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  • Title: Endoscopic Endonasal Approach in Skull Base Chondrosarcoma Associated with Maffucci Syndrome: Case Series and Literature Review.
    Author: Beer-Furlan A, Balsalobre L, Vellutini EA, Stamm AC.
    Journal: World Neurosurg; 2016 Jan; 85():365.e7-15. PubMed ID: 26348567.
    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.
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