臀部软组织透明细胞肉瘤一例报告

Haunch soft tissue clear cell sarcoma:1 case report

透明细胞肉瘤（clear cell sarcoma，CCS）是一种罕见的软组织肿瘤，约占软组织肉瘤的1％。Enzinger于1965年首先报告，由于肿瘤细胞内存在黑色素，又被称为软组织恶性黑色素瘤。1978年Tsuneyoshi等进一步将CCS分为黑色素型和滑膜型。虽然其组织起源与皮肤恶性黑色素瘤相似，但其临床表现、组织形态、细胞遗传学特征和预后均与黑色素瘤不同，更趋向于软组织肉瘤。现报告1例发生于臀部的软组织的CCS。

Clear cell sarcoma （ CCS ） is an exceptional kind of soft connective tissue tumor, and tends to occur at the end of the limbs, especially the foot and ankle. CCS rarely occurs in the trunk and head. CCS grows slowly, with a long medical history. And the larger tttmors can result in pain and tenderness. The immunochemistry is positive for the markers of HMB45, S-100 and Vimentin. The primary treatment of CCS is surgical removal. The prognosis is mainly affected by the size of tumors and wide resection or not. The role of radiotherapy and chemotherapy is expected to be testified. Interferon and interleukin may play a role in reducing the recurrence and elevating the patient＇s overall survival rate. In general, the prognosis of CCS is relatively poor. In this article, a case of haunch soft tissue clear sarcoma is reported.