Zhang PC, Wang JJ, Li J, Li AQ. Clinical and pathological features and treatment of nine cases of gastrointestinal composite gangliocytoma/neuroma and neuroendocrine tumor. World J Gastrointest Oncol 2026; 18(9): 120569 [DOI: 10.4251/wjgo.120569]
Corresponding Author of This Article
Ai-Qing Li, Chief Physician, Department of Gastroenterology, The First Affiliated Hospital, Zhejiang University School of Medicine, No. 79 Qinchun Road, Hangzhou 310003, Zhejiang Province, China. 3310015@zju.edu.cn
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Gastroenterology & Hepatology
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research-article
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Zhang PC, Wang JJ, Li J, Li AQ. Clinical and pathological features and treatment of nine cases of gastrointestinal composite gangliocytoma/neuroma and neuroendocrine tumor. World J Gastrointest Oncol 2026; 18(9): 120569 [DOI: 10.4251/wjgo.120569]
World J Gastrointest Oncol. Sep 15, 2026; 18(9): 120569 Published online Sep 15, 2026. doi: 10.4251/wjgo.120569
Clinical and pathological features and treatment of nine cases of gastrointestinal composite gangliocytoma/neuroma and neuroendocrine tumor
Peng-Cheng Zhang, Jing-Jie Wang, Jun Li, Ai-Qing Li
Peng-Cheng Zhang, Jing-Jie Wang, Ai-Qing Li, Department of Gastroenterology, The First Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou 310003, Zhejiang Province, China
Jun Li, Department of Pathology, The First Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou 310003, Zhejiang Province, China
Co-first authors: Peng-Cheng Zhang and Jing-Jie Wang.
Author contributions: Zhang PC and Wang JJ contributed equally to this work and thus qualified as the co-first authors of the paper. Li AQ designed the study and was responsible for developing the methodology and the accuracy of the data analysis; Zhang PC, and Wang JJ participated in the formal analysis and investigation; Li J reviewed all histological samples to confirm the diagnosis; Zhang PC, and Wang JJ performed data analysis and wrote the original draft; Li AQ, Zhang PC, Wang JJ, Li J participated in the review and editing. All authors have read and approved the final manuscript.
AI contribution statement: (1) Doubao (an AI tool) was used to draft the preliminary English translation of the answering-reviewers document. ChatGPT, Grammarly, DeepL and other AI tool were not used; (2) Doubao only provided initial translation. All responses to reviewer comments were revised, restructured and supplemented manually by the authors; (3) Doubao only provided initial translation. No AI tool used for language polishing, data analysis, or writing assistance of the "Answering-Reviewers" document; (4) No AI tool participated in research design and result interpretation; and (5) No images in this document are AI-generated.
Institutional review board statement: This study was conducted in strict accordance with the ethical guidelines outlined in the Declaration of Helsinki and received approval from the Clinical Research Ethics Committee of the First Affiliated Hospital, Zhejiang University, School of Medicine, No. 2025B-1511.
Informed consent statement: Written informed consent was waived considering the retrospective study design.
Conflict-of-interest statement: All the authors report no relevant conflicts of interest for this article.
Data sharing statement: The datasets analyzed in the current study are available from the corresponding author on reasonable request. Please email the corresponding author at 3310015@zju.edu.cn.
Corresponding author: Ai-Qing Li, Chief Physician, Department of Gastroenterology, The First Affiliated Hospital, Zhejiang University School of Medicine, No. 79 Qinchun Road, Hangzhou 310003, Zhejiang Province, China. 3310015@zju.edu.cn
Received: March 3, 2026 Revised: March 30, 2026 Accepted: June 8, 2026 Published online: September 15, 2026 Processing time: 177 Days and 0.2 Hours
Abstract
BACKGROUND
Composite gangliocytoma/neuroma and neuroendocrine tumor (CoGNET), previously known as gangliocytic paraganglioma, is a rare neuroendocrine neoplasm with metastatic potential. CoGNET occurs most frequently in the duodenum, presents with non-specific symptoms, is usually detected incidentally on endoscopy or cross-sectional imaging, and requires histopathological examination as the sole means of definitive diagnosis. To date, clinical research on CoGNET is limited to isolated case reports in China, with a notable lack of more cases to systematically characterize its full spectrum of clinicopathological features, treatment outcomes, and long-term prognosis.
AIM
To investigate the clinicopathological features and treatment of gastrointestinal CoGNET.
METHODS
This retrospective case series was approved by the ethics review committee of a tertiary care hospital in China. Nine patients with pathologically confirmed gastrointestinal CoGNET from 2019 to 2024 were enrolled, and cases with incomplete clinical data were excluded. Clinicopathological data including demographics, clinical manifestations, endoscopic and imaging findings, treatment regimens and pathological results were collected. All patients were followed up via outpatient visits and telephone interviews.
RESULTS
Nine patients with pathologically confirmed CoGNET were included, with a mean age of 51.2 years and a male-to-female ratio of 5:4. Eight lesions were located in the duodenum, and one was the first reported primary rectal CoGNET. All lesions achieved en bloc resection (4 surgical, 5 endoscopic), with one case of regional lymph node metastasis. All lesions presented the typical triphasic cellular differentiation, and no tumor recurrence, metastasis or death occurred during a mean follow-up period of 41 months.
CONCLUSION
CoGNET presents an indolent clinical course and favorable prognosis, and endoscopic resection is effective for eligible patients at low risk of lymph node metastasis.
Core Tip: Composite gangliocytoma/neuroma and neuroendocrine tumor (CoGNET) is an extremely rare neuroendocrine neoplasm. This study reports the largest case series of gastrointestinal CoGNET in China to date, including the world’s first reported case of primary rectal CoGNET as we know. We systematically analyzed the clinicopathological, endoscopic, and radiological characteristics of 9 pathologically confirmed CoGNET cases. Our findings validated the safety and long-term efficacy of endoscopic resection, and confirmed an excellent prognosis for all patients, including the case with lymph node metastasis. This work provides pivotal real-world evidence to optimize clinical diagnosis and treatment strategies for this rare disease.
