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

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