Overview
A malignant peripheral nerve sheath tumor (MPNST) is a very rare type of soft tissue sarcoma. In MPNST, there are malignant (cancerous) tumors in the sheaths or layers of tissue that protect nerves in your peripheral nervous system. You can have this type of tumor in your arms and legs, but it may also affect your pelvis, abdomen, head and neck. Removing tumors with surgery can cure the condition, but the tumors often come back (recur).
How rare is an MPNST?
It’s very rare. Each year, about 1 in 1 million people learn they have a malignant peripheral nerve sheath tumor. This condition typically affects people aged 30 to 50.
People who have the inherited disorder neurofibromatosis type 1 (NF1) often develop MPNST. Between 25% and 50% of people who have a malignant peripheral nerve sheath tumor also have NF1.
In general, people with NF1 develop MPNST earlier than people who don’t have NF1. And NF1-associated MPNST is somewhat more common in men and people assigned male at birth (AMAB) compared to non-NF1 MPNST.
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