incorporation of these innovations can be expected to improve the already highly favorable outcome for all pineal region improve the already highly favorable outcome for all pineal region tumors.tumors.

Stereotactic biopsyStereotactic biopsy

Stereotactic biopsy has been described as the Stereotactic biopsy has been described as the procedure of choiceprocedure of choice for obtaining a tissue for obtaining a tissue diagnosis in certain situations such as widely diagnosis in certain situations such as widely disseminated disease, clearly invasive disseminated disease, clearly invasive malignant tumor, or patients with multiple malignant tumor, or patients with multiple medical problems. medical problems.

Early experience with stereotactic biopsies Early experience with stereotactic biopsies resulted in morbidity and mortality specifically resulted in morbidity and mortality specifically related to targeting periventricular structures related to targeting periventricular structures adjacent to the deep venous system. adjacent to the deep venous system.

More recent studies, however, have shown More recent studies, however, have shown stereotactic biopsy to be a stereotactic biopsy to be a safe and efficientsafe and efficient means of obtaining a tissue diagnosis. means of obtaining a tissue diagnosis.

Stereotactic biopsyStereotactic biopsy

In their 1996 series, Regis and colleagues revealed a In their 1996 series, Regis and colleagues revealed a mortality rate of 1.3% and a morbidity rate of less than mortality rate of 1.3% and a morbidity rate of less than 1.0% in 370 patients with stereotactic biopsies of pineal 1.0% in 370 patients with stereotactic biopsies of pineal region tumors. The study included data from 15 French region tumors. The study included data from 15 French neurosurgical centers and documented statistical neurosurgical centers and documented statistical homogeneity among the different centers. homogeneity among the different centers.

In a similar study, Kreth and colleagues (1996) In a similar study, Kreth and colleagues (1996) retrospectively evaluated the risk profile, diagnostic retrospectively evaluated the risk profile, diagnostic accuracy, and the therapeutic relevance of the accuracy, and the therapeutic relevance of the stereotactic approach in 106 patients. They showed a stereotactic approach in 106 patients. They showed a morbidity rate of morbidity rate of 2 out of 1062 out of 106 patients, a mortality rate patients, a mortality rate of of 9 out of 106 patients9 out of 106 patients, and a definitive tissue , and a definitive tissue diagnostic rate of diagnostic rate of 103 out of 106 patients103 out of 106 patients. While . While stereotactic biopsy clearly can be performed safely and stereotactic biopsy clearly can be performed safely and effectively at centers familiar with the technique, it is effectively at centers familiar with the technique, it is disadvantageous to patients who would benefit from disadvantageous to patients who would benefit from complete or near-complete resection of tumor. complete or near-complete resection of tumor.

(Reyns, Hayashi et al. 2006)Pineal region (Reyns, Hayashi et al. 2006)Pineal region tumors and the role of stereotactic tumors and the role of stereotactic biopsy: review of the mortality, morbidity, biopsy: review of the mortality, morbidity, and diagnostic rates in 370 cases.and diagnostic rates in 370 cases.

[Neurosurgery. 1998][Neurosurgery. 1998] 370 stereotactic biopsies of pineal region 370 stereotactic biopsies of pineal region

tumors were reviewed, from 15 French tumors were reviewed, from 15 French neurosurgical centers. neurosurgical centers.

RESULTSRESULTS: The mortality rate was 1.3% (5 : The mortality rate was 1.3% (5 patients of 370), and 3 patients suffered patients of 370), and 3 patients suffered severe neurological complications. severe neurological complications.

This study is the first to clearly demonstrate This study is the first to clearly demonstrate that the mortality, morbidity, and diagnostic that the mortality, morbidity, and diagnostic rates for stereotactic biopsies are not different rates for stereotactic biopsies are not different in the pineal region. in the pineal region.

CONCLUSIONCONCLUSION: Our conclusion is that : Our conclusion is that stereotactic biopsy must remain a main stereotactic biopsy must remain a main diagnostic modality for tumors of the pineal diagnostic modality for tumors of the pineal region.region.

PopovicPopovic EA EA, , Kelly PJKelly PJ., ., Stereotactic procedures Stereotactic procedures for lesions of the pineal region.for lesions of the pineal region. , Mayo Clin , Mayo Clin

Proc. 1993 Oct;68(10):965-70Proc. 1993 Oct;68(10):965-70 During the 7-year period between June 1985 and May During the 7-year period between June 1985 and May

1992, 34 patients with pineal lesions underwent 66 1992, 34 patients with pineal lesions underwent 66 stereotactic procedures (37 biopsies, 19 third stereotactic procedures (37 biopsies, 19 third ventriculostomies, 6 cyst aspirations, 3 instillations of ventriculostomies, 6 cyst aspirations, 3 instillations of 32P into cysts, and 1 insertion of an Ommaya reservoir 32P into cysts, and 1 insertion of an Ommaya reservoir into a cyst) at the Mayo Clinic. into a cyst) at the Mayo Clinic.

RESULT:RESULT: No mortality or permanent morbidity No mortality or permanent morbidity was was associated with the 66 stereotactic procedures; 2 associated with the 66 stereotactic procedures; 2 patients had temporary complications--1 neurologic patients had temporary complications--1 neurologic (transient diplopia) and 1 nonneurologic (pulmonary (transient diplopia) and 1 nonneurologic (pulmonary embolism). embolism).

