Clinicopathological analysis of head and neck rhabdomyosarcoma: a series of 10 cases and literature review

• Flávia-Sirotheau-Correa Pontes ^[1] ; Jonas-Ikikame de Oliveira ^[1] ; Lucas-Lacerda de Souza ^[1] ; Oslei-Paes de Almeida ^[3] ; Eduardo-Rodrigues Fregnani ^[4] ; Rafael-Sarlo Vilela ^[5] ; Wanessa-Miranda Silva ^[4] ; Felipe-Paiva Fonseca ^[2] ; Hélder-Antônio-Rebelo Pontes ^[1]
  1. [1] Universidad Federal de Pará

     Universidad Federal de Pará

     Brasil

     
  2. [2] Universidade Federal de Minas Gerais

     Universidade Federal de Minas Gerais

     Brasil

     
  3. [3] Department of Oral Diagnosis (Pathology), Piracicaba Dental School, University of Campinas (Piracicaba/Brazil)
  4. [4] Department of Oral Medicine, Sírio-Libanês Hospital, São Paulo/Brazil
  5. [5] Department of Pathology, Sírio-Libanês Hospital, São Paulo/Brazil

  Mostrar afiliaciones +
• Localización: Medicina oral, patología oral y cirugía bucal. Ed. inglesa, ISSN-e 1698-6946, Vol. 23, Nº. 2 (marzo), 2018
• Idioma: inglés
• Enlaces
  • Texto completo
• Resumen

  • To describe the clinicopathological characteristics of a series of head and neck rhabdomyosarcoma (RMS) and to review the literature.

    Cases diagnosed as RMS affecting the head and neck region were retrospectively retrieved from the files of two Brazilian institutions from January 2006 to January 2017. Data on clinical features (sex, age and affected site), microscopic subtype, immunohistochemical results, treatment employed and follow-up status were obtained from the patient’s medical charts.

    During the period considered, 10 cases of RMS were identified. Females predominated (4M:6F), the mean age at diagnosis was 16.5 years-old and the orbit was the most affected site (4 cases). Microscopically, most cases were classified as embryonal RMS (6 cases) and the Desmin/Myogenin/Myo-D1 immunohistochemical positivity was useful to confirm the diagnosis. Chemotherapy and radiotherapy were applied to 9 and 8 patients respectively, whereas 2 patients were treated by surgery. Recurrences occurred in 3 patients and distant metastasis in 2 cases. Nine patients were alive in their last follow-up, 3 of them with disease, whereas 1 patient died due to the disease.

    Head and neck RMS is an aggressive malignant neoplasm which demands especial concern to achieve early diagnosis and successful treatment.