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GIANT CELL TUMOUR OF SCAPULA

IDENTIFICATION DATA

Name

: Jahuriya Anbi

Age

: 17 years old

R/N

: 616800

CASE HISTORY

Miss Jahuriya is a 17 years old Malay women who presented to the orthopedic clinic with a complaint of left shoulder pain for the past 1 year. The pain was dull aching in nature, constantly present non-radiating and became progressively worsen the past 6 month. The pain was present even at night and there was no associated relieving or aggravating factors. She denies any history of significant related trauma fever. The severity of pain had limited her movement of her left shoulder. There were no neurovascular symptoms of her left upper limb, neck or spine. There was no history of contact with tuberculosis patients.

On examination, she was afebrile; vital signs were stable. Local examination of her left shoulder revealed no obvious swelling or any marked skin changes. She had remarked tenderness over her anterior shoulder joint line. There was no axillary lymphadenopathy. There was no neurological deficit of her left upper limb. Radial and brachial pulses were felt and strong. Examination of other systems was normal. Her left upper limb was immobilized in arm sling.

Radiograph of her left scapula region showed lytic lesion present over the superior part of the glenoid area with multiseptate cavity. The left coracoid process appears sclerotic. Based on radiograph, the initial differential diagnosis was giant cell tumor and aneurysmal bone cyst. Chest radiograph was clear.

CT scan of her left shoulder showed a well-defined expansile lytic lesion present in the left scapula involving the coracoid process, glenoid and part of the body with multiple septation within. It has a well-defined sclerotic margin and measured 5 x 3 x 4 cm. There was a break in cortex medially with a small soft tissue component measuring 1.5 cm thick. The glenohumeral join appears normal and not involved. The left humerus is osteopenic and muscles of the left arm appear atrophic. CT thorax showed no secondary deposit in the lungs.

A bone scan was done to stage the lesion and rule out any other metastatic lesion. There was presence of abnormally increased focal trace uptake is seen involving the scapula at the superior aspect of the left glenoid area with increased vascularity. Tracer distribution is other areas are physiological.

Fine needle aspiration under image intensifier guidance was performed. The osteolytic lesion was identified and using Islam needle, a specimen consist of bone tissue and bloody aspiration were obtained. The tissue specimen were sent for histopathological examination.

The histophatological section showed fragment of tissue composed of sheets of mononuclear stromal cell admixed with multinucleated giant cells. Their nuclei are rounded and uniform closely resemble those within the multinucleated giant cells. The giant cells are diffusely arranged. No significant mitotic figures noted. The features obtained are consistent of giant cell tumor of the bone.

CT scan of the tumor suggested that the tumor is big and locally aggressive with the break of the cortex; thus an en bloc resection with the need of massive bone graft and endoprosthesis is the best option in view of salvaging the shoulder joint and reducing the rate of local recurrences.

The patient was then referred to HUSM Kubang Kerian for further assessment and management.

MANAGEMENT

Surgical Management of Shoulder Girdle Tumors (Roger Dee)

A. Tikhoff Linberg Resection.

The Tikhoff Linberg resection is a limb sparing procedure for sarcomas arising around the shoulder girdle. The resection consist of en bloc removal of the scapula, clavicle and proximal humerus with preservation of a functional arm and hand.

Indication for these procedures are low and high grade sarcomas of the scapula and peri and suprascapular soft tissue sarcomas. Modified Tikhoff Linberg resection involves resection of a large portion of the humerus with preservation of a portion of the scapula. Absolute contraindication to the resection is tumor involvement of the neurovascular bundle and / or chest wall; in both types of involvement require a forequarter amputation. Relative contraindications include pathologic fracture, poorly planned biopsy with widespread tumor contamination, and lymph node involvement.

Type of Tikhoff Linberg Resection:

Type Iintra - articular proximal humeral resection

Type IIpartial scapulectomy

Type IIIintra - articular total scapulectomy

Type IVextra - articular scapula and humeral head resection

Type Vextra - articular humeral and glenoid resection

Type VIextra - articular humeral and total scapula resection

The goal of this procedure is to stabilize the remaining arm so that elbow flexion can position the hand in respect to the body. Functional result following this procedure is similar as those following a total scapulectomy. No active abduction of shoulder is possible; however retains useful hand and good elbow function. The shoulder is stable without any external orthosis.

