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CENTRAL NERVOUS SYSTEM CENTRAL NERVOUS SYSTEM TUMORS IN CHILDRENTUMORS IN CHILDREN
CENTRAL NERVOUS SYSTEM CENTRAL NERVOUS SYSTEM TUMORS IN CHILDRENTUMORS IN CHILDREN
DR BIKASH RANJAN PRAHARAJ
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Percentage Distribution of Childhood Cancers
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INTRACRANIAL TUMORS (ICTS)
Primary or MetastaticOccur with equal frequency in adults, but in children primary tumors are far more common.Primary ICTs account for ~2% of cancers in adults 20% of all cancers in children.In children 70% of ICTs arise in Posterior fossa (infra-tentorial). asIn adults 70% of ICTs arise in Supra-tentorial.Cranial exposure to ionizing radiation is a risk factor.
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Because of its location, a benign ICT may have fatal “malignant” effects.Malignant ICTs spread by: Direct infiltration of adjacent tissues May disseminate within the CNS via CSF.Gliomas account for 60% of primary ICTsMeningiomas for 20% & all others 20%.All CNS tumors behave as malignant clinically (Limited space)
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Cytologic origin of CNS tumors
Neuro-ectodermalNeuro-ectodermal – most important are the – most important are the GliomasGliomas;;
MesenchymalMesenchymal – most frequent ones are the – most frequent ones are the MeningiomasMeningiomas;;
Ectopic tissuesEctopic tissues – from tissues „displaced” – from tissues „displaced” during embryogenesis: Ex., during embryogenesis: Ex., DermoidDermoid cyst; cyst;
Retained embryonal structuresRetained embryonal structures: various cysts – : various cysts – Paraphyseal cystParaphyseal cyst;;
MetastasesMetastases:: Lung, Breast, Melanoma, etc. in Lung, Breast, Melanoma, etc. in 50% of cases50% of cases
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Neuro – ecto - dermal tumors
Glial cells:
astrocytes (A) - Astrocytoma
Oligodendroglial cells - Oligodendroglioma
Ependymal cells – Ependymoma
Neurons - Gangliocytoma
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CLINICAL PRESENTATION
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The unique features of CNS tumors – “ICP”
1. They grow in a unique environment: the intracranial space.2. The intracranial contents - incompressible Brain and blood contained within a rigid unyielding bony structure.3. Intracranial pathologies (tumors, abscess, hematoma, infarction, edema, etc.) eventually produce life threatening increase of the intracranial-pressure: ICP.
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Increased Intracranial Pressure (ICP)
Headaches, progressively worsening
Vomiting (morning)
Irritability
Papilledema rare < 2 y/o - head can expand
“Double vision” with 6th nerve palsy
Head tilt
Bulging fontanelle (infant)
In a young child with ? brain tumor:In a young child with ? brain tumor:Measure head circumference and observe gaitMeasure head circumference and observe gait
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Supratentorial Tumors
Signs depend on age and location:Younger child:
Developmental delay or loss of milestones
Older child: Deteriorating school performance Personality changes
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Depending on location
Supratentorial tumours:
- Motor weakness
- Sensory changes
- Speech disorders
- Seizures
- Reflex abnormalities
- Hand preference
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In midline/infratentorial tumours:- Classical triad of headache, nausea & vomiting
and Papilloedema- Blurred vision, nystagmus & diplopia- Disorder of equilibrium, gait & coordination.- Torticolis: in cerebellar tonsil herniation Diencephlic syndrome:- Failure to thrive- Emaciation & decreased appetite- Euphoric affect
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Brain stem tumours:
- Gaze palsy
- Multiple cranial N palsies
- UMN deficits Optic N pathway tumours:
- Visual disturbances like decreased visual acuity, marcus gunn pupil, nystagmus & visual field defects
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Suprasellar & 3rd Ventricle region tumours
Leads to neuroendocrine disturbances like DI Galactorrhoea Precocious puberty Delayed puberty Hypothyroidism
Perinaud syndrome: - Tumour of pineal region- C/F : paresis of upward gaze, pupillary dilation, reactive
to accommodation,not to light,eyelid retraction
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Spinal cord tumours:
- long N tract motor &/or sensory deficits
- Bowel & bladder deficit
- Radicular or back pain
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Seen in kids with increased cellularity
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ASTROCYTOMASAccount for ~ 80% of primary ICTS in adults
MC in the cerebral hemispheres
MC Symptoms: headaches, seizures, focal neurologic deficits ( usually in the anterior or middle)
Low-grade Astrocytomas: Gross:
Poorly defined gray-white infiltrative tumors.
