Malignant peripheral nerve sheath tumor with divergent differentiation.

ABSTRACT

A malignant peripheral nerve sheath tumor (MPNST) is an uncommon spindle cell sarcoma accounting for approximately 5% of all soft tissue sarcomas. A 55-year-old female with a right suprarenal tumor showed MPNST with additional foci of epithelioid, rhabdomyoblastic, osteogenic and lipogenic differentiation. Although the capacity of MPNST to undergo epithelioid, rhabdomyoblastic, osteogenic and very rarely lipogenic differentiation is reported in literature, the occurrence of all these differentiation in one case has not been described in literature before. To the best of our knowledge, this is only the second MPNST case with lipomatous differentiation.