血管内乳头状血管内皮增生临床病理分析及文献复习

Intravascular papillary vascular endothelial hyperplasia:a clinicopathological analysis with review of literature

目的探讨血管内乳头状血管内皮增生(IPEH)的临床病理特征、鉴别诊断及预后。方法收集9例血管内乳头状血管内皮细胞增生病例,分析其临床特点,观察其病理学形态及免疫组织化学表型,结合相关文献进行回顾性分析并随访。结果患者年龄26~66岁,平均年龄(44.0±13.7)岁,男性3例,女性6例。好发于头颈部及胸部皮肤或黏膜下,均为单发病灶。临床表现为体表无痛性缓慢生长的丘疹样斑块或隆起性结节,病变范围局限。大体表现为棕褐色实性或囊实性结节,最大径0.6~2.0 cm,平均最大径(1.17±0.50 cm)。显微镜下典型的组织学形态是血管腔内增生的内皮细胞呈乳头状生长,间质纤维玻璃样变,常伴有不全机化血栓或凝血。免疫组化表达CD31、CD34及F8。鉴别诊断的关键是不要误诊成血管肉瘤。随访显示病变无复发或转移。结论IPEH是一种罕见的良性血管病变,病变局限,具有典型的临床病理学特点,手术完整切除后预后良好,应避免过度治疗。

Objective To investigate the clinicopathological features,differential diagnosis and prognosis of intravascular papillary vascular endothelial hyperplasia(IPEH).Methods Nine cases of IPEH were collected,and their clinical features were analyzed.Pathological morphology and immunohistochemical phenotype were observed.Retrospective analyses and follow-up were performed based on relevant literature.Results The patients ranged in age from 26 to 66 years with an average of(44.0±13.7)years.There were 3 males and 6 females.Most of them occured in the skin or submucosa of the head,neck and chest with single lesions.The clinical manifestations were painless and slow-growing papular plaques or protuberant nodules on the surface of the body,and the lesion scope was limited.The general appearance was tan solid or cystic solid nodules with a maximum diameter of 0.6-2.0 cm and an average maximum diameter of(1.17±0.50)cm.Under the microscope,the typical histological morphology was the papillary growth of endothelial cells proliferating in vascular lumen,hyaline degeneration of interstitial fibers,often accompanied by incomplete thrombosis or coagulation.Immunohistochemical showed the expression of CD31,CD34 and F8.Differential diagnosis from angiosarcoma was usually required.Follow-up showed no recurrence or metastasis.Conclusion IPEH is a rare benign vascular lesion with typical clinicopathological characteristics.It is very important for its recognition and correct diagnosis.The lesion is circumscribed and has favourable prognosis after complete surgical resection,and so excessive treatment should be avoided.