Epithelioid sarcoma of the upper extremity with cerebral metastases.

AJ Rege, RS Dhir, V Pradeep 
 SDB Orthopaedic Centre and Research Institute, KEM Hospital, Parel, Bombay, Maharashtra.

Correspondence Address:
A J Rege
SDB Orthopaedic Centre and Research Institute, KEM Hospital, Parel, Bombay, Maharashtra.

Full Text

::   Introduction

Epithelioid sarcoma is a rare but distinctive type of soft tissue neoplasm of uncertain histiogenesis. It commonly presents with benign appearing, slow growing nodules, which have tendency to ulcerate and recur after excision. Distant metastases usually occur quite late in the course.

::   Case report

A forty-year-old male was admitted on 10th May 1991 with a grotesque deformity of the right hand. Four years ago the patient had swelling of the middle finger, which was treated by incision and drainage at a village dispensary. As the wound failed to heal despite dressings and antibiotics the finger was later amputed. Despite this, the swelling increased to involve the whole hand and part of the forearm.

When the patient presented to us, the hand was swollen and deformed and the fingers were fusiform in shape (See [Figure:1]). The nails were pale and brittle. There were multiple irregular ulcers which had crater like punched out edges and raised margins. The floor was covered with slough, which bled on touch. The base of the ulcers was indurated and fixed to the deep tissues. There were nodules from 0.5 cm to 3 cm in size with areas of central necrosis. Small joints of hand, wrist and elbow were stiff. Sensations of the hand were blunted. The axillary lymph glands were enlarged, discrete and the mass measured 6 cms x 4 cms. They were firm, mildly tender, mobile with intact overlying skin. With these findings a clinical diagnosis of epithelioid sarcoma was made.

Radiological examination of the forearm and hand showed generalised rarefaction of bone with diffuse periosteal reaction. At places there were areas of cortical erosion and attempts at new bone formation. Swab cultures from the floor of ulcers demonstrated presence of pseudomonas organisms.

Biopsy specimen from one of the nodules appeared grayish white and firm with areas of haemorrhage and necrosis. On histopathology [Figure:2] the tumour was composed of large, plump, polygonal cells with moderate to abundant eosinophilic cytoplasm, slightly indistinct cell borders and large bizarre hyperchromatic nuclei. Spindle cells and multinucleated giant cells were occasionally present with chronic inflammatory cell inflitrates. Diagnosis of epithelioid sarcoma was confirmed.

On 19th May 1991 a below-elbow amputation was performed 5cms proximal to the tumour margin. However, the axiiiary mass was not disturbed. Post-operatively patient failed to regain consciousness. Tracheostomy was performed for stridor. Till the 3rd day patient responded only to the painful stimulus. On the 5th day, he developed left sided hemiparesis with evidence of cerebral metastases. He was weaned off tracheostomy on the 9th day. On the 12th post-operative day, the patient had severe bout of haemoptysis and expired.

On autopsy examination, there were enlarged (7cm x 4cm x 13cm) group of axillary lymph nodes, with a grayish white, homogenous cut surface. The wound at amputation site was not disrupted. The distant metastases were represented by multiple pleural plaques, three small grayish-white nodules in right kidney and six small metastatic nodules in cerebral hemisphere. Lungs showed foci of intrapulmonary haemorrhages.

Microscopic examination of lymph nodes showed replacement of iymphoid tissue by the tumour with a predominant spindle cell component, few areas of necrosis and marked desmoplasia. Microscopic examination of tumour tissue and metastases was same as described before.

::   Discussion

The first description of epithelioid sarcoma as a clinicopathological entity was provided in 1961[1] but it received widespread recognition as a distinctive tumour after Enzinger's report[2] of 62 cases in 1970.

The tumour is most prevalent in young adults between the age of 20 and 40. It is twice more common in males than females[1]. The commonest sites of origin are hand and feet [3], though it has also been reported to occur in head, neck and trunk

The tumour commonly presents with single or multiple nodules of hard consistency described as 'wart' or 'knot'[4]. These tend to involve dermis, subcutaneous tissues, fascia and tendons. Diagnostic delay is a disturbing feature of this tumour and ranges from 2 to 28, 1-5 years[1][2],[3],[4],[5].

Roentgenogram reveals mostly a soft tissue mass with occasional areas of calcification. Underlying bone infrequently shows periosteal reaction and cortical thinning[6]. Gallium scan is useful to determine bone and soft tissue involvement[7].

Histopathology reveals oval to polyhedral cells with densly eosinophilic cytoplasm. These tumours have to be differentiated from infective granulomas, rheumatoid nodules, fibromatoses, fibrous histoocytoma, synovial sarcoma and squamous cell carcinoma[5].

Three factors determine prognosis of the tumour namely recurrence, vascular invasion and metastases to the regional lymph nodes[7]. Metastases may also occur in lungs, heart, pleura, liver, pericardium and bone[8]. Metastatic lesions have less cell differentiation but extensive necrosis.

Cure may be achieved by amputation or wide en bloc excision in early stages. Chemotherapy and radiotherapy are of questionable value[3]. Recurrence rate is as high as 85% after excision. However, due to slow growth and late metastases to distant organs the patient survives many years after diagnosis.

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