文章摘要

4例腺泡状软组织肉瘤临床病理分析及文献复习

作者: 1,1,1姜乃光, 1朱安超, 1黄英
1 哈尔滨市第一医院病理科,哈尔滨 150010
通讯: 姜乃光 Email: 360888194@qq.com
蒋萍 Email: jiangping0513@hotmail.com
徐明芬 Email: 935872399@qq.com
朱安超 Email: zhu-anchao@163.com
黄英 Email: 1774312866@qq.com
DOI: 10.3978/j.issn.2095-6959.2014.04.029

摘要

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。
关键词: 腺泡状软组织肉瘤;免疫组织化学;临床病理特点

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

Authors: 1,1,1JIANG Ping, 1ZHU Anchao, 1HUANG Ying
1 Department of Pathology, First hospital of Harbin, Harbin 150010, China

CorrespondingAuthor: JIANG Naiguang Email: 360888194@qq.com

DOI: 10.3978/j.issn.2095-6959.2014.04.029

Abstract

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 and 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.

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