Diagnostic tissue was obtained in Diagnostic tissue was obtained in 33 of the 34 patients33 of the 34 patients. . CONCLUSION CONCLUSION :We conclude that :We conclude that stereotactic biopsystereotactic biopsy of of

pineal lesions can be pineal lesions can be performed safelyperformed safely, has a , has a high high diagnostic yielddiagnostic yield, and facilitates rational planning of , and facilitates rational planning of treatment.treatment.

KRETH F. W.; SCHÄTZ C. R., Stereotactic KRETH F. W.; SCHÄTZ C. R., Stereotactic management of lesions of the pineal management of lesions of the pineal region, region, Neurosurgery,1996, vol. 39, no2, pp. 280-Neurosurgery,1996, vol. 39, no2, pp. 280-

291 (53 ref.)291 (53 ref.) OBJECTIVE : The relevance of the OBJECTIVE : The relevance of the computed tomography-guided computed tomography-guided stereotactic approachstereotactic approach for the management of lesions of the pineal region is for the management of lesions of the pineal region is analyzed. analyzed.

METHODS : Retrospective analysis conducted between 1985 and 1993, of METHODS : Retrospective analysis conducted between 1985 and 1993, of the stereotactic approach in 106 patients was studied. the stereotactic approach in 106 patients was studied.

RESULTSRESULTS : A : A histological diagnosishistological diagnosis was obtained in was obtained in 103 of the 106 patients103 of the 106 patients. . – In three patients, a conclusive diagnosis could not be established because of In three patients, a conclusive diagnosis could not be established because of

intraoperative complications. intraoperative complications. – One lesion was misdiagnosed as a pineocytoma instead of a pineoblastoma. One lesion was misdiagnosed as a pineocytoma instead of a pineoblastoma. – Two of the 106 patients died ; Two of the 106 patients died ; – 9 patients experienced perioperative morbidity. 9 patients experienced perioperative morbidity. – In 38 patients, the stereotactic approach was also In 38 patients, the stereotactic approach was also useful for therapyuseful for therapy. . – Cyst aspiration and/or internal drainage was performed in 18 patients with Cyst aspiration and/or internal drainage was performed in 18 patients with

symptomatic cystic lesions. symptomatic cystic lesions. – In 12 patients, the obtained tissue diagnosis was the basis for deferring additional In 12 patients, the obtained tissue diagnosis was the basis for deferring additional

therapy. therapy. – In 43 patients with germ-cell tumors, pineoblastomas, or malignant gliomas, a In 43 patients with germ-cell tumors, pineoblastomas, or malignant gliomas, a

stereotactic biopsy was the starting point for additional stereotactic biopsy was the starting point for additional radiotherapy/chemotherapy. radiotherapy/chemotherapy.

CONCLUSIONCONCLUSION : The : The stereotactic approachstereotactic approach to the pineal region is a relatively to the pineal region is a relatively safesafe procedure in experienced hands. procedure in experienced hands.

The diagnosis obtained by computed tomography-guided stereotactic The diagnosis obtained by computed tomography-guided stereotactic biopsy is a biopsy is a valid basis for treatment decisionsvalid basis for treatment decisions. Long-term follow-up . Long-term follow-up observation of the benign lesions is necessary for a definite confirmation of observation of the benign lesions is necessary for a definite confirmation of diagnostic accuracy. diagnostic accuracy.

Open resectionOpen resection

Open resection carries the obvious advantage of Open resection carries the obvious advantage of complete tumor resection. complete tumor resection.

The long-term benefits of complete tumor resection are The long-term benefits of complete tumor resection are best surmised by tumor type and histology. best surmised by tumor type and histology.

For patients with benign lesions, the surgical resection For patients with benign lesions, the surgical resection can be can be curativecurative. .

In patients with malignant tumor components, evidence In patients with malignant tumor components, evidence suggests that surgical debulking may suggests that surgical debulking may improve the improve the response to postoperative adjuvant therapyresponse to postoperative adjuvant therapy. .

Gross total tumor resection also provides ample tissue Gross total tumor resection also provides ample tissue specimen to the neuropathologist for diagnosis. specimen to the neuropathologist for diagnosis.

This circumvents the potential problems of This circumvents the potential problems of sampling sampling errorerror and erroneous diagnosis associated with the small and erroneous diagnosis associated with the small volume of tissue provided by stereotactic biopsy. volume of tissue provided by stereotactic biopsy.

Mahmoud H. Ragab, M.D., Mark G. Mahmoud H. Ragab, M.D., Mark G. Luciano, M.D., Ph.D., Luciano, M.D., Ph.D., Pineal Region Tumors: Pineal Region Tumors: Endoscopy versus MicrosurgeryEndoscopy versus Microsurgery , , Neurosurgery Online, Volume Neurosurgery Online, Volume

53(2), August 2003, pp 520-52153(2), August 2003, pp 520-521 Between 1995 and 2001, 31 patients underwent surgery for the Between 1995 and 2001, 31 patients underwent surgery for the

management of their pineal region tumors. Twenty-two endoscopic management of their pineal region tumors. Twenty-two endoscopic procedures and nine craniotomies were performed. A combined procedures and nine craniotomies were performed. A combined biopsy and third ventriculostomy was performed in 19 of 22 biopsy and third ventriculostomy was performed in 19 of 22 endoscopic cases with biopsy only in 3 cases.endoscopic cases with biopsy only in 3 cases.

