title: Chondrosarcoma of the nasal cavity in a patient with Maffucci syndrome: case report and review of the literature.
abstract: BACKGROUND
  Maffucci syndrome is a rare, congenital, non-hereditary mesodermal dysplasia, manifested by multiple enchondromas and hemangiomas. Malignant transformation of these lesions is seen in up to 40% of the cases.
  
  
  METHODS
  We present a case of a patient with Maffucci syndrome and an associated chondrosarcoma of the nose. Treatment consisted of surgical resection. Because of the low grade of the tumor, additional treatment, such as radiotherapy, was not necessary.
  
  
  CONCLUSIONS
  Maffucci syndrome is an exceedingly rare mesodermal dysplasia. Its manifestation in the head and neck region is even less common. Malignant transformation of the associated enchondromas is common, and should be considered whenever a change of the clinical course occurs. Random, periodically performed X-ray examinations give little additional information on malignant transformation and are considered useless.
authors.affiliation: Department of Otorhinolaryngology, Medical University of Innsbruck, Anichstrasse 35, A- 6020 Innsbruck, Austria. teresa.steinbichler@googlemail.com.
authors.forename: Teresa B
authors.initials: TB
authors.lastname: Steinbichler
authors.forename: Florian
authors.initials: F
authors.lastname: Kral
authors.forename: Susanne
authors.initials: S
authors.lastname: Reinold
authors.forename: Herbert
authors.initials: H
authors.lastname: Riechelmann
mesh_terms: D000328:Adult; D001859:Bone Neoplasms / Q000209:etiology* / Q000473:pathology / Q000601:surgery; D002813:Chondrosarcoma / Q000209:etiology* / Q000473:pathology / Q000601:surgery; D004687:Enchondromatosis / Q000150:complications* / Q000473:pathology / Q000601:surgery; D005260:Female; D006801:Humans; D009296:Nasal Cavity / Q000473:pathology* / Q000601:surgery; D009668:Nose Diseases / Q000209:etiology* / Q000473:pathology / Q000601:surgery; D011379:Prognosis