Ask Kate: Plexiform Neurofibroma/Optic Glioma/MPNST/Chemo

Post date: Jun 18, 2016 7:42:51 PM

Ask Kate: Plexiform Neurofibroma/Optic Glioma/MPNST/Chemo

Transcripts 2.10.16

All questions are posed by event participants & answered by Kate Kelts, RN unless otherwise noted

Question: What are the current options to treat a plexiform neurofibroma of the brachial plexus? Are there any clinical trials involving these types of tumors? What are the possible complications from a plexiform neurofibroma in this area?

Answer: When it comes to finding out what studies are currently happening for any area of NF the clinicaltrials.gov website is where I look. You search for your specific question.

Question: Sorry if this is dumb question, but how do you know if it’s a plexiform or just a subdermal fibroma? My son just turned 8- I have noticed numerous under the skin bumps on his back along his spine in the last few months- last night I felt a good size growth along the underside of his forearm. I don’t want to be an alarmist, because I know he is going to develop tumors, but the size and consistency of these differ significantly from the bumps I feel on his head, which feel more grainy and moveable. I apologize if this is a little off topic but would appreciate a response. Thanks!

Answer: Subdermal neurofibromas often feel very different than other types of neurofibromas. A softy, squishy texture is common and not necessarily alarming. I'd advise speaking with your NF doc at his next visit and see what he/she has to say. They can always order a scan if there is cause for concern. Not a dumb question! Basically different types of neurofibromas will feel different and vary in texture, but it is always good to point out any new tumors to his provider.

Question (continued): Thanks- I think I am alarmed that they all seemed to appear in a relatively short time frame- this is the longest he has gone without an appointment, 5 months instead of 3 months, and he keeps asking when he's going again, because he says his bumps are hurting at recess and in gym class- his 2 favorites.

Answer: Neurofibromas can definitely cause some pain, especially if they're being pressed on or hit in any way. I understand your concern, you can always call his provider now if you just want some reassurance, but I think waiting for his next appt will be fine also.

Question: my son has a plexiform in his lower trunk.. According to his doctors it remained stable from first MRI at 6 weeks and was reevaluated at 2 years. His latest MRI did not call for a scan of the area.. Should

It still be scanned every 2 years? I have concerns on how we would know if it was still stable

Answer: I can't see any reason why you wouldn't repeat the scan every 1-2 years. There is very low risk with MRI, of course if he needs sedation there is some risk there, but I would revisit this with your provider & see if they can explain clearly why they wouldn't rescan.

Question (continued): From what I was told it was because it was stable for the last one.. I think personally they forgot to order it... If that's possible

Answer: Stable is good, but I would definitely follow up, it's absolutely possible for things to be overlooked. This is why we need parents as advocates, medical personnel are fallible and often overwhelmed with work, never be afraid to speak up and ask for explanations you can understand.

Question: We have been told that our 13 yo son has plexis on his r and l sciatic(both thighs) - cannot see them without MRI (look like a long bunch of grapes in the MRI to me)....question - since he has gone through puberty is the worst over with growth? Or do they still grow post puberty? He has some pain/numbness etc but in the grand scheme of things not bad - I know the neurosurg told us he would not touch the tumour unless they had absolutely no other choice due to its location etc....just dont completely understand the plexiform tumours and growth.

Answer: Okay, with Plexiforms you can see increased rate of growth during puberty, but unfortunately they can continue to grow after that point. Each case is unique, I'm sorry I cannot give you a more definitive answer.

Question: Five year old daughter has plexi form on her right thigh. It is growing and causing pain just from walking. What can be done to help her?

Answer: Pain management is important, ask your providers about a pain team near you. Also, what type of physicians is she seeing? Is she being seen in an NF clinic?

Question (continued): She hasn't been to an nf clinic yet as one just opened up in Toronto and we are waiting on referral. She is still followed by her oncologist and nurse as she has Mris every three mths still. I will ask about pain management. So far they pretty much tell me there's not much we can do. I have been following some clinical studies for plexi forms but nothing promising so far.

