Romañach Mário-José, Carlos Román, Nuyens Michel, de Andrade Bruno-Augusto-Benevenuto, de Almeida Oslei-Paes
DDS, PhD, Oral Pathology, Department of Oral Diagnosis and Pathology, Federal University of Rio de Janeiro School of Dentistry, Brazil.
DDS, Division of Pathology, Centro Clínico de Cabeza y Cuello/ Hospital Herrera Llerandi, Guatemala.
J Clin Exp Dent. 2017 Jun 1;9(6):e825-e831. doi: 10.4317/jced.53888. eCollection 2017 Jun.
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. Extraskeletal myxoid chondrosarcoma, masticator space, parapharyngeal space, immunohistochemistry, children.
骨外黏液样软骨肉瘤（EMC）是一种恶性软组织肿瘤，在儿童和青少年的头颈部很少见。我们报告一例累及咀嚼肌间隙的EMC病例并进行文献复习。一名13岁男孩，出现一个持续6个月的无痛性、弥漫性大肿块，导致进行性面部中部不对称。组织病理学评估显示为多结节性病变，含有散在的圆形空泡状肿瘤细胞，分散于丰富的黏液样基质中，由纤维间隔分隔。免疫组化分析显示波形蛋白、神经元特异性烯醇化酶和嗜铬粒蛋白呈阳性。Ki-67标记指数为42%。患者接受了手术肿瘤切除，随后进行辅助局部放疗。患者在初次诊断后1年因局部肿瘤播散死亡。在头颈部黏液样肿瘤的鉴别诊断中应考虑EMC。骨外黏液样软骨肉瘤、咀嚼肌间隙、咽旁间隙、免疫组化、儿童
