Malignant peripheral nerve sheath tumor with rhabdoid features arising in the temporal region of a patient suffering from neurofibromatosis 1 - A case report

• Autor(a) principal: Amer,Hatem Wael
• Data de Publicação: 2022
• Outros Autores: Shaheen,Hamed Abdelwahab, Ashoub,Madiha Nabil, Mahmoud,Sarah Ahmed Mohamed
• Tipo de documento: Relatório
• Idioma: eng
• Título da fonte: Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)
• Texto Completo: http://scielo.pt/scielo.php?script=sci_arttext&pid=S1646-28902022000200099
• topic: Head; Malignant peripheral nerve sheath tumor; Malignant triton tumor; Neurofibromatosis 1

Abstract The malignant peripheral nerve sheath tumor is one of the rare sarcomas that have a challenging diagnosis. It occurs independently with neurofibromatosis type 1. Some studies referred that malignant peripheral nerve sheath tumor attains a worse prognosis when it occurs in the head and neck region. In the reported case, a male patient with neurofibromatosis type 1 manifestations presented with a large mass in the temporal region. Based on the clinical history, histopathology, and immunohistochemical findings, the lesion was diagnosed as a low-grade malignant peripheral nerve sheath tumor with rhabdoid features. Surgical resection followed by intensity-modulated radiotherapy was the treatment of choice.