Stewart-Treves 综合征13例临床病理分析

Stewart-Treves syndrome: a clinicopathological analysis of thirteen cases

目的探讨Stewart-Treves综合征的临床病理学特征。方法回顾性分析13例Stewart-Treves综合征的临床病理特征,并复习相关文献。结果 13例Stewart-Treves综合征中,有5例发生于乳腺癌术后,3例发生于宫颈癌术后,5例继发于慢性淋巴水肿而无恶性肿瘤病史;其中6例恶性肿瘤患者术后曾行放疗;所有病例均以肢体水肿为首发症状,病变皮肤出现红斑或破溃。镜下表现为真皮层及皮下组织内可见浸润性生长的不规则脉管,部分区域脉管相互交通;部分区域内衬细胞异型性明显,可见核分裂象,肿瘤细胞排列呈乳头、巢片状,并可见出血、坏死。免疫组化:肿瘤细胞CD34、CD31、ERG、D2-40、c-myc、FⅧ和vimentin (+),Ki-67阳性指数30%～90%。患者随访,12例死亡(5～30个月),1例失访。结论 Stewart-Treves综合征少见,预后差,死亡率高,辅助免疫组化检测能够提高诊断的准确性,以达到早期诊断、早期治疗的目的。

Objective To explore the clinical pathologic features of Stewart-Treves syndrome. Method Thirteen cases of Stewart-Treves syndrome were collected between 2008—2017, and an analysis of their clinicopathologic characteristics was performed, with review of the relevant literature. Results Among thirteen cases of Stewart-Treves syndrome, 5 cases occurred secondary to postoperative breast cancer and 3 cases occurred secondary to cervical cancer; 6 patients underwent radiotherapy after surgery and 5 cases had no history of malignancy, secondary to chronic lymphedema; all cases presented with lymphedema as the first sign in pinkish-red papulovesicular or ulcer appeared on the skin lesion. Microscopic examination showed irregular vessels infiltratively grew in dermis and subcutaneous tissues,with obvious cellular pleomorphism, nucleoli and nuclear mitosis can be seen. The tumor cells arranged in papillary pattern and mutual transportation. Immunohistochemistry showed that tumor cells positively expressed CD34, CD31, ERG, D2-40, c-myc, Vimentin and FⅧ; Ki-67 index was 30%-90%. The survival periods were from 5 to 30 months, and one was lost to follow-up. Conclusion Stewart-Treves syndrome is rare with high mortality and immunohistochemistry can assist diagnosis in order to rearch accurate diagnosis and early treatment.