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Peripheral N lesions
Benign and Malignant Peripheral Nerve Sheath Lesions
Question | Answer |
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Different tumors contributed to Peripheral Nerve Sheath lesions | Range of well-defined entities, ranging from benign tumors, such as schwannoma and neurofibroma to high grade malignant neoplasms termed malignant peripheral nerve sheath tumors (MPNST) aka neurofibrosarcoma. |
Syndrome associated with Peripheral Nerve Sheath lesions | Von Recklinghausen Neurofibromatosis :an inherited disorder that has an autosomal dominant inheritance pattern. Caused by a mutation in the NF1 gene on chromosome 17. Common findings: café au lait spots, abnormal bone development, lisch nodules |
Clinical features and demographics for Peripheral Nerve Sheath lesions | Neurofibroma: benign neoplasm; smooth-surfaced soft, nonulcerated nodules; common in young adults. Neurofibrosarcoma: lobulated firm mass with ulceration & bone destruction; 29-36 yo |
Microscopic features of Peripheral Nerve Sheath lesions | Neurofibroma: Well circumscribed interlacing bundles of spindle-shaped cells, wavy nuclei in a fibrous stroma, haphazard arrangement, and may see nerve at edge. MPNST: dyplastic spindle cells with few recognizable nerves |
Differential diagnosis of Peripheral Nerve Sheath lesions | Neurofibromatosis, Melanocytic nevus, Neuromas, Soft fibromas, Acrochordons, Lipomas, Dermatofibromas, Sarcomas |
Treatment for Peripheral Nerve Sheath lesions | Neurofibroma - Conservative surgical excision Malignant peripheral nerve sheath tumors - Radical surgical excision, possibly with adjuvant radiation therapy and chemotherapy |