Is anyone here aware of any info on Ewings Sarcoma.
Where can I get more details (any help would be appreciated)
Thanks
God Bless You

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Originally posted by 7seasfar

Is anyone here aware of any info on Ewings Sarcoma.
Where can I get more details (any help would be appreciated)
Thanks
God Bless You

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Thanks for the link.

I am more interested to know more about the chemotherapy protocol, & the way it affects the cancer tissue. & other possible side effects (& how to deal with it, so the side effects can be minimized).

What else can we do to ensure 100% recovery?

Here is an article in detail for you. Please read the complete article and on its treatment and how it gets eradicated, if discovered in its early stages.

WHAT IS ESFT and the Causes.: TECHNICAL EXPLANATION.
Genetic exchange between chromosomes can cause cells to become cancerous. Most cases of Ewing's sarcoma (85%) are the result of a translocation between chromosomes 11 and 22, which fuses the EWS gene of chromosome 22 to the FLI1 gene of chromosome 11. EWS/FLI functions as the master regulator. Other translocations are at t(21;22) and t(7;22).

Treatment in Detail.:

Currently, the treatment of all Ewing's sarcoma (both soft tissue tumors and bone tumors) is the same. Based on the results of a number of clinical trials, the first line treatment is quite standardized and consists of:

14-17 cycles of chemotherapy, alternating between 2 regimens of drugs
Resection surgery if possible, which frequently involves limb-sparing surgery with prosthetic reconstruction or donor bone grafts if there is bone involvement
Daily radiation treatments for 6 weeks to the primary site may be required if complete surgical resection is not possible
Ewing's sarcoma is an aggressive cancer, and requires 9 months to a year to treat in the best case. If the cancer doesn't respond to "first line treatment" and if there is known disease, there are other drugs to try. If these don't work, the patient may be a candidate for a clinical trial.

Advances in chemotherapy (CTx) have significantly improved survival. Surgical removal of the primary tumor generally occurs after a course of initial chemotherapy. Chemotherapy is started first to attack any potential tumor cells that have broken off from the main tumor (metastasized) but have not yet been detected by the staging studies. Furthermore, this gives the surgeon an opportunity to better plan his or her surgery, which can be very involved, see Figure 4.

Surgery is then followed by further chemotherapy which may be adjusted depending upon the response of the tumor to the drugs. If the tumor has been highly responsive to the drugs a better outcome may be predicted in some cases. In ESFT, radiation therapy may be used in conjunction with or instead of surgery depending upon location and extent of disease. While all aspects of treatment have improved dramatically over the past 30 years, it is very intensive therapy. Treatment generally lasts a year. In essence, an ESFT patient and his/her family is giving up one year of life to hopefully get the rest of them back.

Chemotherapy and Systemic Drugs Used in the Treatment of ESFT
Chemotherapy (CTx) is a critical part of the treatment for ESFT (Wexler 1996; Ludwig 2008, Balamuth 2010). Prior to CTx, the majority of patients, up to 90%, died when radiation and/or surgery were used exclusively. Certain drugs (cyclophosphamide, actinomycin-D and vincristine) were used in the 1960’s to treat ESFT patients and survival improved. Over the past three decades new chemotherapy agents have been developed and the dosing regimens of the different agents have been refined. A five drug regimen of vincristine,doxorubicin, cyclophosphamide, ifosfamide, and etoposide is now most commonly used in the United States and Europe (Ladenstein 2010).

Chemotherapy treatment of ESFT differs some based on whether the tumor has spread (metastatic) at diagnosis. All of the below studies were conducted with patients with localized (non-metastatic disease). The results of the Intergroup Ewing Sarcoma Study III randomized patients to treatment with three drug therapy (with vincristine, doxorubicin, and cyclophosphamide) versus five drug therapy (with ifosfamide and etoposide in addition to the above agents). As reported in the New England Journal of Medicine, patients receiving five drug therapy had improved survival compared to those receiving three drug therapy (survival 72% vs. 61%, p = 0.01) (Grier et al., 348:694-701). This study defined the five drug regimen as the standard chemotherapy regimen for Ewing sarcoma family of tumors. Typically, these agents are given as the combination of vincristine, doxorubicin, and cyclophosphamide (or VDC) over 2 days followed by ifosfamide and etoposide (IE) given over 5 days. The two combinations (VDC and IE) are traditionally alternated every three weeks.

More recently, investigators have studied "dose intense" chemotherapy regimens in an attempt to further improve outcome. Dose intensification means that the same total amounts of drugs are given, but they are administered in a more intense fashion. Dosing regimens may be intensified by increasing the amount of drugs given per interval, shortening the intervals at which the drugs are administered, or both. A Children’s Oncology Group study was performed to see if the standard 5 drug regimen could be given in a "dose intense" fashion by increasing the dosages of the agents while maintaining the dosing intervals at 3 weeks. Although this trial did not show an improvement in survival for those patients treated on the dose intensified arm, it did prove that toxicity was similar between patients treated for 30 weeks versus 48 weeks (Granowetter 2009). The more recent Children’s Oncology Group Trial (AEWS0031) has shown that an every-two-week interval compression regimen is superior to the every 3 week regimen in both event-free and overall survival: 76 versus 65 percent event free survival at 4 years (p=0.029), and 91 versus 85 percent overall survival at 4 years (p=0.026). There was also no difference in toxicity between the two regimens. Now, the standard agents above are commonly used in a dose intensified approach in order to decrease length of therapy while maintaining survival. Other data from this large trial is still being analyzed (Womer 2008).

