ABSTRACT
BACKGROUND: Maffucci's syndrome is a dysembryoplasia of the mesoderm, explaining the dual involvement of cartilage and vascular tissue. The risk of malignant degeneration or associated tumors is high in this uncommon disease. We report a case of Maffucci's syndrome associated with squamous cell carcinoma of the cavum. CASE REPORT: A 37-year-old man consulted for multiple angiomas and chondromas which had developed since childhood. The diagnosis of Maffucci's syndrome was obvious. Radiological exploration of the limbs confirmed chondromatosis and biopsy of the cavum performed because of the occurrence of epistaxis, revealed squamous cell carcinoma. DISCUSSION: Maffucci's angiochondromatosis is a rare, non-hereditary but sometimes congenital disease. Angiomas predominate on the hands and feet. Sarcomatous degeneration is the main complication of these chondromas. Angiomas rarely become malignant, but when they do are generally more aggressive than chondromas. The frequency of neoplasia in Maffucci's syndrome would suggest that there is a supplementary oncogenic factor. Several types of malignancy associated with Maffucci's syndrome have been reported, but to our knowledge there has been no publication concerning an association between Maffucci's syndrome and squamous cell carcinoma of the cavum.
