Extraskeletal myxoid chondrosarcoma of the masticator space in a pediatric patient

• Mário-José Romañach ^[1] ; Román Carlos ^[2] ; Michel Nuyens ^[3] ; Bruno-Augusto-Benevenuto de Andrade ^[1] ; Oslei-Paes de Almeida ^[4]
  1. [1] Universidade Federal do Rio de Janeiro

     Universidade Federal do Rio de Janeiro

     Brasil

     
  2. [2] DDS, Division of Pathology, Centro Clínico de Cabeza y Cuello/ Hospital Herrera Llerandi, Guatemala
  3. [3] MD, Division of Otorhinolaryngology - Head and Neck Surgery, Centro Clínico de Cabeza y Cuello/ Hospital Herrera Llerandi, Guatemala
  4. [4] DDS, PhD, Oral Pathology Section, Department of Oral Diagnosis, Piracicaba Dental School, University of Campinas, Brazil

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• Localización: Journal of Clinical and Experimental Dentistry, ISSN-e 1989-5488, Vol. 9, Nº. 6 (June ), 2017, págs. 825-831
• Idioma: inglés
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• Resumen

  • Extraskeletal myxoid chondrosarcoma (EMC) is a malignant soft-tissue neoplasm rarely described in the head and neck region of children and adolescents. We describe a case of EMC affecting the masticator space and a literature review. A 13-year-old boy who presented a large painless, diffuse mass causing progressive midfacial asymmetry of 6 months duration. Histopathological evaluation revealed a multinodular lesion, containing scattered round vacuolated tumor cells dispersed in an abundant myxoid stroma, separated by fibrous septae. Immunohistochemical analysis revealed positivity for vimentin, neuron-specific enolase, and chromogranin. The Ki-67 labelling index was 42%. The patient was treated surgically with tumor resection followed by adjuvant local radiotherapy. The patient died 1 year after initial diagnosis due to locoregional tumor dissemination. EMC should be considered in the differential diagnosis of myxoid neoplasms in the head and neck region.