Journal article
Composite gangliocytoma/neuroma and neuroendocrine tumour: a contemporary analysis of 71 cases shows risk factors for metastasis
Histopathology, Vol.87(6), pp.933-942
12/2025
DOI: 10.1111/his.15535
PMID: 40792544
Abstract
To describe the clinicopathological features of composite gangliocytoma/neuroma and neuroendocrine tumour (CoGNET) and possible risk factors for nodal metastasis.
We compiled a cohort of 71 cases from 19 institutions. Mean patient age was 58 years. Thirty-eight (54%) patients were male. Most patients (65%) had symptoms, including abdominal pain (20%) and gastrointestinal bleeding (19%). Most cases (70%) were described as a subepithelial mass/nodule, and nearly half (45%) were located in the 2nd portion of the duodenum. Mean tumour size was 2.2 cm, and most (87%) were well-circumscribed. Nearly all cases (96%) demonstrated all three histologic components, with the epithelioid component being the most predominant overall (mean 59%). All cases involved the submucosa, with 7 (10%) additionally involving the muscularis propria. Solid areas of ganglion-like cells were identified in 16/69 (23%) cases, glandular structure formation in 15/70 (21%), lymphovascular invasion (LVI) in 6/70 (9%) cases, and perineural invasion and necrosis in one case each. Nodal metastasis was identified at diagnosis in 8 (11%) cases; increased age, increased size, LVI and muscularis propria involvement were all significantly associated with nodal disease (P < 0.05). Follow-up data were available for 68 patients (mean 47 months); nearly all were alive without disease, though one patient developed liver metastasis after 8 months and died of the disease after 63 months.
This largest series of CoGNET to date demonstrates that approximately 10% of cases develop nodal metastases. Large tumour size, muscularis propria involvement, advanced patient age and LVI appear to be risk factors for nodal metastasis.
Details
- Title: Subtitle
- Composite gangliocytoma/neuroma and neuroendocrine tumour: a contemporary analysis of 71 cases shows risk factors for metastasis
- Creators
- Luiz M Nova-Camacho - Biogipuzkoa Health Research InstituteChangqing Ma - Washington University in St. LouisIbrahim Abukhiran - University of Pittsburgh Medical CenterNuha Shaker - University of Pittsburgh Medical CenterM-Nasan Abdul Baki - Allegheny General HospitalKatrina Collins - Indiana University – Purdue University IndianapolisIván González - Indiana University School of MedicineTimothy Chao - University of PennsylvaniaZhaohai Yang - University of PennsylvaniaMonika Vyas - Beth Israel Deaconess Medical CenterMichael Feely - University of FloridaAndrew M Bellizzi - University of Iowa Hospitals and ClinicsDiana Agostini-Vulaj - University of RochesterAaron R Huber - University of RochesterAlexandros D Polydorides - Icahn School of Medicine at Mount SinaiXinyang Xu - Icahn School of Medicine at Mount SinaiStefano La Rosa - University of InsubriaJose J Paricio - Complejo Hospitalario Universitario de AlbaceteSilvia Uccella - Humanitas UniversityMaria L Alvarez - Clinica Universidad de NavarraAinhoa Ordoñez Arrillaga - Donostiako Unibertsitate OspitaleaIrune Ruiz Diaz - Donostiako Unibertsitate OspitaleaDipti M Karamchandani - The University of Texas Southwestern Medical CenterClaudio Luchini - University of VeronaElena Verdú Martin - Hospital Universitario Insular de Gran CanariaTamara Zudaire Fuertes - Hospital San PedroSusana Rubio Mediavilla - Hospital San PedroWei Xiong - University of British ColumbiaBrian Cox - Pacific Rim Engineering (United States)Raul S Gonzalez - Emory University Hospital
- Resource Type
- Journal article
- Publication Details
- Histopathology, Vol.87(6), pp.933-942
- DOI
- 10.1111/his.15535
- PMID
- 40792544
- NLM abbreviation
- Histopathology
- ISSN
- 0309-0167
- eISSN
- 1365-2559
- Publisher
- Wiley
- Language
- English
- Electronic publication date
- 08/12/2025
- Date published
- 12/2025
- Academic Unit
- Pathology
- Record Identifier
- 9984946844502771
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