RESULTSRESULTS: Endoscopic treatment was possible in 22 (71%) of 31 of : Endoscopic treatment was possible in 22 (71%) of 31 of presented pineal region tumors. A combined biopsy and third presented pineal region tumors. A combined biopsy and third ventriculostomy was possible in 18 (82%) of the 22 cases. ventriculostomy was possible in 18 (82%) of the 22 cases. – Tumor diagnosis based on endoscopic biopsy was adequate in 18 (82%) Tumor diagnosis based on endoscopic biopsy was adequate in 18 (82%)

of 22 cases. Hydrocephalus was adequately treated in 17 (89%) of 19 of 22 cases. Hydrocephalus was adequately treated in 17 (89%) of 19 cases. cases.

– Craniotomy was required in 9 (29%) of 31 cases secondary to ventricular Craniotomy was required in 9 (29%) of 31 cases secondary to ventricular or tumor anatomy. or tumor anatomy.

– A pathological diagnosis was adequate in 9 (100%) of 9 cases; however, A pathological diagnosis was adequate in 9 (100%) of 9 cases; however, hydrocephalus required further treatment in 3 (33%) of 9 cases, and the hydrocephalus required further treatment in 3 (33%) of 9 cases, and the complication rate (4 [44%] of 9 cases) was higher.complication rate (4 [44%] of 9 cases) was higher.

CONCLUSIONCONCLUSION: A : A combined endoscopic biopsy and third combined endoscopic biopsy and third ventriculostomyventriculostomy was possible and successful in treatment and was possible and successful in treatment and diagnosis of pineal region tumors with low morbidity.diagnosis of pineal region tumors with low morbidity.

Tissue BiopsyTissue Biopsy

Tissue diagnosisTissue diagnosis is a vital part of management is a vital part of management in most patients with pineal region tumors. in most patients with pineal region tumors.

However, nonoperative management of However, nonoperative management of patients with patients with positive tumor markers is a positive tumor markers is a reasonable optionreasonable option for some patients. for some patients.

A markedly elevated level of AFP and bhCG is A markedly elevated level of AFP and bhCG is pathognomonicpathognomonic for germ cell tumors with for germ cell tumors with malignant components. malignant components.

New strategies currently under study have New strategies currently under study have been aimed at minimizing surgical intervention been aimed at minimizing surgical intervention prior to ascertaining whether a tumor is prior to ascertaining whether a tumor is responsive to radiation and/or chemotherapy. responsive to radiation and/or chemotherapy.


In their 1998 retrospective study, Kim and colleagues In their 1998 retrospective study, Kim and colleagues described the treatment of 107 patients with primary described the treatment of 107 patients with primary intracranial germ cell tumors. This included 60 intracranial germ cell tumors. This included 60 patients with tumors in the pineal region. patients with tumors in the pineal region. Thirty of Thirty of these patients were treated without surgery based on these patients were treated without surgery based on radiological findings and tumor markers. radiological findings and tumor markers.

Univariant analysis of a response to Univariant analysis of a response to trial radiation and trial radiation and chemotherapychemotherapy was shown to be correlative with was shown to be correlative with outcome, justifying the administration of trial outcome, justifying the administration of trial chemotherapy or radiotherapy chemotherapy or radiotherapy without tissue biopsywithout tissue biopsy in in the subgroup of patients with positive germ cell the subgroup of patients with positive germ cell markers. These findings corroborate the results of a markers. These findings corroborate the results of a 1997 study by Sawamura and colleagues evaluating 1997 study by Sawamura and colleagues evaluating the necessity of radical resection in patients with the necessity of radical resection in patients with intracranial germinomas. intracranial germinomas.


Twenty-nine patients treated with radiation Twenty-nine patients treated with radiation and/or chemotherapy were studied and/or chemotherapy were studied retrospectively, including 10 with solitary retrospectively, including 10 with solitary pineal region masses. pineal region masses.

The results showed The results showed no significant difference in no significant difference in outcome related to extent of surgical resectionoutcome related to extent of surgical resection and an overall tumor-free survival rate of and an overall tumor-free survival rate of 100% over a follow-up period of 42 months. 100% over a follow-up period of 42 months.

This retrospective evidence is quite compelling This retrospective evidence is quite compelling in favor of withholding surgical treatment of in favor of withholding surgical treatment of children with pineal region tumors and positive children with pineal region tumors and positive serum or CSF markers. serum or CSF markers.

Regueiro, C. A. (2003). "[Treatment of Regueiro, C. A. (2003). "[Treatment of intracranial germ cell tumours and other intracranial germ cell tumours and other tumours of the pineal region]." tumours of the pineal region]." Neurocirugia (Astur)Neurocirugia (Astur) 1414(2): 127-39.(2): 127-39. The management of patients with central nervous system germ-The management of patients with central nervous system germ-

cell tumours is evolving, and a cell tumours is evolving, and a definitive standard has not been definitive standard has not been achievedachieved. .

A large amount of data indicate that A large amount of data indicate that radiotherapy aloneradiotherapy alone results results in long-term relapse free survival rates of about 90% in patients in long-term relapse free survival rates of about 90% in patients with with germinoma.germinoma.

