Journal List > J Korean Orthop Assoc > v.28(1) > 1113747

Lee, Lee, Baek, Lee, and Park: Extraskeletal Ewing's Sarcoma: Clinical Analysis of 5 Cases

Abstract

Extraskeletal Ewings sarcoma, which occurs mainly in paravertebral region, lower extremity and retroperitoneum, is a rare primary malignant soft tissue tumor that has the same pathological characteristics of skeletal Ewings sarcoma. Clear histopathological separation from other round cell sarcoma, such as primitive rhabdomyosarcoma, neuroblastoma and neuroepithelioma, is oftern difficult without immunohistochemical staining. We analysed clinical findings of five cases of extraskeletal Ewings sarcoma that had been experienced from January 1987 to October 1991. All of the cases were diagnosed with immunohistochemical staining, including PAS and vimentin, There were 3 females and 2 males, whose age was averaged as 19.8 years ranging from 1.8 to 36. Locations of the masses were proximal lower extremity in 2 cases, shoulder in one, pleural cavity in one, and submental triangle in one. Types of treatment were, wide excision with chemotheraphy and radiotheraphy in two patients and wide excision with chemotheraphy and radiotherapy in two patients and wide excision with chemotherapy and radiotherapy in two patients and wide excision with chemotherapy in two. Only biopsy was performed in one patient who was lost in follow-up. The duration of follow-up except lost one, was average 27.8 months, and two of them died of disease. Lung metastasis was found in 3 cases, and one of them who showed metastasis at first visit, had remission after preoperative chemotherapy.

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