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Working in partnership with www.bonecancerresearch.org.uk (Registered Charity No. 1113276)

Ewing Sarcoma Extended Treatment Stages

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Follow the bullet point links below to the relevant subjects, these all link within this one page.
There are also links to other websites that you may find may helpful.

For further information please on these subjects please see our parent information page.

Treatment Option Overview

How the Ewing's family of tumours are treated. It is important for patients to be evaluated by several specialists as early as possible so that treatment may be coordinated effectively from the beginning.

These specialists may include: a radiologist, chemotherapist, pathologist, surgeon, or orthopaedic oncologist and a radiation oncologist. Before treatment decisions are made patients will probably be required to undergo several diagnostic tests including tissue sampling, x-rays, magnetic resonance imaging (MRI) scans, and computed tomography (CT) scans.

There are treatments for all patients with one of the Ewing's family of tumours. Three kinds of treatment are used:

  • Surgery: will involve taking out the cancer in an operation
  • Radiation therapy: involves using high-dose x-rays to kill cancer cells
  • Chemotherapy: the use of drugs to kill cancer cells

For further information about this topic or any other related subject you many fine these two sites very helpful CancerNet and UKCCSG.

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Tests Used

An x-ray of the painful part of the bone is usually able to identify a tumour, although sometimes this can be difficult to see. Other tests are taken to check whether the cancer has spread elsewhere. These include chest x-ray, bone scan and CT* scan.

The booklet 'A Parent's Guide to Children's Cancers' gives details on what the tests are and the scans involve.

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Treatment

Surgery may be required in certain cases to remove the cancer and some of the tissue around it. Surgery may also be used to remove any tumour that is left after chemotherapy or radiation therapy. In some cases the patient may need to have Limb Sparing Surgery (see limb sparing surgery, extended treatment) for further information.

Radiation therapy uses x-rays or other high-energy rays to kill cancer cells and shrink tumours. Radiation for the Ewing's family of tumours usually comes from a machine outside the body (external radiation therapy). Clinical trials are evaluating radiation given inside the body during surgery (intraoperative radiation therapy).

Chemotherapy uses drugs to kill cancer cells. Chemotherapy may be taken by pill, or it may be put into the body by a needle in a vein or muscle. Chemotherapy is called a systemic treatment because the drug enters the blood stream, travels through the body, and can kill cancer cells throughout the body. When more than one drug is given to kill tumour cells, the treatment is called combination Chemotherapy.

For treating the Ewing's family of tumours, surgery or radiation is often used to remove the local tumour and Chemotherapy is then given to kill any cancer cells that remain in the body. It must also be noted that Chemotherapy is not in itself a cure, but we hope we can assist you in understanding this subject and guiding you to any helpful links.

Yahoo Health (we hope you find the information in this site helpful).

The successful treatment of patients with tumours of the Ewing's family (EFTs) requires the use of multi-drug Chemotherapy, in addition to radiation therapy and/or surgical therapy to the primary tumour. 1-6 patients with Metastasis disease at diagnosis, respond well to the therapy given to them (with localized disease0 however, in most cases the disease is only partially controlled or recurs. CT scan (Computed Tomography): Any of several techniques for making X-ray pictures of a predetermined plane section of a solid object by blurring out the images of other planes.

The designations in PDQ, state that treatments are "standard" or "under clinical evaluation" and are not to be used as a basis for reimbursement determinations.

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What is the Ewing Family Tumours?

The Ewing's family of tumours is comprised of bone and soft tissue, small round blue cell neoplasm's of neuroectodermal origin defined by the chromosomal aberration t(11;22) (q24;q12), and closely related variants. Molecular methods now exist to facilitate diagnosis and to defect minimal residual disease.

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Localized Tumours

Molecular methods now exist to facilitate diagnosis and to defect minimal residual disease. Multi-agent chemotherapeutic regimens in concert with adequate local control yield improved survival rates in patients with localized disease at diagnosis.

