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Updated: Jun 20, 2026

A Novel Method: Super-selective Adrenal Venous Sampling
Published on: September 15, 2017
Maffucci syndrome and adrenal cortex tumor
Nelly Puech-Bret1, Johan Bret, Antoine Bennet
1Service de Diabétologie, Maladies Métaboliques et Nutrition, CHU Rangueil, 31059 Toulouse cedex 9, France.
Abstract:
We report the second known case of Maffucci syndrome associated with an adrenal cortex tumor. Endocrine tumors have been reported in patients with multiple enchondromas, although the underlying mechanism of this combination is unknown. Maffucci syndrome is characterized by abnormalities of several mesodermal derivates. Therefore, routine evaluation for involvement of the adrenal cortex may be warranted to improve our knowledge of this syndrome and of its pathophysiology.
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