9561/3 Malignant peripheral nerve sheath tumor with rhabdomyoblastic differentation


Synonyms

MPNST with rhabdomyoblastic differentiation
Triton tumor, malignant
Malignant schwannoma with rhabdomyoblastic differentiation

Definitions

Peripheral nerves and autonomic nervous system
ICD-O-3 topography code: C47
ICD10: C47
           

A malignant peripheral nerve sheath tumor (MPNST) which shows rhabdomyosarcomatous differentiation. More than half of the patients have neurofibromatosis type 1. The prognosis is usually poor.



Connective, subcutaneous and other soft tissues
ICD-O-3 topography code: C49
ICD10: C49
           

A malignant peripheral nerve sheath tumor (MPNST) which shows rhabdomyosarcomatous differentiation. More than half of the patients have neurofibromatosis type 1. The prognosis is usually poor.