Nerve Sheath Tumors

Neurofibroma, which is the most common nerve sheath tumor, is a slow- growing tumor that includes
an admixture of endoneural fibroblasts, Schwann cells, and axons. Neurofibromas may be circumscribed
but are not encapsulated. They are firm and rubbery. Microscopically, the spindle- shaped cells are
arranged in ribbons and cords in a matrix of myxoid tissue and collagen that contains axons. Cytogenetic
studies indicate that the most frequent structural rearrangements involve chromosome arm 9p.

Isolated neurofibromas do not necessarily indicate systemic involvement, but the plexiform type of
neurofibroma is associated with neurofibromatosis  1 (Fig  14-14). “Plexiform” refers to an intricate
network, or “plexus,” classically described as a “bag of worms.” Studies indicate that a limited number of
pathways are potentially involved in tumorigenesis of the plexiform neurofibroma. The CCN1 gene may
be a useful diagnostic or prognostic marker and form the basis for novel therapeutic strategies. The
CCNs (cysteine-Rich proteins) have been shown to occupy key roles as matricellular proteins, serving as
adaptor molecules that connect the cell surface and the extracellular matrix.

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