Various prospective trials evaluated the results of combinations Various prospective trials evaluated the results of combinations of chemotherapy and reduced dose and/or volume radiotherapy. of chemotherapy and reduced dose and/or volume radiotherapy.

The survival rates of The survival rates of combined treatment approachescombined treatment approaches were were similar to the rates achieved with craniospinal radiotherapy similar to the rates achieved with craniospinal radiotherapy alonealone. .

Nevertheless, the relapse rates were probably higher due to the Nevertheless, the relapse rates were probably higher due to the significant number of relapses that arouse outside the volume significant number of relapses that arouse outside the volume treated with radiotherapy. Additional studies are necessary to treated with radiotherapy. Additional studies are necessary to determine the appropriate radiotherapy volumes and the role of determine the appropriate radiotherapy volumes and the role of combined treatments. combined treatments.

Chemotherapy aloneChemotherapy alone results in high relapse rates and results in high relapse rates and can not be can not be recommended. recommended.

Mature teratomasMature teratomas are are benignbenign germ cell tumours that can be germ cell tumours that can be controlled with controlled with complete surgical resection in over 90% of cases.complete surgical resection in over 90% of cases.

Regueiro, C. A. (2003). "[Treatment of Regueiro, C. A. (2003). "[Treatment of intracranial germ cell tumours and other intracranial germ cell tumours and other tumours of the pineal region]." tumours of the pineal region]." Neurocirugia (Astur)Neurocirugia (Astur) 1414(2): 127-39.(2): 127-39.

Non-germinoma germ cell tumoursNon-germinoma germ cell tumours are a heterogeneous are a heterogeneous group of tumours that includes very group of tumours that includes very aggressive tumoursaggressive tumours such as mixed and pure choriocarcinomas, yolk sac such as mixed and pure choriocarcinomas, yolk sac tumours, and embryonal carcinomas; and tumours with tumours, and embryonal carcinomas; and tumours with intermediate aggressiveness such as mixed tumours with intermediate aggressiveness such as mixed tumours with germinoma and teratoma, immature teratomas and germinoma and teratoma, immature teratomas and teratomas with malignant transformation. teratomas with malignant transformation.

Both radiotherapy alone and chemotherapy alone result in Both radiotherapy alone and chemotherapy alone result in quite low rates of tumour control and current treatment quite low rates of tumour control and current treatment approaches include approaches include chemotherapy and radiotherapychemotherapy and radiotherapy, with , with surgical removalsurgical removal of the tumour in some patients. of the tumour in some patients.

PineocytomasPineocytomas are are benignbenign tumours that are controlled in tumours that are controlled in most cases by most cases by complete surgical resectioncomplete surgical resection or partial or partial surgical resection and surgical resection and local field irradiationlocal field irradiation. .

Current treatment approaches for pineoblastomas include Current treatment approaches for pineoblastomas include surgery, chemotherapy, and craniospinal irradiation with a surgery, chemotherapy, and craniospinal irradiation with a local boost. local boost.

Chemotherapy alone was used to delay irradiation in Chemotherapy alone was used to delay irradiation in infants with very little success.infants with very little success.

Konovalov, A. N. and D. I. Pitskhelauri Konovalov, A. N. and D. I. Pitskhelauri (2003). "Principles of treatment of the (2003). "Principles of treatment of the pineal region tumors." pineal region tumors." Surg NeurolSurg Neurol 5959(4): (4): 250-68.250-68. From 1976 to 1999 about 700 patients with tumors of the pineal From 1976 to 1999 about 700 patients with tumors of the pineal

region and posterior third ventricle were managed ,In more than region and posterior third ventricle were managed ,In more than 330 cases the tumor was removed.330 cases the tumor was removed.

There are four main groups of tumors: There are four main groups of tumors: – the germ cell tumors-87 (31%); the germ cell tumors-87 (31%); – the pineal parenchymal tumors-75 (27%); the pineal parenchymal tumors-75 (27%); – the glial tumors-77 (27%); and miscellaneous-43 (15%). the glial tumors-77 (27%); and miscellaneous-43 (15%).

Radiation therapy was administered in 145 (51%) and Radiation therapy was administered in 145 (51%) and chemotherapy in 16 patients. chemotherapy in 16 patients.

RESULTSRESULTS: : – A total tumor removal was achieved in 148 operations (58%), subtotal A total tumor removal was achieved in 148 operations (58%), subtotal

in 74 (29%) and partial in 33 (13%). in 74 (29%) and partial in 33 (13%). – The projected 5-year and 10-year survival rates for patients with The projected 5-year and 10-year survival rates for patients with

malignant pineal tumors, who received irradiation after tumor malignant pineal tumors, who received irradiation after tumor resection or underwent radiation therapy alone, were: 95% and 88% resection or underwent radiation therapy alone, were: 95% and 88% for pure germinomas, 80% and 50% for high grade gliomas, 44% and for pure germinomas, 80% and 50% for high grade gliomas, 44% and 0% for malignant pineal parenchymal tumors, and 20% and 0% for 0% for malignant pineal parenchymal tumors, and 20% and 0% for malignant germ cell tumors, respectively. malignant germ cell tumors, respectively.

CONCLUSIONSCONCLUSIONS: : Benign pineal tumorsBenign pineal tumors should be should be cured with surgery cured with surgery alonealone. . Malignant tumorsMalignant tumors should be treated with aggressive should be treated with aggressive resection followed with irradiation and chemotherapyresection followed with irradiation and chemotherapy. . Pure Pure germinomasgerminomas, which are exquisitely radiosensitive, can be , which are exquisitely radiosensitive, can be cured by cured by conventional radiation therapy alone.conventional radiation therapy alone.

