血管球瘤8例临床病理分析

Clinicopathological features of glomus tumors:a report of 8 cases

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,therapy and prognosis of glomus tumor.Methods The clinical features,histopathological changes,and immunohistochemical findings were analyzed in 8 cases of glomus tumors,with review of the related literature.Results Microscopically,the tumor cells were round,or oval,showing conformity in shape with clear or eosinophilic cytoplasm,no cellular atypia and mitotic figures were noted;blood vessels of the tumor were abundant,the tumor cells arranged around blood vessles showing a solid or hemangiopericytoma-like pattern.Myxoid change and hyaline degeneration were seen in the stroma of glomus tumor.Histological subtypes of 8 cases included classical glomus tumor,glomangioma,glomangiomyoma and myxoid glomus tumor.Immunohistochemically,the tumor cells were positive for SMA,collagen Ⅳ,and vimentin in all the cases,and one case was positive for CD34,but negative for other markers including CD117,S-100,desmin,calponin,CgA,Syn,EMA,CK,LCA,CD38,MPO,CD43,CD30,HMB45,MelanA,and GFAP.All 8 cases had no evidence of recurrence or metastasis in follow-up for 12~48 months.Conclusion Glomus tumor is an extremely rare neoplasm,and the atypical morphology of glomus tumors in unusual location might cause diagnostic difficulty.The diagnosis for glomus tumor in such situation depends mainly on the clinical characteristics,morphology and immunohistochemical expression,and needs to be differentiated from other tumors such as myopericytoma,hemangiopericytoma,neuroendocrine tumor,epithelioid stromal tumors,neoplasms with perivascular epithelioid cell differention,paraganglioma and other tumors.