Histology: Hypercellularity; astrocytic nuclei of mild degree of atypia & astrocytic processes fibrillary background = fingers of
astrocytes
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Low-grade Astrocytomas
Pilocytic Astrocytomas: MC in the cerebellum of children & young adults; and
less commonly in the optic nerve, hypothalamic region or cerebral hemispheres
Morphology:Cystic, with a tumor nodule in the wall of the cyst.Composed of bipolar astrocytes, with long hair-like processes, Rosenthal fibers & Micro-cysts+ calcification = good prognosis
Grow very slowly (some patients have survived for >40 yrs after incomplete resection) & have an Excellent prognosis
DD: not to confuse with low grade Fibrillary Astrocytoma
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Rosenthal fibers
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Gr. III. astrocytoma
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Gr. IV. astro ~ = GBM
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GBM: necrosis/pseudo-palisade
Pseudo pallisding central necrosis with perpendicular cells
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Adverse prognostic factors in pt with high grade Astrocytoma
Older age Histologic f/o glioblastoma Poor karnofsky performance status Unresectable tumour Pt with unmethylated MGMT
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Glioblastoma Cerebri
Highly infiltrating, non enhancing tumour involving more than 2 lobes
Histologically different from Glioblastoma but behave aggressively & has poor outcome
T/t: RT + Temozolamide
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OLIGODENDROGLIOMA
Comprise ~ 5 -15% of GliomasArise in the cerebral white matter MC in the 4th & 5th decadesGross: Well circumscribed, gelatinous, gray masses, with foci
of hemorrhage & calcification.
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Histology: Sheets of cells with rounded nuclei surrounded
by a halo of clear cytoplasm (fried egg appearance).
There is often a delicate network of capillaries & scattered foci of calcification (psammoma bodies)
Grows slowly, presents commonly with seizures, prognosis is better than Astrocytoma, average survival is 5-10 yrs (with modern therapeutic approaches)
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Oligodendroglioma
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EPENDYMOMA
Arise from the Ependymal lining of the ventricles or the central canal of the spinal cord Arise in the Fourth ventricle in children & young adults Spinal cord in the middle aged.Morphology: Highly cellular, tumor cells have regular nuclei May exhibit epithelial features with formation of
“rosettes” (Flexner Landsteiner) also perivascular pseudo-rosettes (homer Wright)
Most tumors are well differentiated
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4th ventricle tumors: May cause hydrocephalus, usually can’t
be completely removed CSF dissemination may occur Average survival is ~ 4 yrs
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Myxo-papillary Ependymomas
Arise in the filum terminale of the spinal cord Prognosis depends on completeness of surgical
excision
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Ependymoma
““Rosettes” & perivascular Pseudo-rosettesRosettes” & perivascular Pseudo-rosettes
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MEDULLOBLASTOMAS
Second MC ICT of childhood (after Astrocytomas).Occurs exclusively in the cerebellum.Derived from fetal external granular layer of cerebellum.Grows rapidly & occludes CSF flow hydrocephalus.
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Seeds through CSF implants around the spinal cord & cauda equina (need irradiation of the whole Neuraxis).Histology: Extremely cellular, anaplastic, small round or
carrot-shaped cells with hyperchromatic nuclei, N/C, may form Homer-Wright pseudo-rosettes
Highly malignant, yet radiosensitive & 5-yr survival 75%.
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Medulloblastoma
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MedulloblastomaHomer-Wright pseudo-rosettesHomer-Wright pseudo-rosettes
carrot-shaped cellscarrot-shaped cells
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MENINGIOMA
Usually Benign slow-growing tumors of adults, F/M 3:2Originate from meningothelial cells of the arachnoid.Usually solitary ( multiple meningiomas NF2 ) Morphology: Firm rounded masses, adherent to the dura and
compressing the underlying brain (no infiltration). Histologic variants include:
Syncytial, fibroblastic, transitional, Psammomatous & papillary ( propensity to recur).