Jaishri O. Blakeley, MD and Stuart A. Jaishri O. Blakeley, MD and Stuart A. Grossman, MD , Grossman, MD , Management of Management of Pineal Region TumorsPineal Region Tumors , , Current Treatment Current Treatment

Options in OncologyOptions in Oncology 2006, 2006, 77:505-516:505-516 Accurate histologic diagnosis important to allow rational Accurate histologic diagnosis important to allow rational

therapeutic planning. therapeutic planning. Evaluation of a pineal lesion should begin with craniospinal Evaluation of a pineal lesion should begin with craniospinal

MRI and analysis of the cerebrospinal fluid (CSF). MRI and analysis of the cerebrospinal fluid (CSF). Certainty of the histologic diagnosis is now a Certainty of the histologic diagnosis is now a requirement requirement

for treatment in for treatment in Western nationsWestern nations, some Asian centers , some Asian centers continue to recommend a test dose of radiation therapy continue to recommend a test dose of radiation therapy based on the high incidence of germinoma in those based on the high incidence of germinoma in those countries. countries.

If there is high clinical suspicion of a germinoma or tectal If there is high clinical suspicion of a germinoma or tectal glioma, glioma, stereotactic or endoscopicstereotactic or endoscopic biopsy may be pursued. biopsy may be pursued.

All other lesions should be referred for All other lesions should be referred for open biopsyopen biopsy with with microsurgical techniques. This approach provides microsurgical techniques. This approach provides adequate tissueadequate tissue for diagnosis, may be for diagnosis, may be curativecurative in low- in low-grade tumors, and may substantially grade tumors, and may substantially improve survivalimprove survival in in patients with malignant tumors. patients with malignant tumors.

Jaishri O. Blakeley, MD and Stuart A. Jaishri O. Blakeley, MD and Stuart A. Grossman, MD , Grossman, MD , Management of Management of Pineal Region TumorsPineal Region Tumors , , Current Treatment Current Treatment

Options in OncologyOptions in Oncology 2006, 2006, 77:505-516:505-516 If open surgery is not desired by the patient or If open surgery is not desired by the patient or

practitioner, stereotactic or endoscopic biopsy may be practitioner, stereotactic or endoscopic biopsy may be followed by radiosurgery for localized, well-demarcated followed by radiosurgery for localized, well-demarcated tumors. tumors.

Radiation therapy is the first-line therapy for Radiation therapy is the first-line therapy for germinomasgerminomas..

Evidence of Evidence of CSF seedingCSF seeding requires requires craniospinal radiation craniospinal radiation and adjuvant chemotherapyand adjuvant chemotherapy regardless of tumor typeregardless of tumor type. .

Diagnosis of any of the Diagnosis of any of the malignant tumorsmalignant tumors (non-germ cell (non-germ cell tumors, pineoblastomas, and parenchymal tumors of tumors, pineoblastomas, and parenchymal tumors of intermediate determination) also requires intermediate determination) also requires craniospinal craniospinal radiationradiation (with local tumor doses of at least 50 Gy) and (with local tumor doses of at least 50 Gy) and adjuvant chemotherapyadjuvant chemotherapy (generally platinum based). (generally platinum based).

Patients with Patients with tectal gliomas may undergo excisiontectal gliomas may undergo excision with with or without postoperative radiation; however, they also or without postoperative radiation; however, they also may be observed with vigilant follow-up alone. may be observed with vigilant follow-up alone.

Nishioka, H., J. Haraoka, et al. (2006). Nishioka, H., J. Haraoka, et al. (2006). "Management of intracranial germ cell "Management of intracranial germ cell tumors presenting with rapid tumors presenting with rapid deterioration of consciousness." deterioration of consciousness." Minim Minim Invasive NeurosurgInvasive Neurosurg 4949(2): 116-9.(2): 116-9. Three patients who presented with rapid Three patients who presented with rapid

deterioration of consciousness but resulted in deterioration of consciousness but resulted in complete remission of the tumor after complete remission of the tumor after emergency surgery for both emergency surgery for both diagnostic diagnostic (biopsy) (biopsy) and and therapeutictherapeutic (for hydrocephalus) purposes (for hydrocephalus) purposes followed by prompt initiation of followed by prompt initiation of radiochemotherapy. radiochemotherapy.

CONCLUSIONCONCLUSION: : – For management of these rapidly deteriorating For management of these rapidly deteriorating

patients, immediate histological verification and patients, immediate histological verification and avoidance of delay in the induction of avoidance of delay in the induction of radiochemotherapy are essential. radiochemotherapy are essential.

– Thus, emergency surgery with a less invasive Thus, emergency surgery with a less invasive procedure is the first choice of treatment, i. e., procedure is the first choice of treatment, i. e., endoscopic surgery for pineal region tumor and CT-endoscopic surgery for pineal region tumor and CT-guided biopsy for basal ganglia tumor.guided biopsy for basal ganglia tumor.

