• Often associated with neurofibromatosis
• 4% of MPNST show perineurial cell differentiation (Am J Surg Pathol 1998;22:1368)
• Often spreads to pleura or lungs

• Extremely poor prognosis if glandular or rhabdomyosarcomatous features

• 50 year old man with 13 cm tumor (Singapore Med J 2009;50:e199)
• 51 year old man (Respiration 2000;67:346)
• 56 year old man with neurofibromatosis type 1 (Surg Today 2008;38:945)

• Unencapsulated, degenerative changes common (fat, hemorrhage, cysts)

• Bizarre cells and tumor giant cells
• Also atypical mitotic figures and necrosis
• May have areas of uniform bland spindle cells
• Rarely has rhabdomyosarcomatous features (“triton tumor”) or glandular differentiation

Low-grade tumor has increased cellularity, nuclear atypia, scattered mitotic figures

Fascicular proliferation of atypical spindle cells

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