A 63-year-old man was admitted at the emergency department in an acute confusional state, and a computerized tomography scan (CT) revealed hydrocephalus. Despite the rapid introduction of a ventriculo-peritoneal shunt, the patient died soon thereafter. At the post-mortem examination, a large solid mass was found to be firmly attached to a nerve root at the L1-L2 level. Microscopically, the tumor showed the classical features of a malignant peripheral nerve sheath tumor (MPNST) with rhabdomyoblastic differentiation. In addition, there were randomly distributed lobules of well-differentiated adipose tissue with unequivocal atypical nuclei. The diagnosis of MPNST with rhabdomyoblastic and lipoblastic differentiation was made. We are not aware of previously reported lipoblastic differentiation in MPNST. Herein, we present details of the case.
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