CAJ | 학술논문

目的探讨腺泡状软组织肉瘤的临床病理学特征及其鉴别诊断.方法对21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研究.结果 21例腺泡状软组织肉瘤,男性11例,女性10例,发病年龄4～56岁,平均25.9岁,发病部位主要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,细胞巢之间可见窦状血管分隔,瘤细胞胞质内含丰富的嗜酸性颗粒.PAS染色,瘤细胞胞质内可见棒状结晶体,免疫组化: MyoD1 10例阳性,desmin 4例阳性,S-100 9例阳性,NSE 11例阳性,Vim 11例阳性, AE1/AE3、CK、EMA、SMA、MSA、Syn全部为阴性.结论 ASPS是多见于青少年和青年的罕见肿瘤,但多数早期出现血液转移,切除后易复发,最终预后欠佳,结合临床病理学特征及免疫组化,可作出正确诊断.
목적 탐토선포상연조직육류적림상병이학특정급기감별진단.방법 대21례ASPS적림상자료진행회고성분석,대표본진행조직병이학관찰급면역조직화학연구.결과 21례선포상연조직육류,남성11례,녀성10례,발병년령4～56세,평균25.9세,발병부위주요위우하지심부연조직내,경하종류세포배렬성선포상혹실성,세포소지간가견두상혈관분격,류세포포질내함봉부적기산성과립.PAS염색,류세포포질내가견봉상결정체,면역조화: MyoD1 10례양성,desmin 4례양성,S-100 9례양성,NSE 11례양성,Vim 11례양성, AE1/AE3、CK、EMA、SMA、MSA、Syn전부위음성.결론 ASPS시다견우청소년화청년적한견종류,단다수조기출현혈액전이,절제후역복발,최종예후흠가,결합림상병이학특정급면역조화,가작출정학진단.
Purpose To investigate the clinical and pathological features and differential diagnosis of alveolar soft part sarcoma (ASPS).Methods The clinical data were retrospectively analyzed in 21 cases of ASPS. Histopathological and immunohistochemical changes of the tumors were also observed in the paraffin-embedded tissue samples.Results In 21 cases, 11 were male and 10 females. Ages of the patients ranged from 4 to 56 years old,with mean 25.9 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. There existed crystals in the cytoplasm of tumor cells after PAS staining.Immunohistochemically,10 cases were positive for MyoD1, 4 positive for desmin,9 positive for S-100,11 positive for NSE, and 11 positive for Vim. All of ASPS were negative for AE1/AE3,CK, EMA, SMA, MSA and Syn.Conclusions ASPS is a rare malignant neoplasm in adolescents and young adults. It is prone to early hematogenous metastases and a high recurrence rate after conservative surgical excision. Therefore, the prognosis of ASPS is poor. Careful analysis of the clinicopathological features and immunohistochemisitry will be helpful to reach an accurate diagnosis.