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.
Service de Diabétologie, Maladies Métaboliques et Nutrition, CHU Rangueil, 31059 Toulouse cedex 9, France.
Nelly
N
Puech-Bret
Johan
J
Bret
Antoine
A
Bennet
Eric
E
Huyghe
Catherine
C
Mazerolles
Laurent
L
Zabraniecki
Bernard
B
Fournie
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