骨上皮样血管内皮细胞瘤9例诊治分析

Diagnosis and therapy of bone epithelioid hemangioendothelioma: a report of 9 cases

目的 探讨骨上皮样血管内皮细胞瘤(B-EHE)的诊断、治疗方法.方法 回顾性分析2002年2月—2015年9月解放军东部战区总医院骨科收治的9例B-EHE患者的临床资料.其中男6例、女3例,年龄15~64岁.术前X线及CT检查均表现为溶骨性改变,1例MRI可见周围软组织受累,1例出现肺部转移灶. 3例选择病灶刮除、植骨内固定;1例腰椎病灶选择肿瘤切除椎板减压内固定;3例近关节病灶选择肿瘤切除假体置换,其中1例术后化疗;1例多发病灶伴肺转移选择活检确诊后化疗治疗;1例病理性骨折伴出血选择行截肢术后综合治疗.结果 9例中失访1例,其余随访时间8~72个月.现7例存活. 3例行病灶刮除植骨内固定,2例局部复发,二次手术后治愈. 1例腰椎肿瘤切除减压固定,术后恢复良好. 3例行肿瘤切除假体置换术,1例失访;2例术后治愈,关节功能良好,其中1例术后辅助化疗,未出现局部复发和病灶转移.1例多发病灶伴肺部转移行化疗,肺部无进展,局部无进展.1例行截肢术后综合治疗者,肿瘤复发迁延性出血,最终因器官衰竭死亡.结论 B-EHE为低-中度恶性肿瘤,术前影像学无特异性,诊断困难,主要依靠术后病理结果诊断.治疗首选边界切除,辅以化疗或放疗,可降低局部复发率和远处转移.

Objective To analyze nine cases of bone epithelioid hemangioendothelioma(B-EHE),retrospectively,and discuss the diagnosis and treatment.Methods Retrospective analysis of 9 patients of B-EHE in Eastern Theater General Hospital of PLA from February 2002 to September 2015 was performed,6 males and 3 females,ranged from 15 to 60 years.The surgical method of three cases was entire surgical excision,internally fixating with bone graft.One patient that lesion was located in lumbar vertebra was chosen scraped for decompression and stabilization.The 3 cases of adjacent joints lesions were en bloc excision with a modular resection prosthesis,one adopted chemotherapy after surgery.One case who presented as multifocal with pulmonary metastasis,conducted chemotherapy when identified by biopsy.One case occurred pathological fracture and hemorrhage and accepted comprehensive therapy after amputation.Results In 9 cases,1 case was lost to follow-up.The follow-up time of the rest was from 8 to 72 months.Seven cases survived.Three patients underwent bone grafting and internal fixation;two had local recurrence,and were cured after the second operation.One case that the tumor locate lumbar was resected and decompression fixation,and had good function after operation.Three patients underwent tumor resection and prosthesis replacement,and 2 patients were cured postoperatively with good joint function.Among them,1 patient received postoperative adjuvant chemotherapy without local recurrence and metastasis,and 1 patient lost follow-up.In 1 case,multiple lesions with pulmonary metastasis were treated with chemotherapy,with no pulmonary progression and no local progression.One patient received comprehensive treatment after amputation,and the tumor recurred and extended bleeding,and finally died of organ failure.Conclusions B-EHE is a low-to-moderate malignant tumor,which has no specificity in preoperative imaging and is difficult to diagnose.It mainly depends on postoperative pathological results for diagnosis.The preferred treatment is boundary resection,combined with chemotherapy or radiotherapy,local recurrence and distant metastasis.