Answer: Yes, I definitely recommend pain management for these kids. And that isn't just medication, a pain team can provide a lot of different options for improving quality of life.

Question: I hear a lot of stories about tumor resections for plexiforms. What are the indications for surgery? Why is there such a hesitation to remove Plexiforms? We have been told one of our daughter's plexiforms is operable, but the surgeons keep telling us to wait despite the fact that is is growing and causing pain. Also, has there been any success in using Chemotherapy post Plexiform resection to prevent any regrowth?

Answer: There is hesitation to operate on Plexiforms because they are so complex. They involve all of the vital structures they touch, wrapping around muscles, nerves, and vessels. Often resection can result in a loss of function or other new symptoms and because we cannot effectively prevent regrowth at this point they will likely regrow. It is a matter of weighing risks & benefits and each case will be very different. Where the Plexiform is and what it involves are important things to consider of course. At this point we have not found a "homerun" drug, chemo or otherwise, that prevents regrowth of Plexiforms. However, there is research happening all the time, much of which is being funded by CTF.

Question: What are you're thoughts on prominent bilateral tourtosity of the optic nerves? Would you consider these gliomas or not? His left eye has started to intermittently drift inwards, should we be worried or could this just be a sign he needs glasses and has nothing to do with the optic nerves (he is 4)?

Answer: They are sometimes referred to as pseudotumor cerebri, so they are not gliomas and treatment is quite different in my understanding. Also, prognosis can vary. Are you seeing a pediatric ophthalmologist?

Question (continued): Yes, and a neurologist. The ophthalmologist called them gliomas , but neurology said no because it didn't refer to them as gliomas on the MRI report. We are just confused on what it is and what his eye drifting means. The also started measuring his UBOs, what does this mean?

Answer: UBO's are "Unidentified Bright Objects", almost every brain with NF1 will have UBO's, they have not been connected with ANY health issues including learning disabilities and disappear by the time a child reaches adulthood. I've had patients where there is confusion as to what to call this, but if the MRI says tortuosity then it's not a glioma. This article might be helpful: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3904184/

Question: Two questions: 1) My son has pre-chiasmal glioma behind one eye. He is almost five and it has not grown. Is there an age where if it has not grown it likely will not, or is there really always a possibility that it will start growing? 2) Are the beginnings of plexis always evident from a young age? Or can they spontaneously pop up later in life? Just wondering if he doesn't have any now if that will always be the case or not. Thanks!

Question (continued): I would like to follow this too. My daughter has bilateral OGs and she's going though chemotherapy to shrink them because her vision is affected by them. I wonder too if there's an age she hits where we won't have to worry about regrowth? I know she will have lots of MRIs in her day....

Answer: Great questions. 1: Generally if we do not see growth by the age of 7 we are optimistic that it will not grow and in some cases may disappear. This is not a guarantee but it is statistically likely.

2. Plexiforms are believed to be congenital, which means they are there when a child is born. However in many cases they are SO small they can go unnoticed for years. They may appear as a CAL spot that is has some texture or hair growing from it, or they may be internal and unseen until they cause symptoms or an MRI is done for other reasons.

Question: Is there any reason to get any kind of scan to detect them early before they cause problems?

Answer: There is new technology that allows for a full body MRI and some NF patients are pursuing this as an option for identifying tumors in their earliest stages. However, due to the nature of plexiforms we are still not able to surgically remove them entirely, no matter when they are discovered and they are highly likely to regrow.

Question: How often should you be scanned (she’s 11 )if you've had a MPNST in 2012 that was just debulked with chemo and proton in neck ,(still there) and optic nerve glioma of the chiasm in 2014 with the full year of chemo

Answer: I apologize but I really cannot answer this question. With such a complex history of chemotherapy & tumor growth but without actually being involved in her care I can't advice on specific medical treatment. I assume she's being followed by NF doctors, an oncologist, neurologist? If you have concerns about her care please feel free to email me directly at kkelts@ctf.org