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单词 Malignant peripheral nerve sheath tumor
释义

Malignant peripheral nerve sheath tumor

英语百科

Malignant peripheral nerve sheath tumor

MPNST
Autosomal dominant

A malignant peripheral nerve sheath tumor (MPNST) (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.

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