Epithelioid sarcoma: an analysis of 22 cases indicating the prognostic significance of vascular invasion and regional lymph node metastasis

Abstract

Twenty-two cases of epithelioid sarcoma seen and/or treated at Memorial Hospital are presented. With the exception of one tumor which occurred in the neck, all the sarcomas arose in the upper (15 cases) or lower extremities (6 cases). Ten originated in the hand. At the time of initial treatment, 14/22 patients were between 20 and 40 years of age. The tumor most commonly presented as a nodular lesion and involved dermis, subcutaneous tissue, fascia and tendons, and when it recurred (13 cases) it tended to grow proximally along tendons and fascial planes. Of special interest was the finding of vascular invasion in surgical specimens removed from 5 patients. Four of these represented recurrent tumor, and in two cases the vascular invasion was massive. Two of these patients also demonstrated tumor emboli in pulmonary vessels and cardiac involvement at autopsy. Metastasis to regional lymph nodes occurred in eight cases (42%). Follow-up ranging from 2 to 34 years (average of 8 years) was obtained for 19 cases (86%). Eleven patients are alive with disease or died as a result of their sarcoma (58% of the follow-up group). Adverse prognostic factors included recurrence after initial local excision, vascular invasion and lymph node metastasis. All of the patients with intravenous extension of tumor and 6 of 8 patients with lymph node involvement died with pulmonary metastases. Our data suggest that cure may best be achieved by amputation or by an exceptionally wide en bloc excision as early as possible, depending upon the location and extent of the original tumor.