卡波西形血管内皮瘤临床病理特征

Clinicopathologic features of kaposiform hemangioendothelioma

目的 探讨卡波西形血管内皮瘤 (KHE)的临床病理学特征。方法 对 3例KHE进行临床、病理学观察并结合文献进行讨论。结果 其中女性 2例 ,男性 1例 ,均为婴儿。肿瘤位于腋胸部 2例 ,腰部 1例 ,临床上均伴有血小板减少性紫癜综合征。组织学上瘤组织主要由梭形内皮细胞构成 ,肿瘤呈结节状 ,并均见较为特征性的肾小球样结构。梭形细胞表达CD34及CD31。 2例患者伴有淋巴管瘤病。 2例患者KHE完整切除 ,1例患者KHE大部切除 ,随访均无瘤或带瘤存活。结论KHE是一种少见的主要发生于婴幼儿具有侵袭性的血管肿瘤 ,临床上常伴有血小板减少性紫癜综合征 ,组织学上具有婴幼儿毛细血管瘤和卡波西肉瘤的特点。治疗以扩大切除为主。其预后决定于原血管肿瘤的大小、部位和出血程度 。

Objective To explore the clinicopathologic features of kaposiform hemangioendothelioma(KHE) Methods The clinicopathologic data were studied in three cases of KHE and review the literatures Results Two cases were female and one was maleAll cases occurred in infancyTwo tumor located in axillary chest wall and one in lumbar regionAll of the three patients had Kasabach-Merritt syndromeHistologically,the tumor was composed of spindle-shaped cells in all cases nodular growth pattern was seenImmunohistochemically,Neoplastic spindled cells expressed CD34 and CD31Associated lymphangiomatosis was present in two casesTwo tumors were resected completely,one was resected partlythe follow-up period ranged from 6 months to 3 years,and all were alive Conclusions Kaposiform hemangioendotheliomais a rare locally aggressive vascular tumor that mainly occurred in early infancyIt is frequently complicated by Kasabach-Merritt syndrome,and it has features common to both capillary hemangioma and Kaposi sarcomaThe prognosis of KHE is determined by the size,location and the hemorrage degree of vascular tumorBetter outcome might be achieved in patients with KHE of the skin and in the soft tissues under the skinIt appears that the main treated measure should be wide local excision