Clear cell sarcoma: The Roswell Park experience

Background and Objectives: Clear cell sarcoma of the tendons and aponeurose s (CCSTA) is an aggressive, rare soft-tissue tumor with approximately 300 r eported cases. Although it appears to be histogenetically related to melano ma, its clinical behavior resembles soft tissue sarcoma with a propensity f or lymph node metastases. We report our experience at a tertiary cancer cen ter. Methods: Eight cases of CCSTA evaluated at Roswell Park Cancer Institute be tween 1970 and 1998 were reviewed retrospectively. Patient data analyzed in cluded patient age, gender, anatomic location, size of tumor, development o f local, regional and distant recurrence, and patient status at last follow up. Results: Six of eight patients were alive at 2 years, while three of seven patients were alive at 5 years. Of the patients alive with no evidence of r ecurrence, two had tumors of less than 2 cm, and the remaining patient had incomplete information regarding tumor size. Five patients recurred within 2 years of definitive surgical management. Four had tumors > 5 cm. All five patients progressed to metastatic disease at a median follow up of 20 mont hs (range 1-108 months) following definitive surgical management and all ev entually died of their disease at a median of 3 months (range 0-24 months) from presentation with metastatic disease. Four of five patients with lesio ns > 5 cm received adjuvant chemotherapy with intent 34, and 41 months from to cure, but all eventually died of disease at 4, 77 initial presentation. Conclusions: CCSTA is an aggressive tumor of the soft tissues. Early recogn ition and management are associated with an excellent long-term prognosis. Tumors greater than 5 cm warrant aggressive surgical management and treatme nt, and are at high risk of the development of distant disease. Aggressive multiagent chemotherapy appeared to have no impact on outcome. Other adjuva nt therapeutic options including immunotherapy should be investigated. (C) 2001 Wiley-Liss, Inc.