Chondrosarcoma of the nasal cavity in a patient with Maffucci syndrome: case report and review of the literature.
World J Surg Oncol
; 12: 387, 2014 Dec 17.
Article
em En
| MEDLINE
| ID: mdl-25519205
ABSTRACT
INTRODUCTION:
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. CASE REPORT 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.CONCLUSION:
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.
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Neoplasias Ósseas
/
Doenças Nasais
/
Condrossarcoma
/
Encondromatose
/
Cavidade Nasal
Tipo de estudo:
Prognostic_studies
Limite:
Adult
/
Female
/
Humans
Idioma:
En
Revista:
World J Surg Oncol
Ano de publicação:
2014
Tipo de documento:
Article
País de afiliação:
Áustria