Treatment for localized tumours of the Ewing's family may be one of the following:

  1. A clinical trial of chemotherapy followed by radiation therapy.
  2. Combination chemotherapy followed by surgery with or without radiation therapy.
  3. A clinical trial of intensified chemotherapy.
  4. A randomized trial of post-surgical chemotherapy with or without stem cell transplant.

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Metastasis Tumours

Patients with Metastasis disease still suffer poor survival rates; programs attempting to cure Metastasis patients with intensive therapy as facilitated by peripheral stem cell and antilogous marrow rescue have shown some promise. Intensive regimens with and without rescue are being explored for high-risk patients.

Treatment options:

Standard treatment with alternating vincristine, doxorubicin, cyclophosphamide and ifosfamide/etoposide combined with radiation therapy to all sites of gross disease and possibly selected surgical excision for patients with Metastasis Ewing's tumour of bone/Ewing's tumour of soft tissue often results in complete or partial responses, however the overall cure rate is 20%.

1-2 for patients with lung/pleural metastases only, cure rates are approximately 30%. Patients who did not receive lung irradiation had a worse outcome than those receiving lung radiation. 3 Patients with only bone/bone marrow metastases have an approximate 20% to 25% cure rate. Patients with combined lung and bone/bone marrow metastases have less than 15% cure rate.

This was obtained from:

Mount Sinai School of Medicine
(Metastic Tumours)
Jack and Lucy Clark
Department of Pediatrics
New York
NY 10029-6574
USA

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Recurrent Tumours

Recurrent Tumours of the Ewing's Family:

The prognosis for patients with recurrent or progressive Ewing's family of tumours (EFTs) is poor, although the prognosis for patients relapsing off therapy is better than for those patients who relapse while on their initial chemotherapy regimen. The selection of further treatment depends on many factors, including the site of recurrence and prior treatment, as well as individual patient considerations. Ifosfamide and etoposide are active in EFTs and should be considered for patients who have not previously received these agents. Aggressive attempts to control the disease, including myeloablative regimens, may be warranted. 2-3 radiation therapy to bone lesions may provide palliation. Residual disease in the lung may be surgically removed.

Under clinical evaluation:

Clinical trials investigating new agents and new combinations of agents are available and should be considered. Information about ongoing clinical trials is available from the NCI www.cancernet.nci.nih.gov/trialsrch.shtml.

References:

Ifosfamide with mesnauroprotection and etoposide: an effective regimen in the treatment of recurrent sarcomas and other tumours of children and young adults. Journal of Clinical Oncology 5(8): 1191-1198, 1987.

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Treatment Second Stage

Treatment will depend on a number of factors including the size, position and stage of the tumour. In general, chemotherapy is given to shrink the main tumour - this may be given before and/or after the tumour is removed by surgery.

If surgery is needed, it may be carried out at a specialist orthopaedics centre. Often surgery can remove the tumour without causing too much damage. If the tumour is in one of the main bones of the arm or leg however, it may be necessary to remove the whole limb (amputation) or part of the affected bone, which is then replaced by some form of false limb (prosthesis). If only part of the affected bone is removed, this is known as limb-sparing surgery.

Amputation of the limb is sometimes unavoidable if the cancer has affected the surrounding blood vessels and nerves. After amputation, a false limb will be fitted and this will be regularly adjusted as the child grows. The function of a false limb can be very good. It should be possible for the child to join in with normal activities and even sport.

See the section on 'Limb-Sparing Surgery' for further details.
Follow the link at the top of the page.

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PNET: Peripheral Neuroectodermal Tumour

Tumour grades, what you need to know about cancer:
Tumours Treatment

Soft tissue Ewing sarcoma - peripheral primitive neuroectodermal tumour with atypical clear cell pattern shows a new type of EWS-FEV fusion transcript.