B. Total scapulectomy

It is indicated in tumors involving body of scapula without involvement of the suprascapular region or glenoid. It is not recommended for tumors extending anteriorly or laterally or of involvement of the glenoid or rotator cuff. It is contraindicated when there is neurovascular or chest wall involvement. Overall function following total scapulectomy consists of a stable shoulder that allows for useful positioning of the hand via elbow flexion.

C.Partial scapulectomy

It involves removal of scapular body with preservation of scapula spine. This procedure is indicated for low-grade lesion involving only the body of scapula. Functional results after partial scapulectomy are superior to those that follow total scapulectomy.

D.Forequarter amputation

This radical procedure involves removing the entire upper limb including scapula and clavicle. The main indications are high grade tumor involving the shoulder girdle and proximal humerus. The plane of dissection is between the scapula and clavicle and the chest wall.

INTRODUCTION

Benign locally aggressive bone tumours include giant cell tumor, aneurysmal bone cyst, chondromyxoidfibroma and osteoblastoma. While their clinical behavior is essentially benign these tumor in general and giant cell tumor in particular may invade and destroy local structures.

CT is a primary bone neoplasm characterized by varying numbers of multinucleated giant cells dispersed in a stroma of round, oval or spindle-shaped mononuclear cells that fuse to form the giant cells of the lesion (Eckardt, 1986). The lesion usually behaves in a benign manner but has the tendency for local recurrence both in bone and in surrounding soft tissues and it may transform into a High-grade sarcoma (Campannacci, 1993; Carrasco,1989). True giant cell tumor however should no longer be considered innocent growths, on the contrary, they represent a progressive, potentially malignant process that can recur in about 20 40% of patients, undergo sarcomatous transformation in about 5 10%, even produce metastases without apparent previous malignant changes (Gitelis S, 1994)

EPIDEMIOLOGY

The incidence of true giant cell tumors represents 8.6% all bone tumors. The incidence in female was greater than in male by ratio of 1.5 to 1. Giant Cell Tumor (GCT) frequently occurs in proximity to major weight bearing joints. In most reported series of giant cell tumor, almost half of the neoplasm involve the distal femur or proximal tibia. They frequently cause significant bone destruction, placing the joint at greater risk for pathological fracture (Gitelis S, 1994). The majority of GCT occurs in patients between 20 and 40 years old and in the long tubular bones, particularly in proximity to the epiphysis (Sung et al, 1975).

LOCATION

GCT is always a monostotic process that occurs in the end of the long bones. The most common sites of involvement are the distal femur, the proximal tibia and the distal radius. Less frequent locations are, in order, distal tibia, proximal humerus, proximal femur, proximal fibula and distal humerus (Campanacci, 1993; Carrasco, 1989). Amongst the flat and short bones, GCT most frequent occurs in the sacrum followed by the vertebrae, the pelvis and the carpal bones. In the vertebrae, the tumor primarily involves the vertebral body. Patients with a primary GCT of the bones of the carpal phalanges has an 18% incidence of multicenteric disease. Very rare (about 0.5%) GCT is multicenteric in the skeleton (Campancci, 1993; Eckardt, 1986). Almost all GCT were located at the epiphyseal end of a long tubular bone in a skeletally mature patient, most frequently abutting the articular cartilage and extending towards the metaphysis.

CLINICAL FEATURES

In the most patients, pain was the first symptom. In the more superficial bones or more expanded tumors, swelling, tenderness and increase local temperature become apparent. Joint effusion and limited joint motion are signs of advanced destructive lesion. Pathologic fracture with haemarthrosis is not uncommon (about 10%) (Campanacci, 1993; Carrasco, 1989). In the spine and sacrum, nerve root involvement may cause intense radiating pain and neurological deficit.

Pathology

Gross specimen shows extensive soft fleshy areas that alternate in color from gray to a light red or dark haemorrhagic hue. The tumor is situated eccentrically in the epiphysis extending toward the articular cartilage and toward the metaphysis (Gitelis S, 1994).

The overlying cortex has usually undergone resorption and contour of the bone is expanded by the tumor. The metaphyseal aspect of the tumor is usually outlined by thin shell of fibrous tissue or reactive bone (Carrasco, and Murray, 1989).

