Case Report
Copyright ©The Author(s) 2021. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Gastroenterol. Aug 21, 2021; 27(31): 5288-5296
Published online Aug 21, 2021. doi: 10.3748/wjg.v27.i31.5288
Plexiform angiomyxoid myofibroblastic tumor treated by endoscopic submucosal dissection: A case report and review of the literature
Jian-Di Wu, Yi-Xiong Chen, Chang Luo, Feng-Hua Xu, Lei Zhang, Xiao-Hua Hou, Jun Song
Jian-Di Wu, Yi-Xiong Chen, Chang Luo, Feng-Hua Xu, Lei Zhang, Xiao-Hua Hou, Jun Song, Division of Gastroenterology, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan 430022, Hubei Province, China
Author contributions: Wu JD reviewed the literature and contributed to manuscript drafting; Xu FH and Chen YX were the patient’s primary doctors and substantially contributed to the conception and design of the study and acquisition of the data; Luo C and Zhang L substantially contributed to the data acquisition; Hou XH and Song J were responsible for the revision of the manuscript for important intellectual content.
Informed consent statement: Informed written consent was obtained from the patient for publication of this report and any accompanying images.
Conflict-of-interest statement: The authors declare that they have no conflicts of interest to disclose.
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: http://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Jun Song, MD, Associate Chief Physician, Doctor, Division of Gastroenterology, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, No. 1277 Liberation Boulevard, Wuhan 430022, Hubei Province, China. song111jun@126.com
Received: March 22, 2021
Peer-review started: March 22, 2021
First decision: June 3, 2021
Revised: June 8, 2021
Accepted: July 26, 2021
Article in press: July 26, 2021
Published online: August 21, 2021
Processing time: 148 Days and 17 Hours
Abstract
BACKGROUND

Plexiform angiomyxoid myofibroblastic tumor (PAMT) is a rare mesenchymal tumor characterized by multiple nodular plexiform growth patterns and an immunophenotype with myofibroblasts. The pathological characteristics, immunohistochemistry, diagnostic criteria, differential diagnosis, and gene-level changes of PAMT have been reported in many studies. At present, the main treatment for PAMT in the reported cases is surgery; only eight cases were treated via endoscopy (excluding 1 thoracoscopic resection), and the lesions were all smaller than 5 cm. There are no reports on the prognosis and follow-up of young patients with lesion sizes reaching 5 cm who undergo endoscopic submucosal dissection (ESD). Herein, we present the first case of a young patient with a lesion size reaching 5 cm who was diagnosed with PAMT via endoscopic submucosal dissection.

CASE SUMMARY

A 15-year-old young man with upper abdominal pain for 2 years presented to the Gastroenterology Department of our hospital. Painless gastroscopy showed a semicircular bulge approximately 5 cm in size in the lesser curvature near the cardia of the fundus; the surface was eroded, and shallow ulcers had formed. The pathological manifestations of the biopsy were spindle cell proliferative lesions with interstitial mucinous changes, and the surface mucosa showed chronic inflammatory changes with active lesions; immunohistochemistry showed smooth muscle actin (SMA) (+), CD117 (-), CD34 (-), DOG-1 (-), S-100 (-), and Ki67 (LI: < 1%). We performed ESD on the patient. The lesion that we removed was 5 cm × 4 cm × 2 cm in size. Pathologically, the resected tissue displayed typical manifestations, such as fat spindle-shaped fibroblasts and myofibroblast-like cells showing irregular nodular hyperplasia. Immunohistochemistry staining of the tumor cells revealed the following: CD34 (partially +), SMA (weakly +), CD117 (-), DOG-1 (-), S-100 (-), SDHB (+), PCK (-), and Ki67 (labelling index: 2%). There was no recurrence or metastasis during the 3-mo follow-up after the operation, and the treatment effect was good. We also performed a review of the literature on the clinical manifestations, pathological features, immunohistochemistry, and differential diagnosis of PAMT.

CONCLUSION

At present, the diagnostic criteria for PAMT are relatively clear, but the pathogenesis and genetic changes require further study. PAMT is benign in nature, and these patients are less likely to experience local or metastatic recurrence. The main treatment is still surgery if the lesion is in the stomach. Partial gastrectomy and distal gastrectomy are the most frequently performed surgical treatments for PAMT, followed by local resection, subtotal gastrectomy, and wedge resection. But for comprehensive evaluation of the disease, ESD can be considered a suitable method to avoid excessive treatment.

Keywords: Plexiform angiomyxoid myofibroblastic tumor; Endoscopic submucosal dissection; Stomach; Cardia-preserving; Benign; Case report

Core Tip: Plexiform angiomyxoid myofibroblastic tumor (PAMT) is a rare mesenchymal tumor. The main treatment for PAMT in the reported cases is surgery. We present a rare case of PAMT in a young patient who underwent endoscopic submucosal dissection (ESD). The biopsied and resected tissue showed interstitial mucinous changes and myofibroblast-like cells showing irregular nodular hyperplasia, and immunohistochemistry revealed that the tissue was SMA (+). There was no recurrence during the 3-mo follow-up after ESD, and the treatment effect was good. For comprehensive evaluation of the disease, ESD can be considered a suitable method to avoid excessive treatment.