[Maffucci's syndrome associated with primary brain tumor: report of a case]

A rare case of Maffucci's syndrome associated with astrocytoma at the right frontal lobe and intracranial chondroma is reported in detail with a review of literature. A 24-year-old male who had diagnosed as multiple chondroma at the age of 14 years old was admitted to the hospital with complaint of double vision. Neurological examination revealed no abnormality except for the left abducens palsy. Multiple subcutaneous blue nodules in the left hand was carried for biopsy as cavernous hemangioma. Craniograph showed expansive radiolucent area in the parasellar region. CT scan demonstrated a round shaped low density area in the right frontal lobe, part of which was iso-density and turned out to be enhanced following contrast infusion. Roentgenogram disclosed many well-demarcated radiolucent area in phalanges of the left hand and leg, and right leg and third rib. The frontal tumor was totally extirpated and was histologically fibrillary astrocytoma. This is the ninth case of the Maffucci's syndrome associated with the primary intracranial tumor in the literature. It is emphasized that the Maffucci's syndrome is associated with a high incidence of malignancy. And the reason for tendency to develop malignant tumors is discussed.