Sporadic or associated with neurofibromatosis (von Recklinghausen's disease).
Mutation in NF1 gene whose product neurofibromin functions as tumor suppressor
Not proven whether somatic inactivation of second NF1 gene occurs in neoplasms
Proliferation of cells exhibiting predominantly features of Schwann cells.,
Most are solitary cutaneous or in association with peripheral nerve.
Numerous in neurofibromatosis, type 1, particularly plexiform type.
|General Gross Description|
Well circumscribed firm, gray tan dermal mass or
Irregular expansion of a peripheral nerve
|General Microscopic Description|
Proliferation of cells with delicate wavy nuclei
Stroma of plexiform variant often loose and myxoid.
Mast cells and fibroblasts mixed in
Dermal lesions have abundant collagenous stroma
Cotran RS, Kumar V, Robbins SL: Robbins Pathologic Basis of Disease. 5th ed. Philadelphia, W.B. Saunders, 1994, pp. pp. 1352-3.
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||Synopsis by: Melinda Sanders M.D. (TX0500M95400)