Malignant Peripheral Nerve Sheath Tumor

My 52 year old husband has had a recurrence of his sarcoma and it has now spread to his lungs and liver. He has more than 30 mets to the lungs alone and is on 75mg of tarceva. We don't know yet if it's going to work but he is getting extremely weak and the change in the past week has been dramatic. Does anyone out there know of any other treatments or clinical trials? He only has one kidney and that presents a problem with many drugs. His doctor has advised that we contact hospice. We are desperately seeking any help anywhere in the world (legitimate medical).

Thank you.

My husband, Phil, had a soft tissue sarcoma in his left tricep that was graded at Stage 1A. After 25 treatments of radiation at Princess Margaret Hospital in Toronto to shrink it, the tumour was surgically removed in March/05. He was given a very positive prognosis - 97% chance of CURE. In July however he found a large tumour in his left armpit. A CT-scan and surgical biopsy confirmed it was an aggressive cancer and also confirmed several small spots on both lungs. We were devastated but Phil is determined to beat this. As we understand it, the new cancers are spindle cell peripheral nerve sheath types but the pathologist was not able to determine the exact kind. We were told that spindle cell cancers tend not to respond well to chemo (20-40% chance of chemo being effective).
Phil has just completed his 3rd round of chemo (treatment protocol = 3 days continuous IV drip of doxirubicin and ifosfamide, then two weeks off; then 3 days of chemo, 2 weeks off, etc.). A CT-scan done after the 2nd round of chemo revealed minimal change in the size of the tumours - but thankfully, they were no larger. The chemo seemed to have slowed down the growth. We were relieved that the growing had stopped but our oncologist was disappointed that there hadn't been a decrease in the size. He noted that there might be some evidence of necrosis (dying of cells in the centre of the armpit tumour).
I would say that our initial excitement has worn off and now we're worried again. We asked the oncologist if he would try tarceva if the combo of doxirubicin and ifosfamide doesn't work; however he said that tarceva is only used if lung cancer is the primary site rather than the result of metastases. Does anyone have experience with tarceva or any other drug working on sarcoma mets in the lungs?

My husband is currently taking tarceva to try and slow the progression of mets to his lungs and liver. He's been on it for 38 days but so far, we haven't seen any responsiveness from the drug. There are several clinical trials going on with tarceva and sarcoma. City of Hope under Dr. Warren Chow, is doing one right now. It's a phase II study. There are several other names that this drug is known--elotinib, OSI-774 and I believe iressa is also the same thing. The drug can be given outside the trial as my husband is doing because he did not meet the clinical protocol. It's a vicious disease and it seemed to grow right through his chemo of ifosamide/mesna, adrymycin/cistplatin and 6 weeks of radiation. His original tumor was removed and at that time (and part of it certainly responded because it was also partially necrotic), there was no spread but less than a year after surgery, it came marching back. I wish you and your husband much luck in fighting this.

Dear "Whatavoice",
Thank you for your informative response. I am so sorry about the recurrence that you and your husband are coping with. My prayers will be with you. This is bloody awful disease.
Would you mind letting us know how your husband fares on Tarceva? My husband has had a limited response to chemo thus far (only 2 rounds), so we are interested in options we may later need to pursue.
We have also commenced treatment with a naturopath - daily injections of extract of European mistletoe, called Helixor; and twice weekly IV injections of high doses of Vitamin C. Both are reputed to boost the immune system (restore white blood cells), reduce the negative side effects of chemo, and attack cancer cells. While on first glance it sounds farfetched, there is impressive outcome data associated with both.
Best to you,
Joan M.

My daughter Lindsay, at the age of 8 was diagnosed with an Orbital MPNST. She went through Radation treatment in 2004. In Feb. this year (2006) The tumor returned. At age 10 our beautiful little girl has has her right eye removed and is under going Chemo. 6 rounds of of Ifosamide and 5 rounds of Doxorubicin. Iv drip three days in the hospital and 18 days home. MRI has shown no signs of reoccurence and Ct scan has shown no increase or movement of a nodule founf in her lung found at initial diagnosis. We have just finished round 4 of her treatments.
I am looking for any information I can find on this terrible disease and entional support as well.

 

On 8/5/2006 Beetlbug wrote:

My daughter Lindsay, at the age of 8 was diagnosed with an Orbital MPNST. She went through Radation treatment in 2004. In Feb. this year (2006) The tumor returned. At age 10 our beautiful little girl has has her right eye removed and is under going Chemo. 6 rounds of of Ifosamide and 5 rounds of Doxorubicin. Iv drip three days in the hospital and 18 days home. MRI has shown no signs of reoccurence and Ct scan has shown no increase or movement of a nodule founf in her lung found at initial diagnosis. We have just finished round 4 of her treatments. I am looking for any information I can find on this terrible disease and entional support as well.

I would love to hear about Lindsay's progress and treatment.  I would love to find out where you are receiveing treatment as well. I am also the mom of a beautiful 15 year old daughter who has recently been diagnosed with a MPNST.  She had a large tumor removed in February 2007 from her right axillary/shoulder area. Her doctors believe that what we were told was just a small calcified cyst when she was 10 (the size of her thumb nail) was actually the start of her tumor.  When her tumor was removed it was the size of a large grapefruit and had grown at a rapid pace within weeks. At this point, no radiation or chemo has been recommended. Thank you!