Preoperative detailsPreoperative details

Patients presenting with hydrocephalus Patients presenting with hydrocephalus and radiographic evidence of a and radiographic evidence of a malignant pineal region tumor may malignant pineal region tumor may have their hydrocephalus treated with have their hydrocephalus treated with third ventriculostomythird ventriculostomy or or ventriculoperitoneal ventriculoperitoneal (VP) shunt(VP) shunt prior to prior to biopsy or resection. biopsy or resection.

The The staged procedurestaged procedure allows for allows for definitive control of the hydrocephalus definitive control of the hydrocephalus prior to surgical resection of lesions prior to surgical resection of lesions suspicious for being malignant. suspicious for being malignant.


A similar strategy may be employed for A similar strategy may be employed for patients with marked symptomatic patients with marked symptomatic hydrocephalus and benign-appearing lesions. hydrocephalus and benign-appearing lesions.

The timing of the second procedure can vary The timing of the second procedure can vary according to the surgeon's preference. according to the surgeon's preference.

Peritoneal seedingPeritoneal seeding with shunting is a rare, but with shunting is a rare, but well-documented, complication in these well-documented, complication in these patients. patients.

However, the However, the use of a filteruse of a filter to decrease the to decrease the incidence of seeding has been associated with incidence of seeding has been associated with frequent shunt malfunctions and generally is frequent shunt malfunctions and generally is not recommended, particularly because third not recommended, particularly because third ventriculostomy is a better option ventriculostomy is a better option


Improved endoscopic techniques have Improved endoscopic techniques have made third ventriculostomy an easy and made third ventriculostomy an easy and reliable method to divert CSF. reliable method to divert CSF.

As with all CSF diversion procedures, CSF As with all CSF diversion procedures, CSF may be acquired and sent for cytologic may be acquired and sent for cytologic and biochemical analysis during the case. and biochemical analysis during the case. Third ventriculostomy has the added Third ventriculostomy has the added advantage of potentially allowing for a advantage of potentially allowing for a biopsy during the procedure by biopsy during the procedure by endoscopic guidance. endoscopic guidance.

This provides the opportunity to make an This provides the opportunity to make an intraoperative diagnosis with subsequent intraoperative diagnosis with subsequent tailoring of further therapy. tailoring of further therapy.

Cipri, S., A. Gangemi, et al. (2005). Cipri, S., A. Gangemi, et al. (2005). "Neuroendoscopic management of "Neuroendoscopic management of hydrocephalus secondary to midline and hydrocephalus secondary to midline and pineal lesions." pineal lesions." J Neurosurg SciJ Neurosurg Sci 4949(3): 97-(3): 97-

106106 In 14 select cases, hydrocephalus was secondary to midline and pineal In 14 select cases, hydrocephalus was secondary to midline and pineal lesions, with a follow-up ranged from 3 months to 5 years after discharge. lesions, with a follow-up ranged from 3 months to 5 years after discharge.

In 9 cases the endoscopic procedure represented the only surgical In 9 cases the endoscopic procedure represented the only surgical treatment. treatment.

In 5 cases, microsurgical removal of the lesions and/or ventriculo-In 5 cases, microsurgical removal of the lesions and/or ventriculo-peritoneal shunts placement were performed, as additional treatment, peritoneal shunts placement were performed, as additional treatment,

while adjuvant radiotherapy was utilized in 4 cases; high dose while adjuvant radiotherapy was utilized in 4 cases; high dose chemotherapy followed by bone marrow transplantation was performed in chemotherapy followed by bone marrow transplantation was performed in 3 cases. 3 cases.

RESULTSRESULTS: obstructive hydrocephalus secondary to midline and pineal : obstructive hydrocephalus secondary to midline and pineal lesions, was successful treated by neuroendoscopic approach alone in 9 lesions, was successful treated by neuroendoscopic approach alone in 9 cases, with an unremarkable course and good outcome, except in 1 case. cases, with an unremarkable course and good outcome, except in 1 case.

CONCLUSIONSCONCLUSIONS: : Neuroendoscopic approachNeuroendoscopic approach affords a affords a minimally invasiveminimally invasive way to obtain 4 objectives by one-step surgical approach, way to obtain 4 objectives by one-step surgical approach, – such as resolution of obstructive hydrocephalus by endoscopic third such as resolution of obstructive hydrocephalus by endoscopic third

ventriculostomy (ETV), ventriculostomy (ETV), – cerebrospinal fluid sample to detect tumor markers and to perform cytological cerebrospinal fluid sample to detect tumor markers and to perform cytological

analysis, analysis, – biopsy specimens and tissue diagnosis, biopsy specimens and tissue diagnosis, – associated to absence of shunt-related complications. associated to absence of shunt-related complications.

Therefore, in experienced hands, ETV should be the treatment of first Therefore, in experienced hands, ETV should be the treatment of first choice, in cases of hydrocephalus secondary to lesions of the pineal gland. choice, in cases of hydrocephalus secondary to lesions of the pineal gland.

Yamini, B., D. Refai, et al. (2004). "Initial Yamini, B., D. Refai, et al. (2004). "Initial endoscopic management of pineal region endoscopic management of pineal region tumors and associated hydrocephalus: tumors and associated hydrocephalus: clinical series and literature review." clinical series and literature review." J J NeurosurgNeurosurg 100100(5 Suppl Pediatrics): 437-(5 Suppl Pediatrics): 437-41.41.

