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
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.