About 15% of patients will have metastatic disease at diagnosis, and this accounts for the most adverse prognostic factor in ESFT. Patients who have metastasis only in the lung (in addition to their primary tumor) seem to perform better than those with disseminated (or many sites of) disease. For patients with metastasis of disease at time of diagnosis, the standard five drug chemotherapy agents are used for front line therapy. However, as metastatic ESFT is more difficult to treat, high dose chemotherapy with autologous stem cell rescue is sometimes employed in this group of patients. Melphalan and busulfan are active agents against ESFT and have been used with some effectiveness in the context of autologous stem cell transplants for advanced cases of Ewing sarcoma family of tumors. However, the extent of myelosuppression they induce renders their use prohibitive in routine clinical settings. A 2006 study from a single institution in United Kingdom reported 38% five year survival with these agents in combination with bone marrow transplantation in advanced cases of ESFT (McTiernan 2006). Results of the European trial for patients with disseminated disease (Euro-EWING 99 trial) are now available and show that these patients have an event free survival of 27% with an overall survival of 34% at 3 years (Ladenstein 2010). Of the patients that went to high dose chemotherapy with stem cell rescue, 57% and 25% had a complete response or partial response, respectively. This trial confirmed that patients with more disease at diagnosis and those with larger primary tumors at diagnosis do not do as well as those with smaller, localized tumors. This helps researchers and clinicians continue to learn about how to best treat patients with different variations of the same type of tumor.

Researchers and clinicians continue to focus on new, innovative treatments for patients with ESFT. A new trial from the Children’s Oncology Group that will add topotecan to the current standard medication regimen for patients with localized disease is slated to open within the next few months. Currently, there are ongoing trials to look at the possibility of using irinotecan and temozolomide for patients with advanced ESFT (Wagner 2007, Casey 2009). In addition, new biologic agents work to target specific cancer cells in the body, rather than killing any rapidly growing cells in the body making this treatment much more specific and likely to cause fewer side effects. Treatments such as insulin-like growth factor receptor antibodies (IGFR-1) are being used to target Ewing sarcomas (Olmos 2010; Toretsky 2010). Some of the more promising new treatments will be discussed in the final section of this review

For further information about chemotherapy protocols, the reader is referred to the chemotherapy references at the end of this paper.

http://sarcomahelp.org/ewings-sarcoma.html

http://en.wikipedia.org/wiki/Ewing\'s_sarcoma


FH

Adriamycin (Doxorubicin) has potential cardiac toxicity. Is there anything which can be done to protect that from happening, more like a cardiac protector.
The protocol being followed is-
VAC for 3days then 3weeks rest followed by IE for 5days then 3weeks rest, this process is to be repeated for couple of cycles, along-with monitoring the progress.

Adverse effects [edit]

The most dangerous side effect of doxorubicin is heart damage. When the cumulative dose of doxorubicin reaches 550 mg/m², the risks of developing cardiac side effects, includingCHF, dilated cardiomyopathy, and death, dramatically increase. Doxorubicin cardiotoxicity is characterized by a dose-dependent decline in mitochondrial oxidative phosphorylation. Reactive oxygen species, generated by the interaction of doxorubicin with iron, can then damage the myocytes (heart cells), causing myofibrillar loss and cytoplasmic vacuolization.

Another common and potentially fatal complication of doxorubicin is Typhlitis, an acute life-threatening infection of the bowel.

Acute adverse effects of doxorubicin can include heart arrhythmias, nausea, and vomiting. It can also cause neutropenia (a decrease in white blood cells), as well as completealopecia (hair loss).

A more mild side effect is discoloration of the urine, which can turn bright red for up to 48 hours after dosing.

Additionally, some patients may develop PPE, characterized by skin eruptions on the palms of the hand or soles of the feet, swelling, pain and erythema.
Due to these side effects and its red color, doxorubicin has earned the nickname "red devil" or "red death."

Chemotherapy can cause reactivation of hepatitis B, and doxorubicin-containing regimens are no exception.
Doxorubicin and several chemotherapeutic drugs (including cyclophosphamide) cause dyspigmentation. Other groups of drugs that cause this problem include antimalarials, amiodarone, heavy metals (but not iron), tetracyclines, and antipsychotics.

http://www.uic.edu/classes/pmpr/pmpr652/Final/bressler/chemocardiac.html

http://link.springer.com/article/10.1023/A%3A1007094214460#page-1

Here they are trying to eliminate ESFT taking a calculated risk. Most of the articles that I can access are from the web. The more defined ones are available at a cost.

It is a team that decides upon the treatment and under a constant watch and steady monitoring and I can only suggest that you go along with the team. It is a nail biting decision and it is for the betterment of the patient and for a long term well being.

Don't Look back.

Good Luck.


FH

God bless and my best wishes. You will be in my prayers.
This is what we did...
http://www.chrisbeatcancer.com" rel="nofollow">LINK

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