小肠转移性上皮样血管肉瘤临床病理分析

Metastatic epithelioid angiosarcoma of the small intestine:a clinicopathological analysis

目的探讨小肠转移性上皮样血管肉瘤(EA)的临床表现、组织学特征、诊断及鉴别诊断,进一步提高对该病的认识。方法回顾性分析我院收治的2例小肠转移性EA的临床资料,进行光镜及免疫表型观察,并复习相关文献报道的3例小肠转移性EA。结果小肠转移性EA发病年龄36~80岁(平均62岁),男3例,女2例。临床出现腹痛2例、黑便2例、贫血及钡灌肠异常1例。原发病灶2例来源于大腿软组织,2例来源于脏器-肝和肺,1例原发灶不详。病理组织学改变与原发性EA病灶相似,均表现为巢片状或索状排列的上皮样细胞,异型性明显,可见核分裂像、核仁、血窦及血管网结构。免疫表型4例表达CD31,3例表达Fli-1、ERG,2例表达ⅧRAG、CKpan。结论小肠转移性EA临床少见,预后较差,临床主要表现为腹痛、黑便及贫血等消化道症状,各部位EA均可转移,临床诊断较困难,但病理组织学改变有特点,结合临床病史及免疫组化染色血管内皮相关抗原呈阳性有助于诊断。

Objective To investigate the clinical manifestations,histological features,diagnosis and differential diagnosis of metastatic epithelioid angiosarcoma(EA)of the small intestine.Methods The clinical data of 2 cases of small intestinal metastatic EA in our hospital were analyzed retrospectively.The light microscopy and immunophenotype were observed.The related literatures of 3 cases of small intestinal metastatic EA were reviewed.Results The age of patients with small intestinal metastatic EA ranged from 36 to 80 years old,with an average age of 62 years old,including 3 males and 2 females.The main clinical manifestations were abdominal pain in 2 cases,melena in 2 cases,anemia and abnormal barium enema in 1 case.Two cases of primary lesions originated from thigh soft tissue,two from organs liver and lung,and one case was unknown.The histopathological changes were similar to those of primary EA lesions.All of them showed nest like or cord like epithelioid cells with obvious atypia.Mitotic images and nucleoli could be seen,and blood sinus and vascular network could be found.Immunophenotype showed CD31 in 4 cases,FLI-1 and ERG in 3 cases,and Ⅷ rag and ckpan in 2 cases.Conclusions Small intestinal metastatic EA is rare and has poor prognosis.The main clinical manifestations are abdominal pain,melena,anemia and other gastrointestinal symptoms.All parts of EA can be metastasized.The clinical diagnosis is difficult,but the pathological changes have characteristics.Combined with the clinical history and immunohistochemical staining,the positive expression of endodermis associated antigen is helpful for the diagnosis.