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

World Journal of Surgical Oncology

Herbert Riechelmann¹  Susanne Reinold²  Florian Kral¹  Teresa B Steinbichler¹ 

[1] Department of Otorhinolaryngology, Medical University of Innsbruck, Anichstrasse 35, A- 6020 Innsbruck, Austria;Department of Pathology, Medical University of Innsbruck, Müllerstrasse 44, A-6020 Innsbruck, Austria

关键词: Maffucci syndrome;    Hemangioma;    Enchondroma;    Chondrosarcoma;   

Others  :  1146825
DOI  :  10.1186/1477-7819-12-387

 received in 2014-07-22, accepted in 2014-12-03,  发布年份 2014

【 摘 要 】

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.

【 授权许可】

   
2014 Steinbichler et al.; licensee BioMed Central.

【 图 表 】

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