MPNST

[Alive9]

I have a 16yr old daughter who was diagnosed with Malignant perphial nerve sheath tumor of the triton category. she had surgery and now has 100% clean margins. The doctors are recommending Chemo + radiation. I am leary of the Chemo because of the risks in harm to her body. has anyone been in or had experience with this? I am leaning towards radiation only.

[HeinrikMD]

Hi,

Unfortunately this type of tumor is not well-studied- there have been issues regarding classification and hence, the actual benefit of chemotherapy is difficult to measure.

The decision will call for a value judgment.

Using chemotherapy now will likely translate into a longer period of time before the disease returns, but there is no clear translation into cure (this is a very controversial statement I am making - this is because, since this is a rare disease the statistics are not so robust. Further, the few studies performed involve other similar tumor types, not exclusively MPNST, so the merit of the results is difficult to apply to specific cases). This means, the side effects of treatment will be taken, but if she goes through life without the feared relapse - we won't really know if the chemotherapy made any difference. Hence, this approach is aggressive, in the sense that she will be risking more, with an uncertainty of how large a benefit she will be gaining by doing so. Another problem is that when she relapses - she will have less options - because the best drugs have been used for the disease already. Hopefully by then, newer drugs are available - at present there is a promising new drug - but drug development is a lot less for these rarer tumors (put coldly, most pharmaceutical companies wouldn't invest in a drug for a disease affecting only a few).

The alternative scenario is to reserve the chemotherapy when the disease returns  and it is no longer amenable to local treatment (another surgery or radiation). This is a more conservative approach, because there will be a clear indication for chemotherapy, with a measurable goal.

There is no right or wrong answer here, there are only choices based on what you think is acceptable or not.

Hope this post helped more than it confuses.

[Alive9]

Thank you for the info..

When you say she will have less options does that mean chemotherapy can not be administered more than once? With the new promising drug, is this something that is used outside of the US?

[Noelle66]

Hello. My 14 y/o son was diangosed with NF1 at birth (due to the fact that I was diagnosed with it and he has other "hallmarks"). 10/06 he had a 9x12 cm mass removed from his abdomen that was dianosed as an MPNST (11/2/07). The tumor grew back after three weeks of the inital removal. He was on I.V. chemo at the hospital and is currently on a regimine of 3 weeks of Cyclophosphomide (a one week break) and three weeks of Etoposide and the cycle repeats itself. So far the tumor has not grown any more (which means that the chemo is working). This disease *****!!!!!!!!!!!!!!!!!! to put it bluntly. Have faith in God (I know it is easier said than done). But God does not give us more than we can handle. I hope I can be of support to anyone and would like some support in return.

God Bless
Noelle