胃肠道的血管周上皮样细胞肿瘤(PEComa)非常罕见,目前文献报道的不足20例,且大多为个案报道。作者收集35例胃肠道PEComa,分析临床病理特征及免疫表型,并作预后参数评估。35例中男性19例、女性16例,年龄7~70岁(中位年龄45岁)。发病部位:结肠19例、小肠12例、胃2例、胆囊及网膜各1例。肿瘤直径0.8~22 cm(中位直径6.2 cm)。3例肿瘤位于黏膜或黏膜下层,8例累及肌层,15 Gastrointestinal perivascular epithelioid cell tumor (PEComa) is very rare, less than 20 cases reported in the literature, and most of the case reports. The authors collected 35 cases of gastrointestinal PEComa, clinical and pathological features and immunophenotype analysis, and prognosis parameters. Of the 35 patients, 19 were males and 16 were females, aged 7 to 70 years (median age 45 years). The incidence of parts: 19 cases of colon, 12 cases of small intestine, stomach 2 cases, 1 case of gallbladder and omentum. Tumor diameter 0.8 ~ 22 cm (median diameter 6.2 cm). Three of the tumors were located in the mucosa or submucosa and eight in the myometrium