Surgical treatment of a massive chondrosarcoma in the skull base associated with Maffucci’s syndrome: a case report

Abstract

BACKGROUND A successfully treated massive chondrosarcoma in the skull base associated with Maffucci’s syndrome is presented. The purpose of this report is to discuss the surgical approach to the tumor and reconstruction of the skull base. CASE DESCRIPTION A 36-year-old woman who had a history of multiple enchondromas and subcutaneous hemangiomas presented with decreased right visual acuity and left papilledema. Computed tomography (CT) and magnetic resonance imaging (MRI) demonstrated a mass in the skull base. The tumor occupied the nasal and paranasal cavities, and extended to the anterior, middle, and posterior intracranial spaces. The midline skull base structures and the left middle cranial base were destroyed. Using a combined anterior craniofacial and left orbitozygomatic approach, the tumor was totally resected. The large skull base defect was reconstructed with a vascularized outer table parietal bone graft attached to a bipedicled temporoparietal galeal flap. The postoperative course was uneventful except for decreased left visual acuity, and temporary diplopia and facial hypesthesia. In 40 months of follow-up there was no recurrence. CONCLUSIONS A skull base approach should be selected to perform total resection of an extensive skull base tumor. The bipedicled temporoparietal galeal flap and vascularized calvarial bone was useful for simultaneous reconstruction.