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Case Reports

Indian Pediatrics 1999; 36:194-197 

Synovial Sarcoma of the Hand


M.M. Harjai
R.K. Bal
B.M. Nagpal
I.P. Sadhotra
K.K. Maudar

From the Department of Surgery, Armed Forces Medical College, Pune, India and Comamnd H.ospital, Southern Command, Pune, India.

Reprint requests: Lt. Col. .Man MohanHarjai, Reader, Department of Surgery, Armed Forces Medic. cal College, Pune-411 040, India.

Manuscript Received: April 22, 1998; Initial review completed: June 30, 1998;
Revision Accepted: August 20,1998.

The overall incidence of soft tissue sarcoma is approximately 5-7% of the malignant neoplasm's seen in children(l,2). Their rare presentation in distal portion of an extremity has limited the number of patients in most reports. Among' soft tissue tumors, synovial sarcoma is uncommon and occurs rarely in children. Till date only three cases of synovial sarcoma of the left palm has been reported in the English literature(3-5). Here we describe a fourth such case of this rare entity and its, management.

Case Report

A 6-year-old male child was admitted with complaints of gradually increasing swelling over the palm of left hand since 3 years. To start with the swelling was of a grain size and was noticed by the parents. Onset was spontaneous and initially the course was progressive, subsequently increasing rapidly to attain the present size. There was no history of pain. Examination revealed a small, 3 x 3 cm irregular swelling present over hypothenar region of left palm. It was firm, non tender and fixed (Fig. 1). No distal neurovascular deficit was detected. A single 2 x 1 cm soft, mobile and nontender lymph node was. palpable in the left axilla which was biopsied and turned out to be reactive hyperplasia. Radiographs of hand and chest' were normal. Computed tomography scan of hand revealed no evidence of any bone erosion (Fig. 2). An incisional biopsy was done which was reported as synovial sarcoma with predominant epithelial components. The immuno
histochemistry profile of the tumor revealed cytokeratins (CK) strongly positive, vimentin and epithelial membrane antigen (EMA) positive and smooth muscle antigen (SMA) focally positive. Local wide excision with 2 cm margin clearance was carried out. The skin loss was made up with application of a split skin graft. Post operative recovery was un- eventful. Histopathological examination of the excised specimen confirmed the diagnosis of synovial sarcoma (epithelioid variant) with incomplete clearance of deeper aspect. Radio- therapy of 6600 cGy was given. The patient is under regular follow up.


 

Fig. 1 Photograph of left Palm showing growth in the hypothenar region.

 

Fig 2. Computed tomography scan of hand showing extent of the tumor with no bony erosion.


Discussion

Malignant soft tissue sarcoma of hand is rare in children. Most large series on hand tumors report findings from adult patients. The overwhelming majority of reported soft tissue tumors of hand and wrist have been benign. In contrast to the predominance of synovial sarcoma in adults, most cases of soft tissue sarcoma in children are rhabdomyosarcoma.

Synovial sarcoma rarely occurs before 10 years. The most common site is in the region of the knee and distal portion 'of the thigh, followed by foot and ankle. It is rarer in the hand. Till date only three cases of synovial sarcoma of the palmar aspect of the left hand are reported(3-5).

Growth of the synovial sarcoma is very slow. The time elapsed between the first symptom and diagnosis generally ranges from 2 to 4 years. In this case it was 3 years. Unlike most other nonrhabdomyosarcoma soft tissue tumors of hand, synovial sarcoma frequently spreads to regional lymph nodes. However, axillary lymph nodes were free of metastasis in our case.

The demonstration of some differentiation-associated antigens are of great value as an adjunct to routine histological appearance of synovial sarcoma. The immunohistochemical staining served to distinguish the monophasic fibrous synovial sarcoma from a malignant Triton tumor in a 9-year-old boy located on thenar prominence of the hand(6). The synovial sarcoma is composed of two morphologically distinct types of cells that form a characteristic biphasic pattern. The biphasic synovial sarcoma. includes epithelial cells with a surrounding spindle or fibrous component. The spindle cells stain positive for cytokeratin and epithelial membrane antigen (EMA). Vimentin is demonstrable in spindle cells but. absent in epithelial cells(7). Some cases of synovial sarcoma may demo strate immunostaining for S-100 protein.

Because of prominent location of the epitheloid sarcoma in the hand, it requires to be differentiated from synovial sarcoma. The epitheloid sarcoma show a distinct multi- nodular pattern with central necrosis of the tumor nodules and deep eosinophilia of tumor cells. There is lack of basal laminae ultra structurally. However, like synovial sarcoma the cells stain positively for immunokeratin.

The varied clinical presentation of synovial sarcoma of the hand may impede an early diagnosis. Furthermore, after the diagnosis has been confirmed, there is some difference of opinion as to the best method for staging these lesions and their treatment. The pro- posed current approach to the management of soft tissue sarcoma of hand includes a high index of suspicion that a mass in the hand. may be a sarcoma, local staging with use of magnetic resonance imaging, distant staging with examination of regional lymph nodes and a computed tomography scan of the chest(8). Wide local excision with at least a 2 cm margin is the treatment of choice. When this surgery is likely to result in significant functional disability then limited excision and adjuvant radiation is the alternative option. Amputation is the last option.

In our case we did wide local excision with sacrifice of both branches of the ulnar nerve. Since the deeper margins were positive histologically and malignancy was of high grade, radiotherapy (6600 cGy) was given. The child is under close follow up. The favorable prognostic variables in this case are small tumor size «5 cm diameter), a primary site in hand, a younger age and a predominant epitheloid pattern.

In summary, because of rarity of synovial
. sarcoma, there are no clear guidelines on their management. A functional limb saving attitude without compromising the principles of malignancy should be individualized.

 

 References


1. Miser JS, Izzok PA. Soft tissue sarcomas in childhood. Pediatr Clin North Am 1985; 32: 779-800.

2. Kransdorf MJ. Malignant soft tissue tumors in a large referral population: Distribution of di- agnoses by age, sex and location. Am J Roentgenol1995; 164: 129-134.

3. Gross E, Rao BN, Pappo AS, Michalkiewicz E, Hudson MM, Kaste SC, et al. Soft tissue sarcoma of the hand in children: Clinical outcome and management. J Pediatr Surg 1997; 32: 698- 702.

4. O'Connell JX, Browne WL, Gropper PT, Berean KW. Intraneural biphasic synovial sarcoma: An alternative "glandular" tumor of pe- ripheral nerve. Mod Patho11996; 9: 738-741.

5. Bouziani A, Khlil A, Zarrouk H, Kammoun N, Debbiche A, Doss N, et al. Correlation be
tween epitheloid sarcoma and poorly differentiated fibroblastic synovial sarcoma. Arch Anat Cytol Patho11991; 39: 34-37.

6. Schmidt D, Harms D, Leuschner I. Cytokeratin expression in malignant Triton tumor. Pathol Res Pract 1990; 186: 507-511.

7. Fisher C. Synovial sarcoma: Ultra structural and immunohistochemical features of epithelial differentiation in monophasic and biphasic tumors. Human Pathol 1986; 17: 996-1008.

8. Brien EW, Terak RM, Geer RJ, Cald well G, Brennan MF, Healey JH. Treatment of soft tissue sarcoma of the hand. J Bone Joint Surg 1995; 77: 564-571.

 

 

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