Cure Alveolar Soft Part Sarcoma International (iCureASPS)

Hi to everybody. We are fighting against ASPS for 3 years now. Here is th story of our personal nightmare:
Our daughter Mona was 2 3/4y when she started complaining about headache frequently.

March 2004
MRI demonstrated a well-demarcated retrobulbar extraconal vascular tumor in dthe right superomedial orbit that measured 28 mm in greatest diameter. It displaced the globe and the optic nerve infralaterally and the lamina papyracea medially.

April 2004
Dr. Rose in London proceeded with a right anterior orbitotomy. He encountered a lobulated tumor that involved the superior oblique muscle.

The diagnosis of ASPS was confirmed by several institutions

Dr. Rose recommended postoperative stereotactic RT to the retrobulbar orbit, orbital apex, and neighboring right ethmoid sinus.
It was then decided to observe Mona.

CT chest, bone scan, bone marrow biopsiy, etc. everything was unremarkable.

March 2005
CT, MRI demonstrated recurrence of the tumor in orbital apex, the right anterior cavernoussinus, the right posterior ethmoid air cells, and the adjacent extradural space of the anterior cranial fossa.

CT chest demonstrated 3 pulmonary metastases, measuring 5 mm each.

We contacted Dr. Liebsch at Boston MGH, to consider Mona for proton radiation therapy. The consensus was:
1, the recurrent orbital ASPS was resectable only with major surgical morbidity
2, a mutilating curgical procedure was not indicated in view of the lung metastases
3, there was no effective chemotherapy with a proven track record
4, stereotactic RT should be offered to achieve loco-regional control of the orbital ASPS
5, the pulmonary metastases should be resected of restaging warranted their removal.

We opted for proton RT in Boston at MGH. The plan:
HIgh-dose RT to achieve local control of the recurrent orbital ASPS. USE 3D treatment planning and proton RT to maximize the dose to the tumor and possible microscopic tumor extensions and minimize the dose to adjacent critical anatomical structures.

Treatment from 5/5 - 6/28/2005 over 54 calendar days. Total dose of 76.0 Cobalt-Gy-equivalent was administered in 38 fractions of 1.82 proton-Gy each, utilizing the 235 MeV proton beam at the NOrtheast Proton Therapy Center.

MOna tolerated the treatment well with minimal acute side effects. Her energy was good, her appetite was normal and her weight remained stable.

CT, MRI Jan 2006
showed no growth of tumor. The metastases were remained stable

April, Mai 2006
resection of the metastases in two operations (left/right side)

Feb 2007
MRI, CT : no growth of tumor, no metastases

Mona will turn 6 years in Mai and is looking forward to going to school in September.

Hi Julia, I am glad to see that you are finally able to post here. The story is fascinating, my question is - are there no proton radiation treatment avail. in Germany (you live in Germany, right?). I am not that deep about the difference in the physics as what advantages are for the proton versus the usual radiation. Thank you for the sharing the experience with the proton, I knew that on the web-site of the neutron facility (Fermilab) ASPS is shown as neutron sensitive sarcoma so this is one more possible tool to use to kill the unresectable primary and to proceed with the removal of the lung mets. Did you pay for the treatment in Boston out of pocket?

Yes, Olga, we live in Germany. There is only one proton beam institution available in Switzerland (PSI, Villingen), which is able to treat tumor masses in the head of children, who need anesthesia during radiation. Back in 2004 their experience for children of Mona´s age was n=1. Other institutions in Berlin, Munich and Paris treat with proton beam radiation with restriction to certain indications, however the setup did not work for Mona. Therefore we decided to go to Boston, because they are treating children like Mona on a daily bases.
The major advantage of proton beam radiation is the application of a very high dose (Mona got 76Gy; standard radiation 49Gy) on an exact preplanned area. With standard treatment radiation has also be applied to areas around the tumor with a potential drawback to damage this tissue. In Mona´s case, we wanted to apply a very high dose to the tumor (ASPS is not highly sensitve to radiation) without damaging the surrounded tissue (brain, eye) in order to risk major side effects.
Because we could prove that no adequate institution was available for Mona in Europe, our insurance company payed the costs for radiation.