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Malignant Meningioma is very rare Infiltrates the underlying brain, shows
marked nuclear atypia, mitoses, & foci of necrosis.
Other rare sarcomas of meninges include: Hemangiopericytoma, malignant fibrous
histiocytoma & Fibrosarcoma.
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Meningioma
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MeningiomaSyncytialSyncytial PsammomatousPsammomatous Epithelial Membrane Antigen
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NERVE SHEATH TUMORS1. Schwannomas:
Benign tumors of Schwann cells MC in the vestibular branch of the VIII CN at the cerebello-
pontine angle (acoustic neuroma) tinnitus & hearing loss Also involve branches of the trigeminal nerve & dorsal nerve
roots Tumors are encapsulated, attached to one side of the nerve;
axons do not pass through the tumor
Consist of Antoni -A areas of high cellularityAntoni -B myxoid areas
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Schwannoma
Antoni – A hyprecellularAntoni – A hyprecellularAntoni –B Sparsely cellularAntoni –B Sparsely cellular
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2. Neurofibromas: Benign tumors composed predominately of Schwann cells, but also
containing fibroblasts & perineural cells May involve single or multiple dorsal spinal nerve roots (multiple in
patients with von Ricklinghausen's disease – NF1) CN involvement is extremely rare May present as
Localized fusiform enlargement of a nerve or
Extensively infiltrate along the nerve “ropy enlargement” of the nerve & it’s branches (plexiform Neurofibroma) Plexiform neurofibromas are usually part of NF1, excision is
very difficult Histology:
Wavy spindle shaped cells, myxoid collagenous stroma with interspersed nerve fibers
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„Acoustic Neurinoma” (Schwannoma)
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METASTATIC ICTS
Very rare in children. Common Primaries: Broncho-genic small cell undifferentiated (oat cell) ca.,
Breast ca., Malignant melanoma, RCC. & Colon ca.Sites of metastases: Cerebral cortex 80%; Rest are in the cerebellum & brain stem.
50% are multiple; at the junction between the gray & white matter.
Vertebral column is a common site for metastases of Breast & Prostatic carcinomas Thoracic spine 60%, Cervical 20% & Lumbar 20%Treatment : Radiotherapy
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CHOROID PLEXUS PAPILLOMA MC in children Arising from the lateral ventriclesIn adults they are found MC in the 4th ventricle Present with Hydrocephalus Due to either over-production of CSF or to
obstruction of the ventricular system.Consist of papillae with fibrovascular stalks covered with a cuboidal or columnar ciliated epithelium, recapitulating the structure of the normal choroid plexus.
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COLLOID CYST OF THE THIRD VENTRICLEA non-neoplastic cystic lesionMorphology: Having a thin fibrous capsule, a lining of Cuboidal to
columnar epithelium & containing gelatinous Proteinaceous material.
Attached to the roof of the third ventricle at the foramina of Munroe & may cause sudden obstruction of the CSF flow acute non-communicating hydrocephalus brain herniation & deathSymptoms: headaches (often positional), “drop attacks”, incontinence Goblet cells are confirmatory
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MISCELLANEOUS (MIDLINE) TUMORS
Pinealomas: True pineocytomas are extremely rare, may also have
pineoblastomas
Germinomas: MC in the pineal & suprasellar regions in adolescents &
young adults Closely resemble testicular Seminomas & ovarian
Dysgerminomas Other GCTs (Teratomas & Choriocarcinomas) also occur
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Craniopharyngioma
Benign cystic tumors of children & adolescents
Develop in the suprasellar region Hypopituitarism
Originate from remnants of Rathke’s pouch & contain squamous & columnar epithelium
calcifications are common.
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CNS LYMPHOMAPrimary CNS lymphomas
Account for ~1% of ICTs MC CNS neoplasm in AIDS & other
immunosuppressed patients often arise deep within the cerebral hemispheres & are commonly bilateral
Lymphoma cells exhibit an angiocentric distribution
Usually are B-cell lymphomas & many appear to be EBV-related.
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PCNSL: Pathophysiology
Focal Lesion most common presentation: others include diffuse, uveal, leptomeningeal, and intramedullary.
Infiltrates normal brain diffusely.
