转移性腺泡状软组织肉瘤临床病理分析  

Clinicopathological analysis of metastatic alveolar soft part sarcoma

作　　者：张姗 李楠[1,2] 杨茹雪 杨盼盼 闫文天 杨宁宁 蔡兆根[1,2] 秦燕子 谷从友[1,2] 冯振中[1,2] Zhang Shan;Li Nan;Yang Ruxue;Yang Panpan;Yan Wentian;Yang Ningning;Cai Zhaogen;Qin Yanzi;Gu Congyou;Feng Zhenzhong(Department of Pathology,First Affiliated Hospital of Bengbu Medical College,Bengbu 233004;Department of Pathology,Bengbu Medical College,Bengbu 233030;Department of Pathology,the First Affiliated Hospital of Anhui Medical University,Hefei 230022)

机构地区：[1]蚌埠医学院第一附属医院病理科,蚌埠233004 [2]蚌埠医学院病理学教研室,蚌埠233030 [3]安徽医科大学第一附属医院病理科,合肥230022

出　　处：《中国组织化学与细胞化学杂志》2020年第1期46-51,共6页Chinese Journal of Histochemistry and Cytochemistry

基　　金：安徽省自然科学基金资助(1908085MH275);蚌埠医学院科技发展基金重点项目(BYKY201710)。

摘　　要：目的探讨转移性腺泡状软组织肉瘤(alveolar soft part sarcoma,ASPS)的临床病理学特征。方法回顾性分析8例转移性ASPS患者的临床病理特征、影像学表现及免疫组织化学表型,并复习相关文献。结果5例男性,3例女性,中位年龄47岁(范围21~70岁),5例肺转移(2例伴随颅内转移),1例空肠转移,1例肾转移,1例腹膜转移,临床症状无特殊表现。镜下见肿瘤细胞呈巢状生长,局部呈腺泡状排列,胞质红染或透亮,胞核呈圆形、卵圆形,肿瘤界限清楚。免疫组织化学表型:8例TFE-3均阳性,Syn、TTF-1、AE1/AE3、Myogenin、PAX-8、HMB-45、vimentin均阴性;其中6例PAS阳性,4例MyoD1阳性,3例desmin阳性,1例S-100阳性,Ki-67(10%~60%)。随访9~62个月,其中6例患者带瘤生存,2例无瘤生存。结论ASPS是一种罕见的软组织肉瘤,易转移至肺、骨、脑等,TFE-3对其诊断具有相对特异性,确诊需通过临床病史、影像学资料、组织学形态及免疫组织化学表型等综合分析。Objective To investigate the clinicopathological features of metastatic alveolar soft part sarcoma(ASPS). Methods The clinical and pathological features, imaging performance and immunohistochemical phenotypes of 8 patients with metastatic ASPS were retrospectively reviewed. Relevant literatures were reviewed. Results There were 5 males and 3 females with median age of 47 years(range 21-70 years old). Among them, 5 cases were with lung metastasis(2 cases with intracranial metastasis), 1 case with jejunal metastases, 1 case with renal metastasis and 1 case with peritoneal metastasis. They had no special clinical symptoms. Microscopically, the tumor cells showed nest-shaped growth, partially arranged in acinar, cytoplasm was red-stained or translucent, and the nuclei were round or oval, and the tumor boundaries were clear. Immunohistochemical phenotype: TFE-3 of 8 cases was positive. Syn, TTF-1, AE1/AE3, Myogenin, PAX-8, HMB-45, vimentin of 8 cases were negative;6 cases were PAS positive, 4 cases were MyoD1 positive, 3 cases were positive desmin and 1 case was S-100 positive. Ki-67 labeling index of 8 cases ranged from 10% to 60%. During the follow-up period of 9 to 62 months, 6 patients survived with tumor and 2 patients survived without tumor. Conclusion ASPS is a rare soft tissue sarcoma that is easy to metastasize to the lungs, bones, brain, etc. TFE-3 is relatively specific for its diagnosis. The diagnosis requires a comprehensive analysis of clinical history, imaging data, histological morphology and immunophenotype.

关 键 词：腺泡状软组织肉瘤 转移性 临床病理特征 TFE3 

分 类 号：R365[医药卫生—病理学]