• André Beer-Furlan, Leonardo Balsalobre, Eduardo A S Vellutini, and Aldo C Stamm.
• São Paulo Skull Base Center, São Paulo, Brazil; DFVneuro Neurosurgical Group, São Paulo, Brazil. Electronic address: beerfurlan@uol.com.br.
• World Neurosurg. 2016 Jan 1; 85: 365.e7-15.

BackgroundMaffucci syndrome is a nonhereditary disorder in which patients develop multiple enchondromas and cutaneous, visceral, or soft tissue hemangiomas. The potential malignant progression of enchondroma into a secondary chondrosarcoma is a well-known fact. Nevertheless, chondrosarcoma located at the skull base in patients with Maffuci syndrome is a very rare condition, with only 18 cases reported in the literature.Case DescriptionWe report 2 other cases successfully treated through an expanded endoscopic endonasal approach and discuss the condition based on the literature review.ConclusionsSkull base chondrosarcoma associated with Maffucci syndrome is a rare condition. The disease cannot be cured, therefore surgical treatment should be performed in symptomatic patients aiming for maximal tumor resection with function preservation. The endoscopic endonasal approach is a safe and reliable alternative for the management of these tumors.Copyright © 2016 Elsevier Inc. All rights reserved.

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