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Ampullary composite gangliocytoma/neuroma and neuroendocrine tumor management

Karam, Elias ; Hollenbach, Marcus ; Heise, Christian ; Ali, Einas Abou ; Gulla, Aiste ; Auriemma, Francesco ; Terris, Benoît ; Souche, François R. ; de Ponthaud, Charles and Hank, Thomas , et al. (2025) In Endocrine-Related Cancer 32(3).
Abstract

Ampullary composite gangliocytoma/neuroma and neuroendocrine tumor (CoGNET), previously called ampullary gangliocytic paragangliomas, is a rare entity, with only few reported cases in the literature. This is a multicentric retrospective cohort study of patients treated with endoscopy or surgery for ampullary CoGNET. A literature review of ampullary CoGNET was also performed. Fifteen patients were included, mostly female (n = 10) with a median age of 50 years. Patients were asymptomatic in seven cases. Four patients were treated with pancreatoduodenectomy, four with transduodenal ampullectomy, and eight with endoscopic papillectomy. Clavien–Dindo III–IV complications occurred in 2 of the 8 surgical cases, but no fatal adverse events were... (More)

Ampullary composite gangliocytoma/neuroma and neuroendocrine tumor (CoGNET), previously called ampullary gangliocytic paragangliomas, is a rare entity, with only few reported cases in the literature. This is a multicentric retrospective cohort study of patients treated with endoscopy or surgery for ampullary CoGNET. A literature review of ampullary CoGNET was also performed. Fifteen patients were included, mostly female (n = 10) with a median age of 50 years. Patients were asymptomatic in seven cases. Four patients were treated with pancreatoduodenectomy, four with transduodenal ampullectomy, and eight with endoscopic papillectomy. Clavien–Dindo III–IV complications occurred in 2 of the 8 surgical cases, but no fatal adverse events were registered. There was only one moderate endoscopic adverse event. The median length of stay was 9 days. The median tumor size was 20 mm, the R0 resection rate was 93.8%, and two patients had nodal involvement. After a median follow-up of 29 months, there was no local or distant recurrence nor death from disease. The literature review confirmed the clinical presentation and excellent outcomes of ampullary CoGNET management, especially regarding survival, even for patients with nodal or distant metastases. Overall, ampullary CoGNET are rare tumors with excellent prognosis, even with incomplete resection or nodal involvement. Treatment should be as minimally invasive as possible, and a long-term follow-up is needed.

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organization
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type
Contribution to journal
publication status
published
subject
keywords
ampulla of Vater, CoGNET, composite gangliocytoma/neuroma and neuroendocrine tumor, endoscopic papillectomy, gangliocytic paraganglioma, pancreaticoduodenectomy, transduodenal surgical ampullectomy
in
Endocrine-Related Cancer
volume
32
issue
3
article number
e240238
publisher
Society for Endocrinology
external identifiers
  • pmid:39745186
  • scopus:85216136138
ISSN
1351-0088
DOI
10.1530/ERC-24-0238
language
English
LU publication?
yes
id
67a9d137-6527-4265-ace7-cb774da17152
date added to LUP
2025-03-20 14:20:42
date last changed
2025-07-25 00:00:09
@article{67a9d137-6527-4265-ace7-cb774da17152,
  abstract     = {{<p>Ampullary composite gangliocytoma/neuroma and neuroendocrine tumor (CoGNET), previously called ampullary gangliocytic paragangliomas, is a rare entity, with only few reported cases in the literature. This is a multicentric retrospective cohort study of patients treated with endoscopy or surgery for ampullary CoGNET. A literature review of ampullary CoGNET was also performed. Fifteen patients were included, mostly female (n = 10) with a median age of 50 years. Patients were asymptomatic in seven cases. Four patients were treated with pancreatoduodenectomy, four with transduodenal ampullectomy, and eight with endoscopic papillectomy. Clavien–Dindo III–IV complications occurred in 2 of the 8 surgical cases, but no fatal adverse events were registered. There was only one moderate endoscopic adverse event. The median length of stay was 9 days. The median tumor size was 20 mm, the R0 resection rate was 93.8%, and two patients had nodal involvement. After a median follow-up of 29 months, there was no local or distant recurrence nor death from disease. The literature review confirmed the clinical presentation and excellent outcomes of ampullary CoGNET management, especially regarding survival, even for patients with nodal or distant metastases. Overall, ampullary CoGNET are rare tumors with excellent prognosis, even with incomplete resection or nodal involvement. Treatment should be as minimally invasive as possible, and a long-term follow-up is needed.</p>}},
  author       = {{Karam, Elias and Hollenbach, Marcus and Heise, Christian and Ali, Einas Abou and Gulla, Aiste and Auriemma, Francesco and Terris, Benoît and Souche, François R. and de Ponthaud, Charles and Hank, Thomas and Caillol, Fabrice and Rhaiem, Rami and Sauvanet, Alain and Napoléon, Bertrand and Regner, Sara and Gaujoux, Sébastien}},
  issn         = {{1351-0088}},
  keywords     = {{ampulla of Vater; CoGNET; composite gangliocytoma/neuroma and neuroendocrine tumor; endoscopic papillectomy; gangliocytic paraganglioma; pancreaticoduodenectomy; transduodenal surgical ampullectomy}},
  language     = {{eng}},
  number       = {{3}},
  publisher    = {{Society for Endocrinology}},
  series       = {{Endocrine-Related Cancer}},
  title        = {{Ampullary composite gangliocytoma/neuroma and neuroendocrine tumor management}},
  url          = {{http://dx.doi.org/10.1530/ERC-24-0238}},
  doi          = {{10.1530/ERC-24-0238}},
  volume       = {{32}},
  year         = {{2025}},
}