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透明细胞肉瘤(CCS)是一种罕见的组织起源尚未最后确定的软组织肉瘤。多发生在青年人,生长缓慢但复发转移率高。肿瘤位于深部软组织,与肌腱和腱膜相连。多为结节状或分叶状。组织学特征为瘤细胞多角形或梭形,胞浆透明,核仁突出,组成大小不等的巢状,其周绕以宽窄不等的胶元纤维分隔。1965年Enzinger首次报道21例肌腱和腱膜的CCS,以后陆续有报道。除Chung和Enzinger报道AFIP搜集的141例以外,多系个案报告。本文根据这些报道,综述CCS的病理形态、生物学行为、组织发生和鉴别诊断等问题。
Clear cell sarcoma (CCS) is a rare tissue origin that has not yet been identified as a soft tissue sarcoma. Occurred in young people, slow growth but high recurrence rate. The tumor is located in deep soft tissue and is associated with tendons and aponeurosis. Mostly nodular or lobulated. Histologically, the tumor cells are polygonal or fusiform, transparent in the cytoplasm, prominent in nucleolus and composed of nests of unequal size, and their circumferences are separated by wide and narrow collagen fibers. Enzinger first reported 21 cases of tendon and decidual CCS in 1965 and was reported one after another. In addition to the 141 cases reported by Chung and Enzinger on AFIP, multiple department reports were reported. Based on these reports, this article reviews CCS pathological morphology, biological behavior, histogenesis and differential diagnosis.