In six patients with pineal tumors and associated hydrocephalus In six patients with pineal tumors and associated hydrocephalus who underwent an endoscopic biopsy procedure and third who underwent an endoscopic biopsy procedure and third ventriculocisternostomy (ETVC) in a single sitting. ventriculocisternostomy (ETVC) in a single sitting.

RESULTRESULT: The ETVC was successfully performed without : The ETVC was successfully performed without complication in all patients; however, a ventriculoperitoneal shunt complication in all patients; however, a ventriculoperitoneal shunt was eventually required in four. was eventually required in four. – Histological diagnosis was successfully established in four patients. Histological diagnosis was successfully established in four patients.

The authors also reviewed the literature to assess reports The authors also reviewed the literature to assess reports involving ETVC and tumor biopsy sampling in patients with pineal involving ETVC and tumor biopsy sampling in patients with pineal tumors and hydrocephalus. tumors and hydrocephalus. – A total of 54 cases, including those in this study, have been reported. A total of 54 cases, including those in this study, have been reported. – Fifteen percent of the patients eventually required placement of a Fifteen percent of the patients eventually required placement of a

ventricular shunt. ventricular shunt. – The transient complication rate was 15% with no death. A positive The transient complication rate was 15% with no death. A positive

tissue diagnosis was established in 89% of the cases overall. tissue diagnosis was established in 89% of the cases overall. CONCLUSIONSCONCLUSIONS: The authors conclude that the endoscopic : The authors conclude that the endoscopic

management of patients with pineal region masses and management of patients with pineal region masses and hydrocephalus may be a preferred initial strategy.hydrocephalus may be a preferred initial strategy.

Intraoperative detailsIntraoperative details

In general, surgical approaches to the In general, surgical approaches to the pineal region can be divided into pineal region can be divided into supratentorial, infratentorial, and a supratentorial, infratentorial, and a combined supratentorial-infratentorial combined supratentorial-infratentorial approach. approach.

Supratentorial approaches include the Supratentorial approaches include the parietal-interhemispheric approach parietal-interhemispheric approach described by Dandy (1936) and the described by Dandy (1936) and the occipital-transtentorial approach occipital-transtentorial approach originally described by Horrax, later originally described by Horrax, later modified by Poppen. modified by Poppen.

Koziarski, A., E. Skrobowska, et al. (2003). Koziarski, A., E. Skrobowska, et al. (2003). "[Own experience in surgical treatment of "[Own experience in surgical treatment of the pineal region and midbrain tumors via the pineal region and midbrain tumors via the infratentorial approach]." the infratentorial approach]." Neurol Neurol Neurochir PolNeurochir Pol 3737(2): 473-84.(2): 473-84.

Nine cases of tumours located in the pineal and midbrain region in Nine cases of tumours located in the pineal and midbrain region in adults operated on between November 1998 and July 2002 adults operated on between November 1998 and July 2002

The histopathological examination revealed The histopathological examination revealed – anaplastic pinealoma in 2 cases, anaplastic pinealoma in 2 cases, – and pineocytoma, pineal cyst, mesencephalic glial cyst, protoplasmatic and pineocytoma, pineal cyst, mesencephalic glial cyst, protoplasmatic

astrocytoma, epidermoid cyst, unclear glial scar, and papillary astrocytoma, epidermoid cyst, unclear glial scar, and papillary ependymoma in single cases. ependymoma in single cases.

Occipito-suboccipital osteoplastic craniotomy was performed in Occipito-suboccipital osteoplastic craniotomy was performed in each case and tumours were totally removed microsurgically via each case and tumours were totally removed microsurgically via the infratentorial epicerebellar approach. the infratentorial epicerebellar approach.

All the patients improved significantly and resumed their previous All the patients improved significantly and resumed their previous life activities. life activities.

Three patients with pineal tumours and one with a small Three patients with pineal tumours and one with a small postoperative ependymoma recurrence were irradiated after the postoperative ependymoma recurrence were irradiated after the surgery. surgery.

CONCLUSIONCONCLUSION : :Very good resultsVery good results of the surgical treatment of of the surgical treatment of tumours in this area suggest that such patients should be tumours in this area suggest that such patients should be referred referred earlier to one stage surgical managementearlier to one stage surgical management, as the procedure is , as the procedure is easier to perform and shunt implantation may be avoided.easier to perform and shunt implantation may be avoided.

Postoperative detailsPostoperative details

Once the physician makes a diagnosis of Once the physician makes a diagnosis of malignant tumor from tissue acquired malignant tumor from tissue acquired intraoperatively, the surgeon is obligated to intraoperatively, the surgeon is obligated to evaluate the patient for spinal metastasis. evaluate the patient for spinal metastasis.

Prior to widespread use of the MRI, patients' Prior to widespread use of the MRI, patients' disease was staged postoperatively with CT disease was staged postoperatively with CT myelogram. myelogram.

Currently, the most sensitive radiographic Currently, the most sensitive radiographic modality for screening is a complete spinal MRI modality for screening is a complete spinal MRI with and without gadolinium. with and without gadolinium.

The first MRI scan should be timed at least 2 The first MRI scan should be timed at least 2 weeks after surgery because spinal canal weeks after surgery because spinal canal enhancement can occur in the early enhancement can occur in the early postoperative period. postoperative period.

Postoperative detailsPostoperative details

Equivocal findings on the initial postoperative Equivocal findings on the initial postoperative scan warrant a repeat scan within 1-2 weeks. scan warrant a repeat scan within 1-2 weeks.

