Case Report
Copyright ©The Author(s) 2022. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Gastrointest Oncol. Jul 15, 2022; 14(7): 1348-1355
Published online Jul 15, 2022. doi: 10.4251/wjgo.v14.i7.1348
Pediatric case of colonic perivascular epithelioid cell tumor complicated with intussusception and anal incarceration: A case report
Luan Kou, Wen-Wen Zheng, Li Jia, Xiao-Li Wang, Ji-Hai Zhou, Jiao-Rong Hao, Zhu Liu, Feng-Yu Gao
Luan Kou, Wen-Wen Zheng, Li Jia, Xiao-Li Wang, Ji-Hai Zhou, Jiao-Rong Hao, Zhu Liu, Feng-Yu Gao, Department of Gastroenterology, Shandong Provincial Maternal and Child Health Care Hospital, Jinan 250014, Shandong Province, China
Author contributions: Kou L analyzed the data and wrote the paper; Zheng WW, Jia L, and Gao FY revised the paper; Wang XL, Zhou JH, Hao JR, and Liu Z collected the patient’s clinical data; Gao FY designed the report.
Informed consent statement: Informed written consent was obtained from the patients for the publication of this report and any accompanying images.
Conflict-of-interest statement: The authors declare that they have no conflict of interest.
CARE Checklist (2016) statement: The authors have read the CARE Checklist (2016), and the manuscript was prepared and revised according to the CARE Checklist (2016).
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: https://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Feng-Yu Gao, MD, Doctor, Department of Gastroenterology, Shandong Provincial Maternal and Child Health Care Hospital, No. 238 Jingshi East Road, Jinan 250014, Shandong Province, China. gaofengyu2000@163.com
Received: December 15, 2021
Peer-review started: December 15, 2021
First decision: April 17, 2022
Revised: April 28, 2022
Accepted: June 27, 2022
Article in press: June 27, 2022
Published online: July 15, 2022
Processing time: 209 Days and 15.7 Hours
Abstract
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.

Keywords: Perivascular epithelioid cell tumor; Colonic; Intussusception; Anal incarceration; Endoscopic direct-vision intussusception treatment; Case report

Core Tip: Perivascular epithelioid cell tumor (PEComa) of the colon is rarely encountered in the clinic, especially in pediatric patients. We describe a rare case of PEComa complicated with intussusception and anal incarceration in a 12-year-old female. We performed endoscopic direct-vision intussusception treatment and surgical removal. The diagnosis of PEComa mainly depends on pathology and immunohistochemistry. Radical resection is the preferred treatment method.