Monday, November 28, 2011

Is It Cancer? MPNST and Neurofibromatosis

No one wants to hear the word Cancer directed at them, or anyone for that matter.  But for those of us with risk factors, we must realize the possibility of it happening.

Neurofibromatosis carries with it a number of issues; neurofibromas,  pigment changes in the skin, skeletal anomalies, and learning disabilities.

Although neurofibromas are benign tumors, malignant peripheral nerve sheath tumors (MPNST) sometimes occur. MPNST, in the past also referred to as “malignant schwannoma” or “neurofibromosarcoma,” and can occur in the general population but is one of the hallmark complications of NF1.

MPNST, typically forms from unexpected growth of a preexisting neurofibroma, particularly a plexiform neurofibroma, the first symptom is typically unexplained or sudden pain, in the area in or around existing tumors.

Symptoms may include:

  • Swelling in the extremities (arms or legs); the swelling often is painless.
  • Difficulty in moving the extremity that has the tumor, including a limp.
  • Soreness localized to the area of the tumor or in the extremity.

The thing to remember is that just because you have a higher risk in developing cancer, doesn't mean you will.  Being aware of your body and noting to your doctor any changes you notice is key in staying healthy, and catching things early.  Be aware of your they feel and what they look like.

What is MPNST?
MPNST is also referred to as malignant Schwannoma, neurofibrosarcoma, and malignant neurilemmoma. This type of cancer usually develops in young or middle-aged adults, more often in men than in women. The average age of MPNST patients is between 29 and 36 years. About half of all cases of MPNST develop in people who have Neurofibromatosis.

To treat these tumors, a patient sees an oncologist and a neurosurgeon.  Also, patients can benefit  from being treated by medical teams that specialize in soft-tissue sarcoma tumors. Treatment of MPNST often involves several steps, depending on the location of the tumor, type of sarcoma, other patient circumstances and overall health.


  • There are three main types of treatment for MPNST. These treatments are surgery, radiation and chemotherapy. Doctors often use all three types in combination to create specific individual treatment plans for a patient.

*Surgical Removal

  • The most common treatment for malignant peripheral nerve sheath tumors is surgical resection. Resection of tumors involves the removal of the tumor and surrounding malignant tissue. The doctors analyze the edges of the area removed, and if cancerous cells remain, they remove a little more surrounding tissue. This continues until the tissues the doctors remove are clear of cancer cells.

Radiation Therapy

  • Radiation is the use of specifically directed ionized radiation in a medical setting. Radiation is a common treatment for these tumors, and is often very effective at different stages. Preoperatively, radiation can reduce the size of a MPNST, making surgery easier for the doctor and therefore reducing the time spent under anesthesia. Radiation helps doctors achieve clear borders without having to cut out more tissue, which is very important when the tumor is in a peripheral area such as an arm or leg. Clearing the borders without going deeper often saves the patient from an amputation. Radiation can also destroy cancer cells that surgery couldn't remove.  (Radiation is sometimes used as a "last resort" with people with NF, because it has been studied that Radiation can make the symptoms of Neurofibromatosis worse.  Your doctor will decide what is best for YOU and your situation


  • While chemotherapy is not particularly effective at treating localized MPNST, doctors often use it to treat cancer that has spread to other areas. Chemotherapy, taken either orally or intravenously, involves taking medication that kills cells.

I didn't want to start the week with a negative post that scares people, but MPNST happens....And the more you know about it, the better chances you will have at catching it sooner.

MPNST is rare...But if you arm yourself with knowledge, IF it does happen to you, your outcome will be much more positive.

Even when it's scary....Even when it's overwhelming...A Positive Attitude is key in fighting ANY battle!  Remember, it's E.A.S.Y. to THRIVE; EDUCATE yourself-Watch your ATTITUDE-SHARE your stories-and YIELD to the possibility that anything is possible!



  1. Love reading your blog. I'm one of the rare people who got sarcoma with my NF. I am an eight year survivor! I was diagnosed with NF when I was 11 and just turned 40. I have two children, both were tested for NF and neither one has it. I am not sure where my NF came from I'm the only one in my family that has it.

  2. I am presently fighting the battle of MPNST. I have had my left pubic ramus bone removed as well as two lesions at the original biopsy site,a lesion in my lung removed and just today a new plan as I have a new mpnst in my left ilium bone. I have had some radiation but I will now get more radiation and chemo for the first time. Its scary but I will not give up or give in and everyday I stay thriving with a positive attitude. I am blessed with great doctors and a great family and friends. My mpnst diagnosis came at age 53 so I think I should change my middle name to rare. It does sort of pop up out of nowhere. So do be aware of your body and the constant changes that NF gives you each day and never be afraid to ask your doctors to take a look via CT scans if you feel something is not quite right. And keeping a good attitude doesn't mean I don't have my moments when I cry but its ok to feel those moments too and then carry on to win the battle.

  3. I was told by my doctor that if I do not do something about these strings growths in my colon I could get cancer my father had nf and died of lung cancer, he had prostate cancer before lung cancer. I met a man who had a plexi became cancer. Going back to my father he led a long eventful life working hard to support his family and was in the service for several years in service of our country.

  4. My son was 12 years old when this nasty MPNST cancer took his life, he battled it for 2 years. He had 2 surgeries 8 weeks of radiation and 14 total cycles of chemo, and noting worked. It took his life way to soon, CANCER SUCKS. I pray that they find a cure before another child or adult gets told that they have it.

  5. i am very concerned about my fiance. he has mpnst behind his left eye which bleeds.the dr.told him its in stage 2.i am unaware of how to go about this.4 health insurance companies have dropped him due to this illness.can someone please give me some guidence on this i dont want to loose the love of my email is thank you.

    1. Unbelievable that he was dropped from insurance--New reports say that EVERYONE is covered....despite pre-existing conditions. Something is not right and you should be on the phone with the ins. company.
      I wish u guys the best....Make sure u see if he can qualify for disability--or state assistance medical-

  6. I was just diagnosed with MPNST this week (inner left thigh) , arising from a slow growing Neurofibroma I have had for years. I had it resection and clear margins (though small because it was in my leg next to nerves and blood vessels).
    Treatment is complicated.

  7. I had a mpnst present itself in my great toe. It was amputated this past December. I have my first follow up next month. Getting a little nervous. I still feel like I know very little about this type of cancer, the more I read on the internet the more confused I seem to get. I don't have nf1... Just randomly appeared.

  8. I did not have nf1 either and literally the same thing as you happened to me. It came in my big toe and they amputated it to. So far ive been fine. Its been two years now. But as a side note if you were a flip flop wearer like me look into sazzi five toed flip flops. I never thought id wear flip flops again and they are cute and comfortable and keep my feet in. You would never know the diffrence.

    1. How do you know you have it in your big toe? I'm afraid I might be the same =(


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