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目的探讨血管球瘤的临床病理特征、诊断与鉴别诊断及治疗与预后。方法分析8例血管球瘤的临床特征、病理学形态和免疫学表型,并结合文献复习进行讨论。结果典型病理形态为肿瘤细胞圆形、卵圆形,形态较一致,胞质丰富红染、透亮,无明显异型性和核分裂象;肿瘤血管较丰富,瘤细胞围绕血管呈实性巢片状、血管外皮瘤样排列;间质可见黏液变性、玻璃样变性。8例血管球瘤组织亚型包括经典型、球血管瘤型、球血管肌瘤型、黏液样型。免疫组化示8例肿瘤细胞SMA、Ⅳ型胶原和vimentin均(+),其中1例CD34(+);而CD117、S-100、desmin、calponin、CgA、Syn、EMA、CK、LCA、CD38、MPO、CD43、CD30、HMB45、melanA和GFAP均(-)。8例患者术后随访12-48个月均无复发、转移。结论血管球瘤是少见的软组织肿瘤,位于少见部位的血管球瘤常不易被识别,确诊依赖于临床特征、组织形态及免疫组化结果相结合,且需要与血管周肌细胞瘤、血管外皮细胞瘤、神经内分泌肿瘤、上皮样胃肠间质瘤、具有血管周上皮样细胞分化的肿瘤和副节瘤等鉴别。
Objective To investigate the clinicopathological features, diagnosis, differential diagnosis, treatment and prognosis of glomus tumors. Methods The clinical features, pathological features and immunological phenotypes of 8 cases of glomus tumors were analyzed and discussed in the literature review. Results The typical pathomorphology of the tumor cells was round and oval, the morphology was more consistent, the cytoplasm was rich in red staining, translucent, no obvious atypia and mitosis. The tumor vessels were richer, Vascular endothelial tumor-like arrangement; interstitial mucus degeneration, degeneration of the glass. 8 cases of angiomatous tissue subtypes include classic, hemangioma, glioma myoma, mucinous type. Immunohistochemistry revealed 8 cases of SMA, type Ⅳ collagen and vimentin (+), of which 1 case of CD34 (+); while CD117, S-100, desmin, calponin, CgA, Syn, EMA, CK, , MPO, CD43, CD30, HMB45, melanA and GFAP (-). Eight patients were followed up for 12-48 months without recurrence and metastasis. Conclusions Angiospioma is a rare soft tissue tumor. The glomerular tumors located in rare sites are often not easily identified. The diagnosis depends on clinical features, histomorphology and immunohistochemical results, and needs to be related to vascular myocytoma, vascular endothelial cells Tumors, neuroendocrine tumors, epithelioid gastrointestinal stromal tumors, differentiated tumors with perivascular epithelioid cells, and paraganglioma.