4例腺泡状软组织肉瘤临床病理分析及文献复习

Clinicopathological characteristics of 4 cases of alveolar soft-part sarcoma and literature review

目的:探讨腺泡状软组织肉瘤(alveolar soft-part sarcoma,ASPS)的临床病理学特征及其鉴别诊断。方法:对4例ASPS的临床资料进行回顾性分析,对标本进行组织病理学观察及免疫组织化学(免疫组化)研究。结果:患者3例为男性,年龄分别为30,25,27岁;1例为女性,34岁。发病部位4例均位于下肢深部软组织内。镜下肿瘤细胞排列成腺泡状或实性,细胞巢间可见窦状血管分隔,肿瘤细胞胞质丰富嗜酸,胞质内可见棒状结晶体。免疫组化:4例均TFE3阳性,3例MyoD1胞质阳性。1例患者随访15年后复发伴肺转移死亡,3例患者随访6个月无瘤存活。结论:ASPS是一种罕见的恶性肿瘤,青少年多见,结合临床、病理学特征及免疫组化,可做出正确诊断。鉴别诊断需除外腺泡状横纹肌肉瘤等血窦丰富的肿瘤,TFE3是该肿瘤的特异性标志物。

Objective： To investigate the morphologic features of alveolar soft-part sarcoma （ASPS） and its differential diagnosis. Methods： The clinical data were retrospectively analyzed in 4 cases of ASPS. Histopathological and immunohistochemical changes of the tumors were also observed in the paraffin-embedded tissue samples. Results： Three cases were male, 30, 25 anc 27 years old respectively; 1 case was female, 34 years old. The lesions were mainly located in the deep soft tissues of legs. Microscopically, tumor cells with granular cytoplasm arranged in alveolar or solid structures, and were separated by sinusoidal vessels. Needle-like crystals were detected in the cytoplasm of tumor cells. Immunohistochemically, 4 cases were positive for TFE3 and 3 cases were positive for MyoD1. One patient died with recurrence and pulmonary metastasis after 15 years, three patients were followed up for 6 months without neoplasm survival. Conclusion： Alveolar soft part sarcoma is a rare malignant neoplasm in young adults. By combining with clinicopathological features and immunohistochemistry, excluding alveolar rhabdomyosarcoma and some adenocarcinomas as differential diagnosis, a correct pathological diagnosis can be made. TFE3 antibody is a useful marker in the pathologic diagnosis of ASPS.