摘要
目的 探讨子宫血管周上皮样细胞肿瘤(PEComa)硬化亚型的临床病理特征、免疫组化特点、鉴别诊断、治疗及预后。方法 收集1例子宫体硬化亚型PEComa的病例,观察其病理常规切片染色镜下组织学形态和免疫组织化学染色结果并复习相关文献。结果 镜下观察肿瘤细胞排列成巢片状或梁索状,肿瘤间质显著玻璃样变且范围大于50%,瘤细胞与血管关系密切,围绕薄壁血管呈放射状排列,细胞形态温和呈上皮样,胞质透亮,部分嗜酸,可见小核仁,异型性较小,核分裂象罕见(约1个/50HPF)。免疫组化标记结果:HMB45(局灶+)、Melan-A(部分+)、TFE3(弥漫+)、SMA(弥漫+)、Desmin(弥漫+)、Ki-67(+,热点区约25%)。结论 子宫硬化亚型PEComa较罕见,病理形态学易与其他女性生殖系统肿瘤混淆。当观察到显著的肿瘤间质玻璃样变,且存在特征性的肿瘤细胞围绕血管的生长方式,结合黑色素和肌源性免疫组化标记物的共阳性,有助于诊断此组织学亚型。
Objective To investigate the clinicopathologic features,immunohistochemical profile,differential diagnosis,treatment,and prognosis of the uterine sclerosing perivascular epithelioid cell tumor(PEComa).Methods Clinical and pathological data of one case of uterine sclerosing PEComa were collected.The histological features and immunohistochemical staining patterns of the tumor on routine pathologic sections were observed,and relevant literature was reviewed.Results Microscopic examination revealed tumor cells arranged in nests,sheets,and trabeculae/cords.The tumor stroma exhibited marked hyalinization(>50%).Tumor cells were closely associated with blood vessels,arranged radially around thin-walled blood vessels.The tumor cells exhibited a bland epithelioid morphology with mild atypia,translucent cytoplasm(partially eosinophilic),and small nucleoli;nuclear pleomorphism was slight,and mitotic figures were rare(approximately 1/50 HPF).Immunohistochemistry showed the following staining patterns:HMB45 was focally positive;Melan-A showed partial positivity;TFE3,SMA,and Desmin were diffusely positive;and the Ki-67 proliferation index was approximately 2%in hot spots.Conclusion The uterine sclerosing PEComa is relatively rare,and its pathological morphology is easily confused with other female reproductive system tumors.The diagnosis of this histological subtype is facilitated by the presence of prominent stromal hyalinization,the characteristic perivascular growth pattern of tumor cells,and the co-expression of melanocytic and myogenic immunohistochemical markers.
作者
李之玮
邱玉涵
焦皖婷
韦柔帆
孙武
张伟
LI Zhiwei;QIU Yuhan;JIAO Wanting;WEI Roufan;SUN Wu;ZHANG Wei(Department of Pathology,the First Affiliated Hospital of Wannan Medical College/Yijishan Hospital,Wuhu 241001,China)
出处
《诊断病理学杂志》
2025年第10期1233-1237,共5页
Chinese Journal of Diagnostic Pathology
基金
安徽省卫生健康科研项目(AHWJ2024BAg20007)
皖南医学院第一附属医院(弋矶山医院)引进人才专项科研启动基金(YR202214)
皖南医学院第一附属医院(弋矶山医院)三新项目(Z22016)。
关键词
子宫
血管周上皮样细胞肿瘤
硬化亚型
病理
Uterus
Perivascular epithelioid cell tumor
Sclerosing variant
Pathology
