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|Year : 1987 | Volume
| Issue : 3 | Page : 140-2
Epithelioid sarcoma of the upper extremity (a case report).
Bhama BA, Dhir RS, Pandit AA
|How to cite this article:|
Bhama B A, Dhir R S, Pandit A A. Epithelioid sarcoma of the upper extremity (a case report). J Postgrad Med 1987;33:140
Epitheloid sarcoma is a clinical curiosity with uncertain histogenesis.,, It is slow growing and is often mistaken in early stages for benign affection due to its harmless appearance. One such case was under our treatment at the K.E.M. Hospital and it forms the basis of this report.
A forty year old farmer from U.P. was admitted to the S.D.B. Orthopaedic Centre and Research Institute, K.E.M Hospital on 10th April 1985 with severe deformity, and multiple large ulcers distributed all over the right upper extremity. His illness had started four and a half years ago with appearance of a longitudinal painless swelling in the right palm which apparently was noticed after a minor injury with a stick. Over the months, the swelling progressed and new swellings developed to involve the flexor aspect of the forearm. The swelling was diagnosed at a local hospital, as a compound palmar ganglion and antituberculous treatment was given for nearly one and half years. Despite treatment, the swelling increased in size and even ulcerated at places. Subsequently, the swellings were locally excised twice at the interval of six months. But, each time the swelling recurred and progressed, further causing limitation of movements of the joints of the affected extremity. After nearly four years, he also developed some nodular patches over the arm and swelling in the axilla, and then he presented at our department.
On clinical examination, the patient was found to be moderately built and in a good general health. His right upper extremity was severely deformed and supported by his left hand. His right elbow and the wrist, both were in 90° flexion and the forearm was in full pronation. The fingers were completely extended and the thumb was adducted reducing the transverse diameter of the palm. There were large ulcerated areas over the palm, the flexor aspect of the forearm and the axilla [Fig. 1]. Skin adjacent to the ulcers was hypopigmented. Ulcerations were irregular, crater like in appearance, with punched out and raised edges. Their sizes roughly measured 6 cm x 4 cm in the palm, 24 cm x 10 cm in the forearm and 5 cm x 4 cm in the axilla. Their bases were firm and irregular and were covered with pink granulation tissue. Their margins were irregular, nodular and woody hard in consistency. The tumour masses were fixed to the deeper tissue and the bones of the forearm and the hand. However, he axillary mass was only fixed to the pectoralis major muscle. There were several irregular hypopigmented, variegated, nodular masses on the extensor and the flexor surfaces of the arm. They had central areas of necrosis and ulceration, laving thin watery discharge. They were all skin deep and not fixed to the deeper tissues. Active movements of the fingers and wrist were not possible; however, elbow flexion was only 30° from the position of fixed flexion deformity of 70°, and shoulder abduction was upto 120°. The external rotation of the shoulder was severely restricted. Sensation to all the fingers were preserved though blunted. Examination of other three extremities and other parts of body showed no abnormality. All the routine laboratory investigations at this stage were within normal limits except that the eosinophil count in the peripheral blood smear was 10% and the E.S.R at the end of the first hour was 62 mm. Repeated cultures and swabs taken from the base of ulcers showed only the colonies of Pseudomonas and Proteus. At no time acid fast bacillus was isolated from the wound. Roentgenogram of the forearm and the hand [Fig. 2] showed demineralization of bones. There was evidence of peri-osteal reaction and new bone formation in the distal half of the radius and the ulna. Roentgenogram of the chest showed no abnormality.
Considering the involvement of large areas of skin and soft tissue along with affection of the regional lymph nodes, a clinical diagnosis of florid and resistant cutaneous tuberculosis was made. The patient was transferred for management to the Dermatology Department where in addition to regular dressing of the lesion, he was given different combinations of three drug therapy for nearly one year. During this period, the biopsy from the base of the ulcer had shown only granulation tissue, clumps of inflammatory cells and areas of necrosis. A typical tuberculous granuloma was never demonstrated. Repeated cultures from the ulcers were also of no help. Despite treatment, the lesions increased in size and extent, hence the patient was transferred back to the Department of Orthopaedic Surgery. Repeat biopsy was performed on 16th April 1986 and four large chunks were taken from the woody nodular margin of the ulcerated mass in the forearm. A diagnosis of epitlieloid sarcoma was made on histopathological examination Subsequent bone scan and repeat chest roentgenogram showed no metastasis. The right upper extremity was finally amputated on 10-6-1986 at the level of surgical neck of the humerus with wide excision of the axillary lymph nodes. The stump healed without any complications and the patient was discharged on 26-6-1986 with advice to follow up every three months.
On gross examination, the cut surfaces of the pieces taken for biopsy were glistening grayish white, mottled with yellowish and brown areas of necrosis and haemorrhage. They also showed predominantly nodular arrangement throughout. Overlying skin was freely mobile except adjacent to the ulcerated areas where the skin was firmly adherent.
