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

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

World Neurosurg. 2016 Jan;85:365.e7-15

Authors: Beer-Furlan A, Balsalobre L, Vellutini EA, Stamm AC

Abstract
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.

PMID: 26348567 [PubMed - indexed for MEDLINE]

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