Perforation of the thinned and widely expanded cortex of occurs and does not indicate that the tumor is malignant. The margins of the tumor within the metaphyseal spongy tissue are not well defined; there is no delimiting fibrous or bone membrane. This explains the frequency of recurrences after curettage (Gitelis S, 1994).

Microscopically, the tumor is composed of a moderately vascularized network of round, oval or spindle-shaped stromal cells and multinucleated giant cells (Carrasco, 1989).

Staging

Campanacci et al (1975) and Enneking have recently developed similar staging systems for GCT based on combined radiographic presentation and histologic grading system of increasing stromal atypia (Eskardt, 1986). Campanaccis radiographic grade 1, 2, and 3 correspond to Ennekings surgical stages 1, 2, and 3, which represent the latest, active and aggrassive clinical presentations. The only difference between them is that Campanaccis grade 3 is a malignant sarcoma that most frequently is found following radiotherapy of a conventional GCT. Ennekings histologic grade 3 has increased stroma atypia, but is still considered non malignant.

Campanacci classification of GCT

Stage 1

These accounts for about 10% of the cases. Clinically, the symptoms are minimal or absent and the course is indolent. Radiograph shows a thinned but preserved cortex, the osteolysis has well defined limits with a thin capsule of mature bone.

The isotope uptake is moderate and limited to lesion, and angiography show scarce or no hypervascularity. The histology is grade 1 or 2, sometime with vast area of necrosis and scarring fibrosis.

Stage 2 (active)

This stage is most frequent (70%). The symptoms are definite and the course is steadily but not rapidly progressive. The imaging shows an osteolysis with precise but not well defined limits, the original cortex is destroyed, but the tumor is contained by a thin rim of reactive bone. The isotope intake is increased in the lesion and about its margin. There is a considerable hypervascularity. The histological grade is usually 2, rarely 1.

Stage 3 (aggressive)

This stage amount to 20% in primary GCT but is more frequent in local recurrence. Clinically, the symptoms are those of an expanded, aggressive lesion, whose course is rapidly progressive. There is a high incidence of pathological fracture. Radiograph shows an extensive osteolysis with ill defined limits, distruption of the cortex, bulging of the tumor into the soft tissue without containment by a reactive bone rim. Isotope uptake is well beyond the limits of the tumor. There is intense tumoral and peritumoral hypervascularity. The histology is grade 2.

The staging system is helpful in planning the initial surgical approach to any specific lesion; a more aggressive approach is required for the more aggressive lesion.

Radiograph

The most common appearance in a long bone was a radiolucent expanding zone, located eccentrically in the epiphyseal end, generally in a skeletally mature patient.

The lesion extended both toward the articular cartilage and metaphyseal region and occupied in more advanced case the entire width of the bone (Gitelis S, 1994). Bony ridges at the periphery of a lobulated tumor will give the radiographic appearance of trabeculations with the tumor. There is no mineralized tumor matrix. Although the radiological appearance is almost diagnostic, a histologic diagnosis is required since other also produces some radiographic features lesions such as malignant fibrous histiocytoma and osteosarcoma (Carrasco and Murray, 1989).

Bone scan

GCT, as most other skeletal neoplasms, produced increased uptake of technetium 99 m, which is the most frequently, used bone-scanning agents.

Angiography

Angiography was used prior to the advent of computed tomography to determine the tumors extraosseous extent and its relationship to major vessels. At present, angiography is seldom used as a diagnostic modality and then only to determine the relationship of very large tumors to major vessels (Carrasco and Murray, 1989).

CT scan

CT scan does not allow easy distinction between tumor and muscle because of their similar attenuation coefficients. The axial slices provided by computed tomography does not allow accurate evaluation of the subartricular cortex because of volume overaging. In addition, reactive changes and edema on other cortical surface or the synovium may mimic tumor extension.

Magnetic Resonance Imaging

The best imaging modality of GCT is MRI, because of its superior contrast resolution. GCT, like most skeletal neoplasms, exhibit long T1 and T2 relaxation times, which translate into signals of low intensity on T1 and high intensity on T2 weighted images relative to the bone marrow. Therefore, the intramedullary tumor is best-seen on T1 weighted images, while its extraosseous portion is best appreciated on T2 weighted images (Brady et al, 1982, Herman et al, 1987).

