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

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• 作者：Teresa B Steinbichler (6)
  Florian Kral (6)
  Susanne Reinold (7)
  Herbert Riechelmann (6)
  
  6. Department of Otorhinolaryngology ; Medical University of Innsbruck ; Anichstrasse 35 ; A- 6020 ; Innsbruck ; Austria
  7. Department of Pathology ; Medical University of Innsbruck ; M眉llerstrasse 44 ; A-6020 ; Innsbruck ; Austria
  
• 关键词：Chondrosarcoma ; Enchondroma ; Hemangioma ; Maffucci syndrome
• 刊名：World Journal of Surgical Oncology
• 出版年：2014
• 出版时间：December 2014
• 年：2014
• 卷：12
• 期：1
• 全文大小：850 KB
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  2. Jacobi, CM, Hiranya, ES, Gay, A, Holzmann, D, Kollias, S, Soyka, M (2014) Enchondroma of the nasal septum due to Ollier disease: a case report and review of the literature. Head Neck.
  3. Hyde, GE, Yarington, CT, Chu, FW (1995) Head and neck manifestations of Maffucci鈥檚 syndrome: chondrosarcoma of the nasal septum. Am J Otolaryngol 16: pp. 272-275 CrossRef
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  11. Rosenberg, AE, Nielsen, GP, Keel, SB, Renard, LG, Fitzek, MM, Munzenrider, JE, Liebsch, NJ (1999) Chondrosarcoma of the base of the skull: a clinicopathologic study of 200 cases with emphasis on its distinction from chordoma. Am J Surg Pathol 23: pp. 1370-1378 CrossRef
  12. Lee, DH, Jung, SH, Yoon, TM, Lee, JK, Joo, YE, Lim, SC (2013) Low grade chondrosarcoma of the nasal septum. World J Clin Cases 1: pp. 64-66 CrossRef
  13. Guo, L, Liu, J, Sun, X, Wang, D (2013) Sinonasal tract chondrosarcoma: 18-year experience at a single institution. Auris Nasus Larynx 41: pp. 290-293 CrossRef
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• 刊物主题：Surgical Oncology;
• 出版者：BioMed Central
• ISSN：1477-7819

文摘

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.