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Peripheral N lesions

Benign and Malignant Peripheral Nerve Sheath Lesions

QuestionAnswer
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
Created by: celinapyune