Chondrosarcoma of the nasal cavity in a patient with Maffucci syndrome: case report and review of the literature

World Journal of Surgical Oncology
Teresa B SteinbichlerHerbert Riechelmann

Abstract

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. 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. 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.

References

Jul 1, 1995·American Journal of Otolaryngology·G E HydeF W Chu
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Mar 11, 2008·Best Practice & Research. Clinical Rheumatology·Stéphanie Pannier, Laurence Legeai-Mallet
Jun 13, 2008·Otolaryngologia polska. The Polish otolaryngology·Wojciech ScierskiAgata Hajduk
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Nov 20, 2013·Journal of Oral and Maxillofacial Pathology : JOMFP·Aarti M MahajanMahendra C Mahajan
Dec 5, 2013·World Journal of Clinical Cases·Dong Hoon LeeSang Chul Lim

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Citations

Feb 28, 2016·BMC Research Notes·Olga ProkopchukHelmut Friess

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Methods Mentioned

BETA
biopsy
surgical resection
X-ray

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