TREATMENT

Giant cell tumors, which are locally aggressive, are difficult because of its inherent ability to be invasive and extensive difficulty in gaining local control. However, it is the main aim of surgical treatment to completely eradicate the tumor whilst preserving normal bone architecture and joint function.

The standard treatment of GCT have been intralesional excision with curettage and autrograft reconstruction of excised tumor cavity with cortical iliac graft and cancellous bone. Intralesional excision however leaves microscopic tumor cells regardless how carefully or thoroughly performed. Carrasco and Murray (1989) had reported of 60% chance of relapse using the above method.

Alternative methods had been developed to reduce local recurrence rate. These include:

Cryotherapy using liquid nitrogen

Sung et al (1975) had described that cryosurgery offers improvement in result when used with curettage and it reduces the necessity of resection and amputation. Devitt et al (1996) had proven the clinical efficacy of the cytotoxic and tumoricidals effect of liquid nitrogen.

The biological effects include thermal shock, dehydration of cells, intracellular ice crystal formation, membrane distruption, denaturation of protein in cells and microvascular failure. However, its use for malignant bone tumors is controversial and should be considered only for carefully selected cases.

The advantage of cryosurgery include:

i. High cure rate

ii. Preservation of adjacent joint

iii. Avoid extensive reconstruction using prosthetic replacement allograft or arthrodesis.

The disadvantage of cryosurgery however include:

i. 5-10% wound problems etc, poor healing

ii. Pathological fracture

Incidence up to 28% have been reported (Marcone et al, 1973)

Phenol irrigation of curreted cavity

Eckardt and Grogan (1986) had shown the reduction of recurrence rate of 260 cases of GCT from 50% to 25% when the lesions were located at the ends of selected long bones.

The technique consist of wide exteriorization of the lesion and vigorous curettage to grossly normal cancellous and cortical bone. The soft tissues are then packed with vaseline gauze and the cavity is then filled with liquid phenol for a period of 30-45 seconds.

Phenol acts by coagulating all proteinaceous substances, thus removing any uncuretted tumor residua. After removal of phenol, the cavity is rinsed with 95% alcohol to dilute the previously installed phenol. Saline lavage is then used to remove any alcohol residua and the cavity is then packed with cancellous autografts.

The advantage of phenol cauterization is, the rarely requires cast immobilization and can achieve a normal range of movement in a short period of time with early physical therapy and initial protected weight bearing.

Methylmethacrylate pack

This method using had been more widely accepted to pack the tumor bed following curettage. Its act by generating local hyperthermic which induces necrosis of any remaining tumor cells, yet it does not extend to normal tissues to give rise to any local complication (Carrasco and Murray, 1989).

Polymethylmethacrylate (PMMA) gives early mechanical support to the tumor cavity and this gives immediate stabilization to allow early physical activity and permit an early return of function.

PMMA have been left in place as long as 9 years without adverse effect with low recurrence rates. The surgeon has the option of removing PMMA and replace it with bone graft in a later date when there is no evidence recurrence.

If the tumor cavity is big and harvesting the autograft would give significant donor morbidity, the combination of autograft on the subchondral surface combined with allograft to fill the less critical area of the cavity must be considered.

If the patient is treated with PMMA earlier to fill the cavity, the surgeon should be certain that no local relapse is going to develop before the technique of autograft on the subchondral bone supplemented with allograft is used.

Other option of replacing a large osteoartricular bony defect would be custom endoprosthetic replacement. Although it is usually reserved for aggressive tumors as part of limb salvage procedures, in selected stage 3 lesion, these method should be seriously considered.

Radiotherapy as the primary or adjuvant treatment of GCT have been condemned because its ability to develop sarcomatous change in the tumor. Dahlin et al (1970) reported incidence of 19% of sarcomatous change following supplemental radiotherapy. Surgical stage 3 accounts only 10-15% of all GCT, however it gives problem in definitive surgical treatment options. A more aggressive surgical approach is indicated in these cases.

