- Be iNFormed
- Cancer
- NF Type 1
- NF1 Tumors
Andrea Gross, MD
Learning about Malignant Peripheral Nerve Sheath Tumors (MPNSTs) is an important step for anyone affected by NF1. While they are a serious concern, understanding the signs, symptoms, and treatment options can empower you and your family. This page provides a brief summary of information from an article by Dr. Andrea M. Gross of the National Cancer Institute.
What is an MPNST?
Malignant Peripheral Nerve Sheath Tumors are a type of aggressive cancer known as a sarcoma. For people with NF1, these tumors most often develop from an existing benign plexiform neurofibroma. The lifetime risk of an individual with NF1 developing an MPNST is between 8 and 13%.
Key Symptoms to Watch For
The most common symptom of an MPNST is pain in the area of a tumor. This could be a new pain or a noticeable worsening of existing pain from a known plexiform neurofibroma. Another sign can be a visible tumor that begins to grow more quickly than it has in the past.
In someone with NF1, new pain, worsening of existing pain and especially pain that wakes one up from sleep, should never be ignored!
Andrea M. Gross, MD, National Cancer Institute
Diagnosis and Treatment
If an MPNST is suspected, a doctor will likely use imaging tests like an MRI or a PET scan to get a better look. However, a biopsy is typically needed to confirm a diagnosis. It’s important to work with a doctor who is familiar with NF1. Treatment plans are unique to each person but often involve a combination of surgery, radiation therapy, and chemotherapy.
Download the Full Article
For a more detailed explanation of MPNSTs, their causes, and treatment, you can download the full resource from Dr. Gross below.