Protocol Entry Criteria - Disease Characteristics:

Histological, verified (at original diagnosis) solid tumour that is relapsed or refractory. The following histologists are eligible:

  • Sarcomas
  • Rhabdomyosarcoma
  • Ewing's sarcoma
  • Peripheral neuroectodermal tumour (PNET), Primitive neuroectodermal tumour (PNET)
  • Osteosarcoma
  • Other soft tissue sarcomas
  • Brain tumours
  • Ependymoma
  • High grade Astrocytoma
  • Brain stem Glioma (histological verification not required)
  • Neuroblastoma

Measurable disease that can be followed clinically or radio logically required.

The following are not considered measurable:

Bone lesions measured by bone scan or bone marrow involvement, central nervous system disease documented by cerebrospinal fluid cytology and pleural effusion.

Prior/Concurrent Therapy

Biologic therapy:

  • Prior bone marrow transplantation allowed
  • Must have stable engraftment without need for significant blood product
  • Support or cytokine therapy
  • No concurrent immunomodulating agents

Chemotherapy:

  • No prior paclitaxel or docetaxel
  • At least 2 weeks since chemotherapy (4 weeks since nitrosoureas)
  • No other concurrent cancer chemotherapy

Endocrine therapy:

  • Concurrent corticosteroids allowed for intracranial pressure in brain tumour
  • patients provided patient has been stable for at least 4 weeks
  • Corticosteroids allowed as pre-treatment for docetaxel

Radiotherapy:

  • At least 2 months since extensive radiotherapy, defined as Craniospinal
  • Volume greater than 50% of abdominopelvic cavity
  • Volume greater than one third of lung volume
  • No concurrent radiotherapy

Surgery is not specified.

Other: No more than 2 prior therapies and fully recovered.

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Limb Sparing Surgery:

Here are several ways in which limb-sparing surgery may be done:

Replacing the bone with a prosthesis (a specially designed artificial part) Replacing with a bone graft. After this type of surgery, the child is usually able to use the limb almost normally. However, it is best not to take part in any contact sports, as if the bone graft or prosthesis is damaged another major operation may be needed to repair or replace it. If the child is growing, a limb prosthesis will need to be lengthened as the bone grows. This will mean further short stays in hospital.

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Extended Information on (PNET)

Primitive Neuroectodermal Tumour (PNET)

The histological appearance of the PNET differs somewhat from ETB and EOE. These tumours are typically composed of round to ovoid hyper chromatic cells with minimal cytoplasm. The tumour cells are typically arranged in nests and trabeculae with variable rosette formation. The rosettes may have a central lumen, but are often ill-defined, composed of tumour cells arranged around an empty space. The classic lobular growth pattern is best appreciated at low power, and differs from the typical diffuse growth seen in classical Ewing's.

Occasionally, groups of cytological uniform, round cells with dispersed chromatin resembling those in classical Ewing's are seen interspersed in an otherwise typical PNET. This overlap of features lends confidence to the concept that these tumours are indeed the same tumour with a spectrum of differentiation. PNETs also show variable staining with some neural markers including neuron-specific enolase, Leu-7, synaptophysin, microfilament and S100.3. The demonstration of neurosecretory granules by electron microscopy enhances the pathologist's ability to make the diagnosis of PNET.4er

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Treatment & Drug Use

Treatment used in clinical trials (Ewing Sarcoma) and other forms of Childhood Cancer.

We hope the information in this section will answer some of your questions regarding the Treatments and Clinical Trials being used and carried out around the world. Our thanks to Barry Sugarman B.S.ENGR who has made us aware of the vast library of information on drugs and treatments used in Ewing Sarcoma. This information also covers the many different forms of cancers and their treatments. We shall link you with the research centres, should you wish to contact them (parent information). We would however like to repeat the warning Barry has placed in the public domain, which is as follows:

Warning: The information herein does not constitute a medical opinion.

These drugs should be discussed with your primary physician and an oncologist prior to use. These drugs are only available upon prescription of a licensed medical doctor. These drugs are for those patients who have already completed the standard drug regimen for Ewing's Sarcoma and have had a reoccurrence of the disease, or those who are participating in a clinical study, or those who are otherwise requested to do so by their treating physician or oncologist. The standard drug regimen products are listed at www.cureourchildren.org/. The standard drug regimen has been tested in large, long term clinical trials, and is the most effective proven regimen known at this time.