En bloc resection of GCT with a portion of normal bone and soft tissue have shown promising results. Campanacci et al (1975) reported the recurrence rate after curettage was 58% as compared to 13% when en bloc resection was done. Sung et al (1975) had shown similar finding with recurrence rate of 41% following curettage as compared to 7.1% after en bloc resection. En bloc resection can routinely assure good result in GCT occurring in expandable bone e.g. Iliac wing, a rib or the scapula.

An aggressive stage 3 GCT occurring at any major joints, needs special consideration. The aim is basically reconstruction of the joint following intralesional excision. An ideal reconstructive material using bone graft or cement must be chosen so that it will restore maximum stability to the articular surface at the earliest moment to allow movement.

In case on small tumor cavity, autograft offers the best option. The uses of allograft are the second option considering that it is indolent in its revascularization.

Local recurrence of GCT signifies incomplete removal of tumor cells from the cavity bed. ODonnel et al (1994) had shown an overall recurrence rate of 25% in curettage and packing of cavity with PMMA. Higher recurrence rate were also noted who had pathological fracture, stage 3 Campanacci classification and those who had not had adjuvant treatment with phenol.

The main aim of treatment to remove the tumor while maintaining the remaining bone function. In radiographic evidence of small recurrence, a repeat curettage and grafting would be the best reasonable option. However, in recurrence which presents with pathological fracture or extensive soft tissue involvement, en bloc resection and osteoartricular reconstruction would be indicated (Eckardt and Grogan, 1986).

By reviewing the natural history of the disease, post-operative examination of GCT patient is important for at least 5 years. In the initial 2 years, chest radiograph and the involved limb should be reviewed. CT scans of the affected area every 6 months to look for recurrence. The earlier the local recurrence is identified, the more the expeditious can be its treatment, and the greater chance to preserve limb function.

SUMMARY

GCT of bone is a benign but locally aggressive tumor with a tendency for local recurrence. Campanaccis classification is helpful in outlining the best treatment options. The ideal aim of surgical management is complete removal of macroscopic tumor while preserving the joint.

Curettage with adjuvant chemical or thermal cauterization and with bone graft or cement instillation gives an option of preserving crucial areas of the bone and allows early movement. In aggressive tumors, en bloc resection with joint reconstruction using autograft, allograft or custom arthroplasty is should be considered.

Radiotherapy is be reserved of areas which are not surgically resectable e.g. spine, though risk of sarcomatous change have been proven.

REFERENCES

1. Brady TJ, Gebhardt MC, Pykett IL: NMR imaging of forearms in healthy volunteers and patients with giant cell tumor of bone. Radiology, 144: 549-552, 1982.

2. Campanacci M, Gunti A and Olmi R: A study of 209 cases with long term follow up in130, Ital-J.Orthop. Traumatol 1: 249, 1975.

3. Campanacci M: Giant cell tumors of bone. Current orthop. 7: 26-35, 1993.

4. Carrasco CH. Murray JA: Giant cell tumors. Clin. Orthop. 20(3), 395-405,1989.

5. Dahlin DC.: Giant cell tumor. A study of 195 cases. Cancer, 25: 1061, 1970.

6. Devitt A, OSullivan T, Kavanagh M, Hurson BJ: Surgery for locally aggressive bone tumors. Irish 165(4); pp278-281, 1996

7. Eckardt JJ, Gragon TJ: Giant cell tumor of bone. Clin. Orthop. (204): 45-57, March 1986.

8. Gitelis S.: Tumors and tumor like lesions of bone: Pathology, radiology and treatment. 2nd edition, Springer-Verlag publication, Ch III; pp257-300, 1994

9. Marcone RC, Lyden JP, Huvos AG.: Giant cell tumors treated by cryosurgery: A report of twenty-five cases. J.Bone Joint Surgery (A) 55A; 1633,1973

10. ODonnell RJ, Springfield DS, Motwani HK, Ready JE, Gebhardt MC, Mankin HJ.: Recurrence of giant cell tumors of the long bones after curettage and packing with cement. J Bone Joint Surgery 76(A); 1827-1833, 1994.

11. Sung HW, Kuo DP, Shu WP, Chai YB, Liu CC and Li SM.: Giant cell tumor of bone: Analysis of 208 cases in Chinese patients. J Bone Joint Surgery 57A; 865-871, 1975.

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