卡波西样血管内皮瘤伴卡梅综合征1例报告与文献复习

Chemotherapy for Kaposiformhemangioendothelioma with Kasabach-Merritt Syndrome: a case report and literature review

目的了解卡波西样血管内皮瘤(Kaposiformhemangio-endothelioma,KHE)伴卡梅综合征(Kasabach-Merritt syndrome,KMS)的诊断、临床特点及治疗方法。方法分析采用甲强龙+长春新碱(VCR)+环磷酰胺(CTX)治疗1例2012年2月我院收治的经病理诊断KHE伴KMS的临床疗效,并复习相关文献。结果 (1)化疗方案采用甲强龙+VCR+CTX。2周血小板恢复正常。观察至2012年6月患儿血小板及凝血功能恢复正常,左侧肩关节活动改善,复查肩部CT提示肿瘤明显缩小。(2)化疗后患儿出现双上眼睑下垂,经营养神经治疗后恢复正常。长春新碱毒副作用可逆。结论 KHE是一种临床罕见,但以儿童和婴幼儿为主要发病年龄的低度恶性血管源性肿瘤,临床上常伴有KMS。本病例提示对于难以手术治疗的患儿可给予激素及免疫抑制剂的联合治疗,能改善KMS并肿瘤缩小。

Objective To understand the clinical manifestation,the diagnosis,and treatment of Kaposiformhemangioendothelioma （KHE） with Kasabach-Merritt syndrome （KMS）.Methods We treated a patient who was pathologically diagnosed as KHE with KMS and received chemotherapy including methylprednisolone,vincristine （VCR） and cyclophosphamide （CTX） in our hospital in February of 2012.The clinical characteristics,laboratory test,therapy and response to chemotherapy were analyzed.The related literature was also reviewed.Results Chemotherapy protocol included methylprednisolone,VCR and CTX.The platelets returned to normal level after two-week treatment.Until to June 2012,the platelets and cruor function returned to normal level and the activities of left shoulder joint improved.The reexamined shoulder CT indicated significant reduction in tumor size.Then the child appeared double upper eyelid ptosis,which return to normal after given trophic nerve treatment.Vincristine toxicity was reversible.Conclusion KHE is a rare type of low malignant vascular tumor which primarily occurred in children and infants.It is often complicated with Kasabach-Merritt syndrome.The experience of this case suggests that if the patient was difficult to be operated on,combination therapy including steroid and immunosuppressant could improve the KMS and reduce the tumor size.