Epithelioid Sarcoma in Childhood: An Immunohistochemical, Electron Microscopic, and Clinicopathologic Study of 11 Cases Under 15 Years of Age and Review of the Literature

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Abstract
Epithelioid sarcoma in a rare tumor and most of the cases occur in young adults. It is rare in childhood. We have been able to obtain data and histologic material for 11 patients with this disease. The primary sites were head and neck in three patients, inguinal region in one, and extremities in seven. The age range of the patients was 12 weeks to 13 years. There was a preponderance of males over females with a ratio of 1.75. The tumors presented with a typical nodular necrotizing pattern. In three cases giant osteoclast-like cells were present. The immunohistochemistry and electron microscopy showed features consistent with previous observations on epithelioid sarcomas. In one case islands of small dark cells noted on light microscopy were surrounded by basal lamina on electron microscopy. The cells inside the nests were undifferentiated. Six tumors studied by flow cytometry were in DNA diploid range. On follow-up, five children are alive and well 2 to 7 years after treatment. Three children died of tumor progression with metastases to lymph nodes and lungs. One child had been diagnosed only recently, and in one the disease has run a protractive course with multiple recurrences. The behavior of these epithelioid sarcomas in children is similar to that seen in adults, the prognosis being dependent on radical tumor surgery preventing recurrent disease. Long-term follow-up is necessary because the tumor may recur many years after the primary tumor was removed.