Malignant peripheral nerve sheath tumor with rhabdoid features arising in the temporal region of a patient suffering from neurofibromatosis 1 - A case report
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. I...
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| Main Authors: | , , , |
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| Format: | Book |
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Sociedade Portuguesa de Estomatologia e Medicina Dentária,
2022-06-01T00:00:00Z.
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| Summary: | 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. |
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| Item Description: | 10.24873/j.rpemd.2022.06.867 1646-2890 1647-6700 |