MAFFUCCIS-SYNDROME ASSOCIATED WITH NASOPH ARYNX EPIDERMOID CARCINOMA

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 un common disease. We report a case of Maffucci's syndrome associated wit h squamous cell carcinoma of the cavum. Case report. A 37-year-old man consulted for multiple angiomas and chondromas which had developed si nce childhood. The diagnosis of Maffucci's syndrome was obvious. Radio logical exploration of the limbs confirmed chondromatosis and biopsy o f the cavum performed because epitaxis occurred revealed squamous cell carcinoma. Discussion. Maffucci's angiochondromatosis is a rare, non- hereditary but sometimes congenital disease. Angiomas predominate on t he hands and feet. Sarcomatous degeneration is the main complication o f these chondromas. Angiomas rarely become malignant, but when they do are generally more aggressive than chondromas. The frequency of neopl asia in Maffucci's syndrome would suggest that there is a supplementar y oncogenic factor. Several types of malignant associated with Maffucc i's syndrome have been reported, but to our knowledge there has been n o publication concerning an association between Maffucci's syndrome an d squamous cell carcinoma of the cavum.