下肢Stewart-Treves综合征8例临床分析

Clinical analysis of 8 cases of Stewart-Treves syndrome in lower extremity

目的探讨下肢Stewart-Treves综合征(STS)的临床特征、治疗方法及预后转归。方法回顾性分析2013年10月~2017年6月北京世纪坛医院淋巴外科收治的下肢Stewart-Treves综合征患者的临床资料。结果共有8例下肢Stewart-Treves综合征患者,其中男2例,女6例,年龄15~66岁,平均年龄(43.3±18.7)岁。主要临床表现为肿胀肢体尤其是足踝部及小腿出现单发或多发青紫色、紫黑色皮肤肿物。磁共振成像(MRI)表现为突出于皮肤表面或位于皮肤真皮、皮下软组织至深筋膜散在的软组织结节。术后病理示异质性脉管肉瘤,免疫组织化学示CD31、CD34、D2-40染色呈阳性。8例患者均行手术治疗,其中1例术后辅助放疗,1例术后辅助化疗,1例术后辅助放疗+化疗。随访至2020年3月,1例生存出现肺转移,1例生存出现腰椎、肺转移,5例死亡,1例失访。结论STS是淋巴水肿的罕见并发症,下肢STS更为少见。STS易远处转移,预后差,死亡率高。

Objective To investigate the clinical features,treatment and prognosis of lower extremity Stewart Treves syndrome.Methods the clinical data of patients with lower extremity Stewart Treves syndrome admitted to Department of lymphatic surgery,Beijing Shijitan Hospital from October 2013 to June 2017 were retrospectively analyzed.Results There were 8 patients with lower extremity Stewart Treves syndrome,including 2 males and 6 females,aged from 15 to 66 years,with an average age of 43.3 years±7 years old.The main clinical manifestations were swollen limbs,especially the ankle and leg,with single or multiple purplish,purplish black skin masses.MRI showed scattered soft tissue nodules protruding from the skin surface or located in the dermis,subcutaneous soft tissue to deep fascia.Postoperative pathology showed heterogeneous angiosarcoma,and immuno histochemistry showed positive staining for CD31,CD34 and D2-40.Eight patients were treated with surgery,1 with postoperative adjuvant radiotherapy,1 with postoperative adjuvant chemotherapy,and 1 with postoperative adjuvant radiotherapy plus chemotherapy.During the follow-up to March 2020,1 case survived with lung metastasis,1 case survived with lumbar spine and lung metastasis,5 cases died and 1 case was lost to follow-up.Conclusions STS is a rare complication of lymphedema,especially in lower extremity.STS is prone to distant metastasis,poor prognosis and high mortality.Clinically,biopsy should be performed as soon as possible for the suspicious skin lesions of lymphedema limbs.Early diagnosis and amputation can prolong the survival time of patients.Active treatment of lymphedema may reduce the incidence of the disease.