DISEASES

Disease-gene associations mined from literature

Human genes for malignant peripheral nerve sheath tumor

Malignant peripheral nerve sheath tumor [DOID:5940]

A malignant peripheral nerve sheath tumor (also known as "Malignant schwannoma," "Neurofibrosarcoma," and "Neurosarcoma") is a form of cancer of the connective tissue surrounding nerves. Given its origin and behavior it is classified as a sarcoma. About half the cases are diagnosed in people with neurofibromatosis; the lifetime risk for an MPNST in patients with neurofibromatosis type 1 is 8-13%. MPNST with rhabdomyoblastomatous component are called Malignant triton tumors.

Synonyms:  malignant peripheral nerve sheath tumor,  DOID:5940,  malignant neoplasm of the peripheral nerve Sheath,  malignant peripheral nerve sheath tumor (disorder),  malignant peripheral nerve sheath tumor (morphologic abnormality) ...