Details

Autor(en) / Beteiligte

• Amer, Hatem
• Shaheen, Hamed
• Ashoub, Madiha
• Mahmoud, Sarah

Titel

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

Ist Teil von

• Revista portuguesa de estomatologia, medicina dentária e cirurgia maxilofacial, 2022-01, Vol.63 (2), p.99-104

Ort / Verlag

Sociedade Portuguesa de Estomatologia e Medicina Dentária

Erscheinungsjahr

2022

Quelle

Electronic Journals Library

Beschreibungen/Notizen

• 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.