Copyright: ©Author(s) 2026. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution-NonCommercial (CC BY-NC 4.0) license. No commercial re-use. See permissions. Published by Baishideng Publishing Group Inc.
World J Gastroenterol. Dec 7, 2026; 32(45): 122826
Published online Dec 7, 2026. doi: 10.3748/wjg.122826
Published online Dec 7, 2026. doi: 10.3748/wjg.122826
Superior mesenteric arteriovenous fistula as a rare manifestation of Cowden syndrome: A case report and review of literature
Zi-Heng Zhao, Jing Qi, Jin-Ming Hu, Ying-Ying Zhao, Ming-Qi Liu, Li Deng, Fan Zhang, Shan-Shan Gao, Department of Gastroenterology, The First Affiliated Hospital of Shandong First Medical University and Shandong Provincial Qianfoshan Hospital, Jinan 250014, Shandong Province, China
Kun Li, Department of Gastroenterology, Shandong Provincial Hospital Affiliated to Shandong First Medical University, Jinan 250021, Shandong Province, China
Co-first authors: Zi-Heng Zhao and Jing Qi.
Co-corresponding authors: Shan-Shan Gao and Kun Li.
Author contributions: Zhao ZH wrote the manuscript and integrated all the data; Qi J collected and verified the clinical data of the in-house case; Hu JM assisted with clinical data collection; Zhao YY analyzed the clinical data; Liu MQ retrieved and curated all literature-reported cases; Deng L and Zhang F conducted the follow-up; Li K supervised the clinical management and follow-up; Gao SS supervised the literature review and performed the final scientific review; all authors have reviewed and approved the final manuscript; Zhao ZH and Qi J contributions in their efforts were equally critical to the conduction of the project and underlie their merit as co-first authors; the collaborative efforts of Li K and Gao S were necessary to completion of the project and its publication, warranting their designation as co-corresponding authors.
AI contribution statement: AI tools (Gemini) were used solely for linguistic refinement and formatting assistance. No AI tool was involved in the generation of research data, interpretation of results, or formulation of conclusions. All AI-generated outputs were critically reviewed and revised by the authors.
Supported by the National Natural Science Foundation of China, No. 82203149 and No. 82000580; the Shandong Medical Association, No. YXH2022ZX02113; and the Shandong Research Hospital Association, No. 2023012.
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 there are no conflicts of interest associated with this manuscript.
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).
Corresponding author: Kun Li, MD, PhD, Chief Physician, Professor, Department of Gas
Received: May 6, 2026
Revised: June 23, 2026
Accepted: July 8, 2026
Published online: December 7, 2026
Processing time: 169 Days and 11.3 Hours
Revised: June 23, 2026
Accepted: July 8, 2026
Published online: December 7, 2026
Processing time: 169 Days and 11.3 Hours
Core Tip
Core Tip: We report the first documented case of Cowden syndrome (CS) complicated by a superior mesenteric arteriovenous fistula. A systematic review of the literature identified 13 cases, in addition to the present case, of CS associated with gastrointestinal and hepatic vascular anomalies. These findings suggest that these lesions may be linked to the imbalance of angiogenesis and vascular remodeling induced by PTEN mutations, thereby expanding the known vascular phenotypic spectrum of CS.