Spreads along CSF pathways.
Rarely spreads outside the CNS.
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Secondary CNS lymphomas
Lymphomas arising outside of the CNS rarely
involve the brain parenchyma
May involve the meninges, intradural spinal nerve
roots & epidural space
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Leptomeningeal Carcinomatosis
Occurs in 5-8% of patients with solid tumors
Most common tumors to metastasize to the leptomeninges are small cell carcinoma (9-18%), and NHL (5-29%)
Other tumors include non-small cell lung, breast, melanoma, and G.U.
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Leptomeningeal Carcinomatosis: Clinical Presentation
SYMPTOMS SIGNS
Headaches 33% Reflex assymetry(71%)
Lower Motor Weakness 38% Weakness 60%
Parasthesias 34%
Back/Neck Pain 26% Sensory Loss 27%
Radicular Pain 33% CN III Paresis 20%
Diplopia 20% CN VII Paresis 17%
Mental Status change 17%
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Carcinomatous Meningitis
Diagnosis:
MRI
Cerebral Spinal Fluid
Rarely myelography
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PHAKOMATOSES
Neurocutaneous syndromes ADHamartomas & Neoplasms
Esp. involving the nervous system & skin Mutations in tumor suppressor genes
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1. Neurofibromatosis Type 1 (NF1): Neurofibromas, Neurofibro-sarcomas Optic nerve Gliomas Pigmented cutaneous macules (café au lait
spots) Pigmented nodules of iris (Lisch nodules)
2. Neurofibromatosis Type 2 (NF2): Bilateral Schwannomas of CN VIII Multiple meningiomas Spinal cord Ependymomas
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3. Tuberous Sclerosis
Hamartomas (“tubers”) in the cerebral cortex, Sub-Ependymal hamartomas (“candle drippings”) & Sub-Ependymal giant cell Astrocytomas
Seizures & mental retardation
Extra CNS findings: Kidney (Angiomyolipoma), Heart
(Rhabdomyoma MCC in kids, adult = myxomas) , skin (Angiofibroma)
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Tuberous Sclerosis
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4. von Hippel-Lindau disease
Hemangioblastomas of the cerebellum, retina, brain stem & spinal cord
Cysts of liver, kidney & pancreas
incidence of RCC, may be bilateral
~ 10% of Hemangioblastomas polycythemia
Total surgical removal is curative
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Von Hippel-Lindau disease
HemangioblastomasHemangioblastomas
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DIAGNOSISDIAGNOSIS
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Evaluation on emergency basis Complete history, physical examination &
neurologic assessment with neuroimaging For primary tumours: MRI with gadolinium
contrast is the gold standard. Low grade glioma: FLAIR MRI MRI better than CT: Tumor of suprasellar,
optic path, infratentorial & pituitary region
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Specific investigations Neuroendocrine dysfunction evaluation : in tumor
of suprasellar,midline, pituitary & optic chiasmal region
Serum & CSF bHCG and AFP: germ cell T Lumbar Puncture:
- Indication: Medulloblastoma, ependymoma & germ cell tumours
- C/I : newly dx hydrocephalous sec. to CSF flow obstruction, tumour causing supratentorial midline shift & infratentorial tumours.
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MANAGEMENTMANAGEMENT Multimodal i.e. surgery +/- chemoradiation Symptomatic & definitive Definitive:- depends on specific tumour
type
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Symptomatic
Glucocoticoids: decrease perilesional edema & improve neurologic function
- DexamethasoneDexamethasone is the DOC bcoz of low mineralcorticoid activity
Seizure: anticonvulsants- leviteracetam, topiramate, lamotrigine, valproic acid &
Lacosamide- Phenytoin & carbamazepine should be avoided as
they may hamper the action of chemo bcoz they are enzyme inducers.
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Venous thromboembolism:
- Ass. With 20-30 % patients with high grade glioma & brain metastasis
- Anticoagulants should be used prophylactically
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Astrocytoma: Surgical Treatment
Accurate histological grade Palliation of mass effect
? Improved quality of life
Decreased dexamethasone requirement
? Impact on overall survival Retrospective studies say yes
No prospective, randomized trials
Advantages of Large Resection
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Radiation Therapy & Chemotherapy
Chemotherapy following external beam radiation is advantageous in adults with anaplastic gliomas. Much less benefit is seen in patients with glioblastoma.
