From the Departments of Pediatric Surgery, Pathology and Pediatrics, Post Graduate Institute of Medical Education and Research, Chandigarh 160 012, India.
Reprint requests: Dr. S.K. Chowdhary, Assistant Professor, Department of Pediatric Surgery, PGIMER, Chandigarh 160012, India.
E-mal: [email protected]

Manuscript Received: February 12, 1999;
Initial review completed: March 24, 1999;
Revision Accepted: September 22, 1999

Epitheloid sarcoma is a soft tissue tumor of young adults. A majority of them arise in the subcutaneous tissue or dermis of the upper extremity around the hand and forearm and start as a small nodule. They have a propensity to recur locally after excision and in a few cases may metastasise. Although this tumor has been reported in children, we have not come across description of this tumour in a neonate.

A one-month-old boy was brought with a lump in the right hand, which had gradually grown since birth (Fig. 1). This was a multi-nodular swelling measuring 8 ´ 10 cm arising from the palmar aspect of the right hand extending between the proximal palmar crease and web spaces. The overlying skin had characteristic neovascularisation and was fixed to underlying structures. The right axilla had a solitary lymph node 3 ´ 3cm, and there was, in addition a nodule in the epigastrium.

Fig. 1. Photograph demonstrating the tumor arising from the palmar aspect of the right hand with typical neovascularisation.

An incisional biopsy of the mass in the right hand revealed nodular arrangement of spindle and epitheloid cells with densely staining large nuclei (Fig. 2). Immunohistochemistry of the specimen revealed positive staining for vimentin and negative staining for desmin. Keratin staining could not be done. Aspiration cytology of the nodular lump in the axilla and epigas-trium revealed similar spindle and epitheloid cells.

Fig. 2. Histological section from the tumor showing epitheloid and spindle cells with large densely staining nuclei.

The parents were not in favour of surgery. Adjuvant chemotherapy was started with weekly doses of vincristine, cyclophosphamide and actinomycin D. However, the tumor continued to grow and three weeks later the baby died with febrile neutropenia.

Benign and malignant soft tissue tumors are not uncommon in children. Infantile fibro-matosis and fibrosarcoma together constitute more than two thirds of the neonatal solft tissue tumors(1). These tumors frequently start as nodule in the extremity and show characteristic neovascularisation. Around 75% of cases recur locally and upto 40% can metastasise with fatal results(2). These nodules are often mistaken as an inflammatory nodule resulting in a delay in treatment. The nomenclature of the tumor is derived from the histological appearance of epithelium like cells merging imperceptibly with spindle cells with minimal cytological pleomor-phism, prominent nuclei and characteristic necrosis in the center of nodular arrangement of cells. A peculiar variant of synovial sarcoma was described in literature characterized by polygonal and polyhedral cells bearing striking resemblance to epithelium long ago(3). The tumor tissue has striking acidophilic appearance due to staining characteristic of cytoplasm and dense mass of hyalinized collagen forming the stroma of the tumor. Calcification may be seen in the area of central necrosis in a few cases. Immuno histochemically, there is positivity for vimentin, keratin, epithelial membrane antigen, CD 34, and tissue polypeptide antigen. How-ever, the co-expression of vimentin and keratin is thought to be a characteristic of this tumor. The exact histogenesis of the tumor remains obscure, although it does demonstrate attempt at epithelial differentiation(4). The tumor is known to spread to noncontiguous areas of the skin, soft tissue, fascia and bone as well as by direct extension. A more aggressive clinical course is associated with a proximal or axial location, increased size and depth, hemorrhage, increased mitotic figures, rhabdoid features and vascular invasion.

Gross et al. reviewed 8 children with epitheloid sarcoma, two of whom had meta-stases and both were dead within 1 year(5). de Vries earlier reported another series on children with epitheloid sarcoma with similar fatal result in 50% cases(6).

The highly malignant nature of the tumor is well documented. A high index of suspicion and early biopsy will promote early diagnosis. Local excision alone fails in a majority of the patients. Radical excision or limited amputation is the surgical treatment of choice. Adjuvant radiotherapy and chemotherapy have a limited role.

1. Stevens MCG. Neonatal tumors. Arch Dis Child 1988; 63: 1122-1125.

2. Chase DR, Enzinger FM. Epitheloid sarcoma: Diagnosis, prognostic indicators and treatment. Am J Surg Pathol 1985; 9: 241-263.

3. Enzinger FM. Epitheloid sarcoma: A sarcoma simulating a granuloma or a carcinoma. Cancer 1970; 26: 1029-1041.

4. Chase DR, Enzinger FM, Weiss SW, Langloss JM. Keratin in epitheloid sarcoma: An immuno-histochemical study. Am J Surg Pathol 1984; 8: 435-441.

5. Gross E, Bhaskar NR, Pappo A, Bowman L, Shearer P, Kaste P, et al. Epitheloid sarcoma in children. J Pediatr Surg 1996; 31: 1663-1665.

6. de Vries J, Hoekstra HJ, Oosterhuis JW, Postma A, Schraffordt Koops H. Epitheloid sarcoma in children and adolescents: A report of four cases. J Pediatr Surg 1989; 24: 186-188.