On microscopic examination, [Fig. 3] there was a distinct nodular arrangement of the tumour mass. Nearly all the nodules had focal areas of necrosis interpersed with areas of haemorrhage. Tumour cells had also infiltrated the dermis and the epidermis adjacent to the site of ulceration. Constituted cellular elements were primarily epiheloid in nature. Their size and shape varied from large ovoid or polygonal appearance to plump spindle cells having deeply eosinophilic cytoplasm. Aggregates of chronic inflammatory cells were also present in between the nodules.
It was only after Enzinger's report of sixty two cases in 1970 that epitheloid sarcoma received widespread recognition as a distinctive tumour type. The tumour is notorious for being misdiagnosed.,,,,, It is likely to be confused with a variety of benign and malignant conditions-especially a granulamatous process, a synovial sarcoma and an ulcerating squamous cell carcinoma. The tumour is most prevalent in adolescents and young adults between 15 and 35 years of age, although it can affect any age. Males are affected twice as commonly as females. Principle sites of affection are fingers, hand and forearm., The next in frequency is the lower extremity. The tumour is extremely rare in trunk, head and neck region. The tumour most commonly presents as a nodular lesion and involves the dermis, subcutaneous tissue; fascia and tendons and when it recurs, it tends to grow proximally along the tendons and fascial planes. When located in subcutis, it usually presents as a solitary or multiple firm nodules usually with a callus hard consistency and is often described as a 'woody hard knot' or a firm lump that is slow growing and painless. Nodules situated in the dermis are often elevated above the skin surface and frequently become ulcerated within a few weeks or months after their detection. Early lesions are often diagnosed as 'indurated ulcer', 'drainage abscess' or 'infected wart' that fails to heal despite intensive therapy. Deep seated lesions are usually firmly attached to tendons, fascial structure or even to the underlying bone. Roentgenogram reveals mostly a soft tissue mass with occasional local calcification and with infrequent cortical thinning and erosion of the underlying bones. Periosteal reaction may be seen.
A superficially located epitheloid tumour in early stages can be confused with infective granulomas, rheumatoid nodules, fibromatosis and fibrous histocytomas. Yet in nearly all cases, the individual tumours are more sharply defined than those of a granuloma, and the constituent cells are larger, more eosinophilic and less mature in appearance. Absence of biphasic pattern and pseudoglandular structure alongwith tendency to ulcerate early, separates it from synovial sarcoma, while complete absence of keratin pearls and dyskeratosis in the adjacent epithelium nearly always allows its differentiation from squamous cell carcinoma.
Epitheloid tumour is known to recur and metastasize even after wide excision. Prat et al reported a metastatic rate of 58% which keeps in confirmity with the findings by Bryan et al, whereas others,,, have reported metastatic rate between 30 and 40%. The tumour metastasizes through lymphatic and vascular invasion to regional lymph nodes, skin, lungs and soft tissue of the other parts of the body. Late metastasis even after excision of the tumour is known. The recurrence is usually noticed within a year but has been seen as late as twenty five years after excision.
Prat et al, observed three factors of prognostic importance: namely, recurrence, vascular invasion and metastasis to regional lymph nodes. Of these, recurrence appeared to be of least importance. Adequate treatment requires radical en bloc excision or amputation as early as possible along with extensive lymph node dissection. The role of radiotherapy and chemotherapy is uncertain. Being a slow growing tumour, the patient often survives for ten years or longer irrespective of the treatment given.
We are thankful to the Dean, Seth G. S. Medical College and K.E.M. Hospital, Bombay, for allowing us to use the hospital data.
|1.||Bryan, R. S., Soule, E. H., Dobyns, J. H., Prichard, D. J. and Linscheid, R. L.: Primary epitheloid sarcoma of the hand and forearm. A review of thirteen cases. J. Bone & Joint Surg., 56A: 458-465, 1974. |
|2.||Enzinger, F. M.: Epitheloid sarcoma; A sarcoma simulating a granuloma or a carcinoma. Cancer, 26: 1029-1041, 1970. |
|3.||Enzinger, F. M. and Weiss, S. W.: "Soft Tissue Tumours." The C. V. Mosby Company, New York, 1983. pp. 781-795. |
|4.||Heppenstall, R. B., Yvars, M. F. and Chung, S. M. K.: Epitheloid sarcoma: two case reports. J. Bone & Joint Surg., 54A: 802-806, 1972. |
|5.||Nelson, F. R. and Crawford, B. E.: Epitheloid sarcoma. A case report. J. Bone & Joint Surg., 54A: 798-801, 1972 |
|6.||Prat, J., Woodruff, J. M. and Marcove, R. C.: Epitheloid sarcoma. An analysis of twenty two cases indicating the prognostic significance of vescular invasion and regional lymph node metastasis: Cancer, 41:1472-1487, 1978. |