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| Malignant peripheral nerve sheath tumor ： ウィキペディア英語版 |
Malignant peripheral nerve sheath tumor
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.
The first-line treatment is surgical resection with wide margins. Chemotherapy (e.g. high-dose doxorubicin) and often radiotherapy are done as adjuvant and/or neoadjuvant treatment.
== Classification ==
Malignant peripheral nerve sheath tumors are a rare type of cancer that arise from the soft tissue that surrounds nerves. They are a type of sarcoma. Most malignant peripheral nerve sheath tumors arise from the nerve plexuses that distribute nerves into the limbs—the brachial and lumbar plexuses—or from nerves as they arise from the trunk.
抄文引用元・出典: フリー百科事典『 ウィキペディア（Wikipedia）』
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