Pediatric case of colonic perivascular epithelioid cell tumor complicated with intussusception and anal incarceration:A case report  

作　　者：Luan Kou Wen-Wen Zheng Li Jia Xiao-Li Wang Ji-Hai Zhou Jiao-Rong Hao Zhu Liu Feng-Yu Gao 

机构地区：[1]Department of Gastroenterology,Shandong Provincial Maternal and Child Health Care Hospital,Jinan 250014,Shandong Province,China

出　　处：《World Journal of Gastrointestinal Oncology》2022年第7期1348-1355,共8页世界胃肠肿瘤学杂志（英文版）（电子版）

摘　　要：BACKGROUND Perivascular epithelioid cell tumor(PEComa)represents a group of rare mesenchymal tumors.PEComa can occur in many organs but is rare in the colorectum,especially in children.Furthermore,PEComa is a rare cause of intussusception,the telescoping of a segment of the gastrointestinal tract into an adjacent one.We describe a rare case of pediatric PEComa complicated with intussusception and anal incarceration,and conduct a review of the current literature.CASE SUMMARY A 12-year-old girl presented with abdominal pain and abdominal ultrasound suggested intussusception.Endoscopic direct-vision intussusception treatment and colonoscopy was performed.A spherical tumor was discovered in the transverse colon and removed by surgery.Postoperative pathologic analyses revealed that the tumor volume was 5.0 cm×4.5 cm×3.0 cm and the tumor tissue was located in the submucosa of the colon,arranged in an alveolar pattern.The cell morphology was regular,no neoplastic necrosis was observed,and nuclear fission was rare.The immunohistochemical staining results were as follows:Human melanoma black 45(HMB 45)(+),cluster of differentiation 31(CD31)(+),cytokeratin(-),melanoma-associated antigen recognized by T cells(-),smooth muscle actin(-),molleya(-),desmin(-),S-100(-),CD117(-),and Ki67(positive rate in hot spot<5%).Combined with the results of pathology and immunohistochemistry,we diagnosed the tumor as PEComa.Postoperative recovery was good at the 4 mo follow-up.CONCLUSION The diagnosis of PEComa mainly depends on pathology and immunohistochemistry.Radical resection is the preferred treatment method.

关 键 词：Perivascular epithelioid cell tumor COLONIC INTUSSUSCEPTION Anal incarceration Endoscopic direct-vision intussusception treatment Case report 

分 类 号：R735.35[医药卫生—肿瘤] R725.7[医药卫生—临床医学]