Associated with neurofibromatosis, type 1, with mutation in NF1 locus and disruption of neurofibromin production
Usually arise de novo, rather than from preexisting neurofibroma.
Proliferation of Schwann cells,
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.
Cotran RS, Kumar V, Robbins SL: Robbins Pathologic Basis of Disease. 5th ed. Philadelphia, W.B. Saunders, 1994, pp. 1353.
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||Synopsis by: Melinda Sanders M.D, (TX0500M95403)