Resumo

AMER, Hatem Wael; SHAHEEN, Hamed Abdelwahab; ASHOUB, Madiha Nabil  e  MAHMOUD, Sarah Ahmed Mohamed. Malignant peripheral nerve sheath tumor with rhabdoid features arising in the temporal region of a patient suffering from neurofibromatosis 1 - A case report. Rev Port Estomatol Med Dent Cir Maxilofac [online]. 2022, vol.63, n.2, pp.99-104.  Epub 30-Jun-2022. ISSN 1646-2890.  https://doi.org/10.24873/j.rpemd.2022.06.867.

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.

Palavras-chave : Head; Malignant peripheral nerve sheath tumor; Malignant triton tumor; Neurofibromatosis 1.

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