Spindle cell hemangioendotheliomas associated with multiple skeletal enchondromas: a variant of Maffucci's syndrome.

Abstract

An unusual case of Maffucci's syndrome confined to an extremity with an additional superficial lymphangioma in the same limb is described. The patient, a 47-year-old woman, presented with several painful subcutaneous spindle cell hemangioendotheliomas (SCHs) and multiple lytic bone lesions, some calcified, in the left leg. She has been followed for two years and continues to do well. The combination of SCHs and multiple enchondromas (ENCHs) should be searched in any patient who presents with SCHs or multiple ENCHs, because there is a significant risk of malignant transformation.