Endoscopic Endonasal Approach in Skull Base Chondrosarcoma Associated with Maffucci Syndrome: Case Series and Literature Review.

Autor: Beer-Furlan A; São Paulo Skull Base Center, São Paulo, Brazil; DFVneuro Neurosurgical Group, São Paulo, Brazil. Electronic address: beerfurlan@uol.com.br., Balsalobre L; São Paulo Skull Base Center, São Paulo, Brazil; São Paulo ENT Center, Professor Edmundo Vasconcelos Hospital, São Paulo, Brazil., Vellutini EA; São Paulo Skull Base Center, São Paulo, Brazil; DFVneuro Neurosurgical Group, São Paulo, Brazil., Stamm AC; São Paulo Skull Base Center, São Paulo, Brazil; São Paulo ENT Center, Professor Edmundo Vasconcelos Hospital, São Paulo, Brazil.
Jazyk: angličtina
Zdroj: World neurosurgery [World Neurosurg] 2016 Jan; Vol. 85, pp. 365.e7-15. Date of Electronic Publication: 2015 Sep 05.
DOI: 10.1016/j.wneu.2015.08.070
Abstrakt: Background: Maffucci 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 Description: We report 2 other cases successfully treated through an expanded endoscopic endonasal approach and discuss the condition based on the literature review.
Conclusions: Skull 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.)
Databáze: MEDLINE
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