Small Intestinal Carcinoid Tumors Clinical Trial
Official title:
Clinic, Pathologic and Genetic Characterization of Patients With Familial Carcinoid Tumors (Study From the GTE, Groupe d'étude Des Tumeurs Endocrines)
Small intestine carcinoid tumors are rare. Small intestine Familial Carcinoid Tumors (FCT)
are defined by the occurrence of at least 2 cases of this tumor type in first- or
second-degree relatives. The estimated prevalence of FCT is 2.6%-3.7% in patients with small
intestine carcinoid tumors. Because of its rarity, epidemiologic, clinic and pathologic
features of FCT have been scarcely described. Molecular abnormalities associated with FCT
have been poorly explored. Constitutional genetic factors predisposing to FCT have not been
discovered to date. Only one abnormality (mutation of the IPMK gene) has been reported in one
FCT family only, but not found in other series.
The main objective of this study is to identify the constitutional factors predisposing to
small-intestine FCT (and other midgut localizations: ascending colon and appendix). The
secondary objectives are to describe the clinic and pathologic features associated with FCT.
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