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目的探讨膀胱血管周上皮样细胞肿瘤(perivascular epithelioid cell tumors,PEComas)的临床病理特点、治疗及预后。方法收集1例膀胱PEComa的临床资料,行组织学观察及免疫组化,并复习相关文献。结果患者男,27岁,体检发现膀胱壁实性占位病变。组织学形态显示肿瘤组织位于膀胱肌壁间,大部分与周围组织分界清楚,局部在肌壁间浸润性生长。镜下上皮样或梭形肿瘤细胞呈巢团状、器官样、束状或编织状排列,瘤组织内富含薄壁和厚壁血管,血管壁广泛玻璃样变;瘤细胞胞质空亮或淡嗜酸性,细胞核呈圆形,囊泡状,可见小核仁,核分裂象。局部瘤细胞具轻度异型性,可见畸形核及多核巨细胞。免疫组化肿瘤细胞vimentin、HMB45、SMA、actin和S-100均(+),CK、EMA、Melan-A、NSE、Syn及CgA等均(-)。结论发生于膀胱的PEComa罕见,组织学形态结合免疫组化共表达HMB45和SMA有助于明确诊断,应与副神经节瘤、转移性肾透明细胞癌及恶性黑色素瘤等鉴别。
Objective To investigate the clinicopathological characteristics, treatment and prognosis of perivascular epithelioid cell tumors (PEComas). Methods One case of bladder PEComa was collected for histological observation and immunohistochemistry. The related literatures were reviewed. Results Male patient, 27 years old, physical examination found that the bladder wall solid lesions. Histological morphology showed that the tumor tissue was located between the bladder muscle wall, most of the clear boundaries with the surrounding tissue, local infiltration of muscle growth between the walls. Microscopic epithelial or spindle tumor cells were clustered in nest, organ-like, bundle or braided arrangement, the tumor tissue is rich in thin-walled and thick-walled vessels, a wide range of glass-like vascular wall; tumor cells cytoplasm empty or Light eosinophilic, the nucleus was round, vesicular, visible small nucleoli, mitotic. Local tumor cells with mild atypia, showing deformed nucleus and multinucleated giant cells. Immunohistochemical staining of vimentin, HMB45, SMA, actin and S-100 (+), CK, EMA, Melan-A, NSE, Syn, Conclusions The PEComa occurred in the bladder is rare. HMB45 and SMA coexpressed histologically with immunohistochemistry are helpful to confirm the diagnosis and should be differentiated from paraganglioma, metastatic renal clear cell carcinoma and malignant melanoma.