Karam Elias, Hollenbach Marcus, Heise Christian, Ali Einas Abou, Gulla Aiste, Auriemma Francesco, Terris Benoît, Souche François R, de Ponthaud Charles, Hank Thomas, Caillol Fabrice, Rhaiem Rami, Sauvanet Alain, Napoléon Bertrand, Regner Sara, Gaujoux Sébastien
Endocr Relat Cancer. 2025 Jan 17;32(3). doi: 10.1530/ERC-24-0238. Print 2025 Mar 1.
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.
壶腹复合性神经节细胞瘤/神经瘤和神经内分泌肿瘤（CoGNET），以前称为壶腹神经节细胞性副神经节瘤，是一种罕见的疾病，文献中仅有少数病例报道。这是一项对接受内镜或手术治疗的壶腹CoGNET患者的多中心回顾性队列研究。还对壶腹CoGNET进行了文献综述。共纳入15例患者，大多数为女性（n = 10），中位年龄为50岁。7例患者无症状。4例患者接受了胰十二指肠切除术，4例接受了经十二指肠壶腹切除术，8例接受了内镜乳头切除术。8例手术病例中有2例发生了Clavien-Dindo III-IV级并发症，但未记录到致命不良事件。仅发生了1例中度内镜不良事件。中位住院时间为9天。中位肿瘤大小为20 mm，R0切除率为93.8%，2例患者有淋巴结受累。中位随访29个月后，无局部或远处复发，也无疾病死亡。文献综述证实了壶腹CoGNET治疗的临床表现和良好结局，尤其是在生存方面，即使是有淋巴结或远处转移的患者。总体而言，壶腹CoGNET是预后良好的罕见肿瘤，即使切除不完全或有淋巴结受累。治疗应尽可能微创，并需要长期随访。
