Artículos de revistas
Rabdomyosarcoma of the Mandible: An Uncommon Clinical Presentation
Fecha
2018-05-01Registro en:
Journal Of Craniofacial Surgery. Philadelphia: Lippincott Williams & Wilkins, v. 29, n. 3, p. E221-E224, 2018.
1049-2275
10.1097/SCS.0000000000004154
WOS:000434303800004
2402682969776875
0000-0001-7015-7175
Autor
Universidade Estadual Paulista (Unesp)
Universidade Estadual de Campinas (UNICAMP)
Institución
Resumen
Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma in children and adolescents. Most patients present with a mass in the head and neck region, urogenital region, or with distal extremity involvement. The authors describe a challenging case of a 6-year-old male patient presenting with mandibular RMS. The clinical/radiographic/tomographic evaluations classified the tumor as an advanced stage (stage IV), with a mass of 6.0cm involving the left side of the mandible and parotid region. The biopsy revealed round, spindled, and pleomorphic cells with hyperchromatic nuclei and rare larger rhabdomyoblasts with eosinophilic cytoplasm. The diagnosis was of embryonal RMS. The patient was referred for treatment with cycles of chemotherapy; however, pulmonary and bone marrow metastasis were identified. Radiotherapy and local surgery with microvascular reconstruction were performed later; however, the patient died after a few months. Early diagnosis is critical for a good prognosis and cure of patients with RMS. Correct diagnosis considering also the histological subtype is important for adequate treatment, which according to the literature is not uniform probably because of the rarity of this neoplasm.