Radiographic artifacts secondary to surgery Radiographic artifacts secondary to surgery regress while drop metastasis remain stable regress while drop metastasis remain stable or increase in size over time. or increase in size over time.

The role for The role for postoperative lumbar puncturepostoperative lumbar puncture and subsequent CSF analysis for cytology is and subsequent CSF analysis for cytology is questionable. questionable.

The presence of abnormal cells The presence of abnormal cells postoperatively does postoperatively does not correlatenot correlate well with well with spinal metastasis due to spinal metastasis due to spillage during spillage during surgerysurgery. .

Follow-up careFollow-up care

Life-long follow-up of children with pineal Life-long follow-up of children with pineal region tumors is required. These tumors can region tumors is required. These tumors can recur locally or appear distally as late as 5 recur locally or appear distally as late as 5 years after diagnosis. In addition, patients can years after diagnosis. In addition, patients can present later in life with new tumor formation present later in life with new tumor formation (eg, meningioma). (eg, meningioma).

MRI scans should be obtained on a periodic MRI scans should be obtained on a periodic basis as determined by tumor histology of basis as determined by tumor histology of original diagnosis, extent of resection, and original diagnosis, extent of resection, and presence of metastasis at time of diagnosis. presence of metastasis at time of diagnosis.

Tumor marker studies for patients with germ Tumor marker studies for patients with germ cell tumors should be performed also on a cell tumors should be performed also on a periodic basis, even if markers were not periodic basis, even if markers were not abnormal at diagnosis. abnormal at diagnosis.

The Management of Pineal Area Tumors:The Management of Pineal Area Tumors:A Recent ReappraisalA Recent ReappraisalPAUL H. CHAPMAN, MD,* AND RITA M. PAUL H. CHAPMAN, MD,* AND RITA M. LINGGOOD, MDt, LINGGOOD, MDt, Cancer Cancer 46:1253-1257, 46:1253-1257, 1980.1980. 22 patients with pineal area tumors from 1972 through 1977. 22 patients with pineal area tumors from 1972 through 1977. Eleven identified tumors included: three gliomas, three Eleven identified tumors included: three gliomas, three

pineoblastomas, two endodermal sinus tumors, one germinoma, pineoblastomas, two endodermal sinus tumors, one germinoma, one epidermoid, and one metastasis. Three others were one epidermoid, and one metastasis. Three others were presumptively germinomas. presumptively germinomas.

Twelve cases were treated by means of radiation and shunting Twelve cases were treated by means of radiation and shunting if necessary. if necessary.

Eight patients underwent direct surgery and then radiotherapy Eight patients underwent direct surgery and then radiotherapy if appropriate. if appropriate.

There were 6 deaths from recurrence. There were 6 deaths from recurrence. Death occurred in spite of radiologic evidence of radiation Death occurred in spite of radiologic evidence of radiation

response. response. Cerebrospinal fluid (CSF) cytologyCerebrospinal fluid (CSF) cytology was was positive in 2 of 7 casespositive in 2 of 7 cases

involving potentially disseminating tumors and involving potentially disseminating tumors and negative in 2 negative in 2 cases involving spinal metastases. cases involving spinal metastases.

CONCLUSION: CONCLUSION: Radiation response and CSF cytology are Radiation response and CSF cytology are insufficientinsufficient to determine optimum treatment. to determine optimum treatment.

Direct operations, which were got associated with mortality or Direct operations, which were got associated with mortality or serious morbidity were most useful for providing a tissue serious morbidity were most useful for providing a tissue diagnosis.diagnosis.

OUTCOME AND OUTCOME AND PROGNOSISPROGNOSIS

The prognosis for patients with pineal region tumors is The prognosis for patients with pineal region tumors is dependent upon dependent upon tumor histologytumor histology and is subject to and is subject to change as more effective adjuvant therapy is change as more effective adjuvant therapy is developed. developed.

In general, patients with intracranial In general, patients with intracranial germinomasgerminomas have have an excellent prognosis because of the radiosensitivity an excellent prognosis because of the radiosensitivity of these tumors. of these tumors.

Children with Children with nongerminomatous germ cellnongerminomatous germ cell tumors tumors have a significantly worse prognosis than do children have a significantly worse prognosis than do children with germinomas or pineal cell tumors. with germinomas or pineal cell tumors.

No conventional approach is designed for managing No conventional approach is designed for managing recurrencerecurrence. Chemotherapy, radiotherapy, or . Chemotherapy, radiotherapy, or radiosurgery can be applied if maximal doses have not radiosurgery can be applied if maximal doses have not already been administered. already been administered.

A second surgical procedure generally is reserved for A second surgical procedure generally is reserved for patients with benign lesions who demonstrate patients with benign lesions who demonstrate recurrence several years later. recurrence several years later.

OUTCOME AND OUTCOME AND PROGNOSISPROGNOSIS

Recurrent germ cell tumors have Recurrent germ cell tumors have been shown to respond to been shown to respond to chemotherapy, as have some chemotherapy, as have some pineal cell tumors, although to a pineal cell tumors, although to a lesser degree. lesser degree.

Radiosurgery may be a Radiosurgery may be a consideration for all recurrences consideration for all recurrences less than 3 cm in diameter.less than 3 cm in diameter.

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Literature Review Of Management Of Pineal Region Tumour

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