Endocrine pathology · 2026
Clinical and Pathological Features of EC-cell Familial Small Intestine Neuroendocrine Tumors: A Nationwide Cohort From the French GTE-RENATEN Network.
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Hunaut Thomas, Gérard Laura, Hercent Agathe, Hentic Olivia, Buecher Bruno, Lepage Come, Lecomte Thierry, Thuillier Philippe, Do Cao Christine, Durand Alice, Roquin Guillaume, Lepere Céline, Lebras Maelle, Dominguez Sophie, Baltzinger Philippe, Devulder Pierre, Guedj Nathalie, Brixi Hedia, Féron Thomas, Cros Jérôme, Pasmant Eric, Lombard-Bohas Catherine, Walter Thomas, Cadiot Guillaume, de Mestier Louis
Аннотация
EC-cell familial small intestine neuroendocrine tumors (EC-cell F-SINET) are a recently described but poorly characterized entity. We aimed to describe their clinical and pathological features, and to compare them to patients with a sporadic form (EC-cell S-SINET). We constituted a nationwide cohort including (retrospectively patients diagnosed before 2012 and prospectively from 2012 to 2022) all patients with F-SINET (histologically proven SINET in ≥ 2 first or second-degree relatives) managed in the French GTE-RENATEN network. Clinical and pathological data were described and compared to the GTE-RENATEN population-based cohort including 2460 patients with EC-cell S-SINET using multivariable logistic regression. The survival impact of EC-cell F-SINET was explored using Cox proportional hazard analyses. We included 92 patients with EC-cell F-SINET from 47 families. Median age at diagnosis was 60.4 years. Among these patients, 22% had a history of another cancer, 76.1% had synchronous or metachronous metastases, 38% had a carcinoid tumor syndrome, and median Ki-67 labeling index was 2%. Median survival was 204.6 months. Multifocal EC-cell SINET were present in 66.7% of patients with EC-cell F-SINET and were associated with poorer prognosis (p = 0.005). In comparison with EC-cell S-SINET patients, those with EC-cell F-SINET had more frequent multifocal primary tumor in the small bowel (p < 0.0001) and carcinoid syndrome (p = 0.038), lower Ki-67 index and tended to be younger (p = 0.094). After adjustment on independent prognostic factors (age at diagnosis, metastatic stage, carcinoid tumor syndrome and Ki-67 index), EC-cell F-SINET did not have a different prognosis than that of EC-cell S-SINET. Patients with EC-cell F-SINET are characterized by more frequent multifocal EC-cell SINETs and more frequent carcinoid tumor syndrome, but their prognosis seems similar compared to patients with EC-cell S-SINET.