A Case of Maffucci's Syndrome Associated with Spindle-cell Hemangioendothelioma

ABSTRACT

A case of a 21-year old female with Maffucci's syndrome is presented where the patient developed multiple cutaneous spindle-cell hemangioendotheliomas. She had multiple pea to walnut sized, non-tender, normal skin colored or slight bluish nodules and bony mass-like lesions on the left upper extremity and hand. A histological examination of the lesions showed that they were composed of irregularly dilated, thin walled cavernous blood spaces containing phleboliths and collapsed vascular spaces separated by spindled fibroblastic cells. Radiologically, the bony lesions showed radiolucent densities with calcified spots within the second proximal phalanx and metacarpal bone of the left hand, which were consistent with enchondromas.