Recurrent chondrosarcoma of the right skull base in a patient with Maffucci syndrome.
Maffucci syndrome is a rare, sporadic disease characterized by the development of multiple enchondromas and subcutaneous hemangiomas. Patients with Maffucci syndrome have a 23-37% risk of malignancy, with chondrosarcomas being the most common. Although the development of a chondrosarcoma in a patient with Maffucci syndrome may be expected, intracranial chondrosarcomas are rare. We present a patient with Maffucci syndrome who was diagnosed with an intracranial chondrosarcoma after presenting with hearing loss and vomiting. After three craniotomies and two recurrences of chondrosarcoma of the right skull base, the patient demonstrated a positive outcome to treatment with external radiation therapy.
Abdelmalek
Mark
M
Department of Dermatology, Drexel University College of Medicine, Philadelphia, Pennsylvania, USA. mark.abdelmalek@drexelmed.edu
Stanko
Christine
C
D000328:Adult; D002813:Chondrosarcoma / Q000175:diagnosis* / Q000209:etiology; D004687:Enchondromatosis / Q000150:complications* / Q000473:pathology; D005260:Female; D006225:Hand / Q000473:pathology; D006391:Hemangioma / Q000473:pathology; D006801:Humans; D008279:Magnetic Resonance Imaging; D008875:Middle Aged; D009364:Neoplasm Recurrence, Local / Q000175:diagnosis*; D012307:Risk Factors; D012878:Skin Neoplasms / Q000473:pathology; D019292:Skull Base Neoplasms / Q000175:diagnosis* / Q000209:etiology