[A case of Maffucci's syndrome with intracranial chondroma (author's transl)].

A rare case of Maffucci's syndrome with intracranial chondroma was reported and the literature reviewed. A 16-year-old female high-school student was reffered to our service with 2 month history of diplopia and headache. She had been noticed to have multiple subcutaneous blueish nodules in the lower extremities and multiple body deformities in the four extremities since 6 months old. A biopsy of a skin lesion revealed cavernous hemangioma with calcified thrombi. Another biopsy from the deformed right femur showed it to be enchondroma. Skull series, carotid and vertebral arteriographies, and CT scan revealed a left parasellar mass, which had brought her sensory impairment of the left V1 and the left abducens palsy. The biopsy of the extradural mass in the medial portion of the left middle fossa proved it to be chondroma identical with the right femur lesion. So far as one year postoperative follow-up, she has been doing well with the postoperative sequela of mild left oculomotor paresis, having no evidence of malignant degeneration.