Familial midgut neuroendocrine tumors (FM-NETs): Results of the nationwide TCF cohort from the GTE-RENATEN network

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Introduction: The clinical specificities of familial midgut neuroendocrine tumors (FM-NET) have been poorly described.

Aim(s): We aimed to describe their clinical and pathological features and to compare them with sporadic midgut NETs.

Materials and methods: The TCF cohort is a nationwide cohort that included (retrospectively from 1990 or prospectively from 2012 to 2022) all patients with FM-NET (histologically proven midgut NET in ≥ 2 first-degree relatives) managed in the GTE-RENATEN network. Clinical and pathological data were compared to a monocentric cohort of 342 patients with sporadic midgut NET managed in one expert center). Characteristics associated with FM-NET and survival were explored with multivariable logistic regression or Cox proportional hazard models.

Conference:

Presenting Author: de Mestier L

Authors: Hunaut T, Hercent A, Walter T, Buecher B, Lepage C,

Keywords: familial, midgut, neuroendocrine, tumors, carcinoid,