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


METHODS
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.
São Paulo Skull Base Center, São Paulo, Brazil; DFVneuro Neurosurgical Group, São Paulo, Brazil. Electronic address: beerfurlan@uol.com.br.
André
A
Beer-Furlan
São Paulo Skull Base Center, São Paulo, Brazil; São Paulo ENT Center, Professor Edmundo Vasconcelos Hospital, São Paulo, Brazil.
Leonardo
L
Balsalobre
São Paulo Skull Base Center, São Paulo, Brazil; DFVneuro Neurosurgical Group, São Paulo, Brazil.
Eduardo A S
EA
Vellutini
São Paulo Skull Base Center, São Paulo, Brazil; São Paulo ENT Center, Professor Edmundo Vasconcelos Hospital, São Paulo, Brazil.
Aldo C
AC
Stamm
D000328:Adult; D002813:Chondrosarcoma / Q000175:diagnosis* / Q000209:etiology / Q000473:pathology / Q000601:surgery*; D003937:Diagnosis, Differential; D004687:Enchondromatosis / Q000150:complications* / Q000235:genetics; D005260:Female; D006391:Hemangioma / Q000209:etiology; D006801:Humans; D008297:Male; D009154:Mutation; D060787:Neoplasm Grading; D044583:Neuroendoscopy / Q000379:methods*; D009666:Nose; D015588:Observer Variation; D012086:Reoperation; D019292:Skull Base Neoplasms / Q000175:diagnosis* / Q000209:etiology / Q000473:pathology / Q000601:surgery*; D016896:Treatment Outcome
Chondrosarcoma; Endoscopic endonasal approach; Endoscopic surgery; Maffucci syndrome; Skull base chondrosarcoma