Definition

• a malignant peripheral nerve sheath tumor (MPNST) with rhabdomyoblastic differenciation

Synopsis

• sporadic or in the setting of neurofibromatosis 1 (NF1)
• scattered rhabdomyoblasts in a stroma identical to ordinary MPNST :
  • usually rather mature, with abundant eosinophilic cytoplasm
  • cross-striations
  • immunohistochemistry : desmin, MyoD1 and myogenin positive

See also

 malignant peripheral nerve sheath tumor (MPNST)