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Alveolar rhabdomyosarcoma of the mandible in an adult invading the parotid gland: A rare case report

INTRODUCTION: Rhabdomyosarcoma (RMS) is a malignant mesenchymal neoplasm uncommon in adults. PRESENTATION OF CASE: We report the case of an alveolar rhabdomyosarcoma in a rare location and in an adult. It concerns a 46-year-old woman with a mandibular location invading the parotid gland. The Immunoh...

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Detalles Bibliográficos
Autores principales: Yassir, Hammouda, Ngham, Hicham, Lyoubi, Mouna, Abada, Reda Allah, Oukessou, Youssef, Roubal, Mohammed, Mahtar, Mohammed
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Elsevier 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7721655/
https://www.ncbi.nlm.nih.gov/pubmed/33395910
http://dx.doi.org/10.1016/j.ijscr.2020.10.052
Descripción
Sumario:INTRODUCTION: Rhabdomyosarcoma (RMS) is a malignant mesenchymal neoplasm uncommon in adults. PRESENTATION OF CASE: We report the case of an alveolar rhabdomyosarcoma in a rare location and in an adult. It concerns a 46-year-old woman with a mandibular location invading the parotid gland. The Immunohistochemical analysis revealed intense positivity for myogenin and desmin, favoring the diagnosis of alveolar rhabdomyosarcoma. The patient had a right hemi-mandibulectomy with parotidectomy and received chemotherapy with radiotherapy. The evolution was good up to 2 years postoperatively then the patient relapsed with a recurrence of tumour rapidly progressing and metastases in the cervical spine. DISCUSSION: RMS is an aggressive but rare disease that is one of the most common malignant head and neck tumors in children. The predilection sites of adult rhabdomyosarcoma are the extremities. Current treatment includes a combination of ablative surgery, chemotherapy, and radiation therapy. CONCLUSION: The prognosis of head and neck rhabdomyosarcomas in adults remains very poor given the often initially advanced stage of the disease and the high metastatic potential.