Maffucci Syndrome with Hemangioma of the Liver

Abstract

The presence of visceral hemangiomas in the syndrome of multiple enchondromas and subcutaneous hemangiomas, also named Maffucci syndrome, is exceedingly rare; until now noncutaneous hemangiomas have been described in 4 patients: they were found in the tongue in one patient, in the oral cavity in the second patient, in both the oral cavity and the colon in the third patient, and in the spleen in the fourth patient. We report the first case of hemangioma localized in the liver in a patient with Maffucci syndrome; furthermore, due to the impossibility of carrying out an MRI to define the hepatic lesion, an ultrasonographic real-time perfusion imaging study with a contrast agent was performed.