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

Citation
E. Tachibana et al., Surgical treatment of a massive chondrosarcoma in the skull base associated with Maffucci's syndrome: A case report, SURG NEUROL, 54(2), 2000, pp. 165-169
Citations number
17
Language
INGLESE
art.tipo
Article
Categorie Soggetti
Neurology
Journal title
SURGICAL NEUROLOGY
ISSN journal
0090-3019 → ACNP
Volume
54
Issue
2
Year of publication
2000
Pages
165 - 169
Database
ISI
SICI code
0090-3019(200008)54:2<165:STOAMC>2.0.ZU;2-X
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 dis cuss the surgical approach to the tumor and reconstruction of the skull bas e. CASE DESCRIPTION A 36-year-old woman who had a history of multiple enchondromas and subcutan eous hemangiomas presented with decreased right visual acuity and left papi lledema. Computed tomography (CT) and magnetic resonance imaging (MRI) demo nstrated a mass in the skull base. The tumor occupied the nasal and paranas al cavities, and extended to the anterior, middle, and posterior intracrani al spaces. The midline skull base structures and the left middle cranial ba se were destroyed. Using a combined anterior craniofacial and left orbitozy gomatic approach, the tumor was totally resected. The large skull base defe ct was reconstructed with a vascularized outer table parietal bone graft at tached to a bipedicled temporoparietal galeal flap. The postoperative cours e was uneventful except for decreased left visual acuity, and temporary dip lopia and facial hypesthesia. In 40 months of follow-up there was no recurr ence. CONCLUSIONS A skull base approach should be selected to perform total resection of an e xtensive skull base tumor. The bipedicled temporoparietal galeal flap and v ascularized calvarial bone was useful for simultaneous reconstruction. (C) 2000 by Elsevier Science Inc.