Maffucci syndrome and adrenal cortex tumor.
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.
Puech-Bret
Nelly
N
Service de Diabétologie, Maladies Métaboliques et Nutrition, CHU Rangueil, 31059 Toulouse cedex 9, France.
Bret
Johan
J
Bennet
Antoine
A
Huyghe
Eric
E
Mazerolles
Catherine
C
Zabraniecki
Laurent
L
Fournie
Bernard
B
D000005:Abdomen / Q000473:pathology; D000306:Adrenal Cortex Neoplasms / Q000150:complications*; D000324:Adrenocorticotropic Hormone / Q000097:blood; D000328:Adult; D000671:Amputation, Surgical; D001859:Bone Neoplasms / Q000000981:diagnostic imaging / Q000473:pathology; D004687:Enchondromatosis / Q000150:complications*; D005260:Female; D005266:Femoral Neoplasms / Q000000981:diagnostic imaging / Q000473:pathology; D005385:Fingers / Q000601:surgery; D006391:Hemangioma / Q000150:complications / Q000473:pathology; D006801:Humans; D006854:Hydrocortisone / Q000097:blood; D007866:Leg / Q000473:pathology; D008279:Magnetic Resonance Imaging; D011859:Radiography; D012509:Sarcoma / Q000473:pathology / Q000601:surgery; D014594:Uterine Neoplasms / Q000473:pathology
D000324:Adrenocorticotropic Hormone; D006854:Hydrocortisone