The few glioblastoma patients who benefit tend to be those that live the longest, suggesting that treatment preferentially benefits patients with favorable prognostic factors (i.e. young age, good P.S., minimal post-op, residual tumor).
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Glioblastoma
Maximal surgical resection f/b
Partial field external beam radiotherapy with concomittant Temozolamide f/b
6-12 months of adjuvant Temozolamide In c/o recurrence: reoperation, BCNU/
bevacizumab
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Temozolomide
Active metabolite of DTIC
Crosses the blood-brain barrier
Oral dosing: 200mg or 150mg/m2/x5d q4 weeks
Well tolerated: nausea/vomiting, myelosuppression
Idiosyncratic myelosuppression
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Temozolomide
Indications: Relapsed Gr III anaplastic gliomas
Grade IV glioblastoma multiformae
Role in initial therapy uncertain: Probably equivalent to nitrosourea or PCV for high
grade gliomas Role as a radiosensitizer highly questionable(?) effective in oligodendroglioma.
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Oligodendroglioma: Treatment
Complete surgical resection optimal Radiation therapy of questionable benefit Anaplastic oligodendrogliomas are chemosensitive tumors:Active regimens include: Single agent nitrosoureas PCV Temozolomide Other alkylating agentsChromosomal karyotypes may help predict therapeutic and clinical outcomes.
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PCNSL: Treatment
For Diagnostic purposes only; extensive resection contraindicated.
Histologic diagnosis may be difficult from a stereotactic biopsy.
Surgery
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PCNSL: Radiotherapy
Historically standard Tx
80% radiographic CR
14-18 month median survival
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PCNSL: Treatment
Methotrexate (high-dose)Procarbazine, CCNU, VincristineBCNU
Ara-CCyclophosphamide, Adriamycin,Vincristine, Prednisone (“CHOP”). Decadron
••Multiple drugs shown to have anti-PCNSL activity
• PCNSL recurrent after radiation is often chemotherapy sensitive, although few (if any) are cured.
• Drugs should possess anti-lymphoma activity and have at least some ability to traverse a partially disrupted, if not intact BBB
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Brain Metastases: Treatment
Solitary MetastasesGoals:
1. Palliate neurologic symptoms
2. Decrease future neurologic morbitiy
3. Prolong Survival
4. Potentially “cure” selected patients
Treatment:
1. Surgery if medically and anatomically indicated.
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Brain Metastases: Treatment
Multiple MetastasesGoals:
1. Palliate neurological symptoms
2 Decrease future neurological morbidity
3. Prolong survival in selected patients
Treatment:
1. Whole brain XRT
2. Chemotherapy for chemosensitive tumors (i.e. breast cancer, small cell lung carcinoma)
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Leptomeningeal Carcinomatosis
Goals of Treatment:
Destroy circulating and perineural
neoplastic cells to stabilize or improve
neurologic dysfunction.
Prolong survival in specific tumor types
(lymphoma, leukemia, breast carcinoma).
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Leptomeningeal Carcinomatosis
Radiation
Involved field for acute cranial or spinal nerve involvement
No cranial-spinal
Chemotherapy
Intra-CSF: Intrventricular vs. Intrathecal (MTX, ara-C, Depocyte, Thiotepa) Ease of administration Patient Comfort Optimal CSF drug distribution
Systemic (?)
Treatment:
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Spinal Cord Compression
DexamethasoneSurgical Decompression
LaminectomyAnterior resection
Radiotherapy treatment of choice.
Treatment:
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Spinal Cord Compression
Indications for Surgical Interventions:Chance of restoring/preserving neurological function (anterior resection)*Initial presentation from an unknown neoplasm.Previously irradiated field. *Vertebral instability. *Progressive neuroloic deteriation during and following XRT. * Intractable pain despite adequate XRT, steroids, and analgesics.
* The decision to surgically intervene ultimately depends on an estimate of the morbidity of surgery versus the potential for significant quality survival.
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Take home points
Brain tumors are second most common Brain tumour with best prognosis in
children is JPA & worst prognosis : Brain stem Gliomas
Over 70% of children diagnosed with
cancer can be cured of their disease. Management is through multimodal
approach
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