Ampullary composite gangliocytoma/neuroma and neuroendocrine tumor management

in Endocrine-Related Cancer
Authors:
Elias Karam Department of Visceral Surgery and Liver Transplantation, Centre Hospitalier Universitaire de Tours, Tours, France

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Marcus Hollenbach Department of Gastroenterology, Endocrinology, Metabolism and Clinical Infectology - University Hospital Marburg UKGM, Marburg, Germany

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Christian Heise Medical Department I, Martin-Luther University Halle-Wittenberg, Halle, Germany

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Einas Abou Ali Department of Gastroenterology, Digestive Oncology and Endoscopy, Cochin Hospital, Paris, France

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Aiste Gulla Institute of Medicine, Faculty of Medicine, Vilnius University, Vilnius, Lithuania
Department of Surgery, MedStar Georgetown University Hospital, Georgetown, USA

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Francesco Auriemma Humanitas Clinical and Research Hospital, Rozzano, Digestive Endoscopy Unit, Division of Gastroenterology, Milano, Italy

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Benoît Terris Université Paris Cité, Faculté de Médecine, Paris, France
Department of Pathology, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris, Paris, France

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François R Souche Department of Digestive Surgery, Centre Hospitalier Universitaire de Montpellier, Montpellier, France

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Charles de Ponthaud Department of Hepato-Biliary and Pancreatic Surgery and Liver Transplantation, AP-HP Pitié-Salpêtrière Hospital, Paris, France
Sorbonne University, Paris, France

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Thomas Hank Department of General-, Visceral- and Transplantation Surgery, Heidelberg University Hospital, Heidelberg, Germany

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Fabrice Caillol Department of Endoscopy, Institut Paoli Calmettes, Marseille, France

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Rami Rhaiem Department of Hepatobiliary, Pancreatic, and Digestive Surgery, Robert Debré University Hospital, Reims, France

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Alain Sauvanet University Reims Champagne-Ardenne, Reims, France
Departement of Digestive Surgery, Beaujon Hospital, APHP, Clichy, France

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Bertrand Napoléon Hopital privé Jean Mermoz, Ramsay Générale de Santé, Lyon, France

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Sara Regner Lund University, Department of Clinical Sciences Malmö, Section for Surgery, Lund, Sweden

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Sébastien Gaujoux Department of Hepato-Biliary and Pancreatic Surgery and Liver Transplantation, AP-HP Pitié-Salpêtrière Hospital, Paris, France
Sorbonne University, Paris, France

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https://orcid.org/0000-0002-1072-7639

Correspondence should be addressed to S Gaujoux: sebastien.gaujoux@aphp.fr
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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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