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目的探讨宫颈腺泡状软组织肉瘤(ASPS)的临床病理特征、免疫表型、遗传学特点及诊断与鉴别诊断。方法对3例宫颈ASPS的临床表现、病理学形态、免疫组化等进行观察,并复习相关文献。结果 3例患者分别为23、13及25岁,均因异常阴道出血而就诊。肿瘤最大直径分别为2.5、3和2.8 cm。镜下肿瘤细胞排列成巢团样或器官样结构,间隔以纤细的纤维血管分割;部分肿瘤细胞团呈假腺样结构。肿瘤细胞PAS染色均可以发现耐淀粉酶消化的嗜酸性颗粒以及针状与杆状结晶体。免疫组化示3例瘤细胞TEF-3、NSE和vimentin均(+),其中2例PR局灶(+)。3例患者随访21～57个月,均无瘤存活。结论宫颈ASPS病理诊断依赖于特征性组织学表现、PAS和免疫组化染色特点以及特异性的细胞和分子遗传学改变。与软组织ASPS相比,宫颈ASPSs症状出现早、体积小,预后更好。
Objective To investigate the clinicopathological characteristics, immunophenotype, genetic characteristics and diagnosis and differential diagnosis of cervical acinar soft tissue sarcoma (ASPS). Methods Three cases of cervical ASPS clinical manifestations, pathological morphology, immunohistochemistry were observed, and review the relevant literature. Results The three patients were 23, 13 and 25 years old, respectively, due to abnormal vaginal bleeding and treatment. The maximum tumor diameter was 2.5, 3 and 2.8 cm respectively. Microscopically, the tumor cells were arranged in the form of nests or organ-like structures separated by slender fibrous blood vessels. Some of the tumor cell clusters were in the form of pseudostructure. PAS staining of tumor cells can be found resistant to amylase-digested amyloid granules and acicular and rod-shaped crystals. Immunohistochemistry showed TEF-3, NSE and vimentin in all 3 cases (+), of which 2 cases were PR (+). Three patients were followed up for 21 to 57 months, all without tumor survival. Conclusions The pathological diagnosis of cervical ASPS depends on the characteristic histological findings, PAS and immunohistochemical staining, and the specific cellular and molecular genetic changes. Compared with soft tissue ASPS, cervical ASPSs early symptoms, small size, the prognosis is better.