Maffucci syndrome and adrenal cortex tumor

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...

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Bibliographic Details
Published in:Joint, bone, spine : revue du rhumatisme bone, spine : revue du rhumatisme, 2009-10, Vol.76 (5), p.556-558
Main Authors: Puech-Bret, Nelly, Bret, Johan, Bennet, Antoine, Huyghe, Eric, Mazerolles, Catherine, Zabraniecki, Laurent, Fournie, Bernard
Format: Article
Language:English
Subjects:
Abdomen - pathology
Adenoma
Adrenal cortex
Adrenal Cortex Neoplasms - complications
Adrenocorticotropic Hormone - blood
Adult
Amputation
Bone Neoplasms - diagnostic imaging
Bone Neoplasms - pathology
Cushing syndrome
Enchondroma
Enchondromatosis - complications
Female
Femoral Neoplasms - diagnostic imaging
Femoral Neoplasms - pathology
Fingers - surgery
Hemangioma - complications
Hemangioma - pathology
Humans
Hydrocortisone - blood
Internal Medicine
Leg - pathology
Maffucci syndrome
Magnetic Resonance Imaging
Radiography
Rheumatology
Sarcoma - pathology
Sarcoma - surgery
Uterine Neoplasms - pathology
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Summary: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.
ISSN:1297-319X
1778-7254
DOI:10.1016/j.jbspin.2009.01.011