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| Neurofibrosarcoma |
| Etiology • Associated with neurofibromatosis, type 1, with mutation in NF1 locus and disruption of neurofibromin production • Usually arise de novo, rather than from preexisting neurofibroma. |
| Pathogenesis • Proliferation of Schwann cells, |
| Epidemiology • May be sporadic • Frequent complication of neurofibromatosis, type 1. • Disease of adults; predominantly associated with peripheral nerves (not cranial). |
| General Gross Description • Associated with a nerve which disappears into the neoplasm • Poorly circumscribed, fleshy, frequently necrotic and hemorrhagic lesion |
| General Microscopic Description • Cellular, pleomorphic, mitotically active neoplasm arranged in fascicles • Cells have spindly, sometimes wavy nuclei • Epithelial and other mesenchymal components such as skeletal muscle, cartilage or bone may be seen. • S-100 positive. |
| Clinical Correlation • Aggressive neoplasms requiring surgical and/or radiation therapy for local disease • Metastasize via the blood stream to lungs mandating multi-agent chemotherapy • Prognosis worsens with tumor size, tumor grade, and age of the patient • Location may also have an impact on resectability. |
| References • Cotran RS, Kumar V, Robbins SL: Robbins Pathologic Basis of Disease. 5th ed. Philadelphia, W.B. Saunders, 1994, pp. 1353. |
| Neurofibrosarcoma |
| Synopsis by: Melinda Sanders M.D, (TX0500M95403)[554] |
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