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

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• 作者：André ; Beer-Furlan¹ ; ² ; ^{beerfurlan@uol.com.br" class="auth_mail" title="E-mail the corresponding author} ; Leonardo Balsalobre¹ ; ³ ; Eduardo A.S. Vellutini¹ ; ² ; Aldo C. Stamm¹ ; ³
• 关键词：Chondrosarcoma ; Endoscopic endonasal approach ; Endoscopic surgery ; Maffucci syndrome ; Skull base chondrosarcoma
• 刊名：World Neurosurgery
• 出版年：2016
• 出版时间：January 2016
• 年：2016
• 卷：85
• 期：Complete
• 页码：365.e7-365.e15
• 全文大小：5228 K

文摘

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.