DISEASES

Disease-gene associations mined from literature

Human genes for neurofibrosarcoma

Neurofibrosarcoma [DOID:3512]

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:  neurofibrosarcoma,  DOID:3512,  Neurosarcoma [obs],  Neurosarcoma obs