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Updated: Apr 4, 2026
Role of Diffusion MRI Tractography in Endoscopic Endonasal Skull Base Surgery
Published on: July 5, 2021
Endoscopic Endonasal Approach in Skull Base Chondrosarcoma Associated with Maffucci Syndrome: Case Series and
André Beer-Furlan1, Leonardo Balsalobre2, Eduardo A S Vellutini1
1São Paulo Skull Base Center, São Paulo, Brazil; DFVneuro Neurosurgical Group, São Paulo, Brazil.
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.