Treatment Rating System:

Category 1:

Front Line, First Choice Treatments for Ewing's Relapse. Substantial Scientific Evidence of Safety and Efficacy. All drugs are FDA approved. These regimens known to be in used as a first choice treatment plan in relapse by reputable oncologists.

Category 2:

Substantial Scientific Evidence of Safety of the individual drugs in the regimen or of the individual or combination of drugs in adults. These regimens are also known to be in use by reputable oncologists when other first choice therapies have failed. These are primarily New Regimens and Combinations of Traditional Chemotherapy with New Agents for Treatment of Ewing's Relapse.

Category 3:

Some evidence of safety and efficacy, but still in clinical trials, and known to be in use by reputable oncologists. Not currently approved by the FDA yet.

Category 4:

No proven evidence of safety or efficacy in humans currently, in Phase I clinical trials.

Category 5:

Developmental and highly speculative or theoretical.

General Reference Links:

American Society of Clinical Oncologists (ASCO) Abstracts 2001,
Paediatric Solid Tumours,
Phone: 703-299-0150

American Society of Clinical Oncologists (ASCO) Abstracts 2001,
Paediatric Leukaemia/Lymphoma,
Phone: 703-299-0150

European Society of Medical Oncology Abstracts,
Lugarno, Switzerland,
Phone: 011-14-91-950-07-85, Fax -87

Bio space Drugs in Clinical Development Search Engine,
Phone: 888-BIOSPACE or 415-355-6500

Category 1:

Ifosfamide, Etoposide, Carboplatin (ICE) or Ifosfamide or Docetaxel or Irinotecan with Vincristine or Topotecan with Cyclophosphamide. These are the front line first choice treatments for Ewing's relapse chosen by oncologists most experienced with Ewing's Sarcoma. Once the tumour is under control again, high dose chemotherapy with stem cell rescue may be helpful and should be discussed with the oncologist.

For more in information regarding 'Stem Cell Transplant' follow the link: www.lymphomainfo.net/therapy/transplants/bmt.html/

Doctors utilizing these regimens include:

Dr. James Miser,
City of Hope,
National Medical Centre,
Phone: 800-535-7119
Email: jmiser@coh.org

Professor Stuart Siegel,
Children's Hospital of Los Angeles,
Phone: 323-669-2205
Email: ssiegel@chla.usc.edu

Dr. Doug Hawkins,
The University of Washington,
Seattle,
Phone: 206-526-2106 or (206) 526-2131
Email: dhawki@chmc.org

Research

  • ICE Supporting Research: here are published research abstracts from ASCO and Medline.
  • Topotecan with Cyclophosphamide Supporting Research: published abstracts from ASCO and Medline.

Please note that the link to ASCO (American Society of Clinical Oncology) www.asco.org/ac/1,1003,_12-002138,00.asp does not appear to work. We suggest that you search their site as they may have moved or re-named the web page.

Drugs Manufactured by:

Cytoxan ® (Generic Name: Cyclophosphamide)
www.astamedica-oncology.com/english/products/index.html/
Manufactured by Astra Zeneca Pharmaceuticals www.astrazeneca-us.com/
Phone: 800-456-3669 or 302- 886-3000

Hycamtin ® (generic name Topotecan) www.hycamtin.com/
Manufactured by Glaxo SmithKline http://us.gsk.com/
Phone: 1-800-456-6670 or 1-412-928-1000

Phase 2 study under the Children's Oncology Group, NCI-supported clinical trials cooperative group devoted exclusively to childhood and adolescent cancer research, follow this link to the protocol http://cancer.ucsd.edu/outreach/clintrials/990221.htm/. Title: Cyclophosphamide Plus Topotecan In Children with Recurrent or Refractory Solid Tumors (POG 9464) (HSC 970117)(HSC 990221).

Principal Investigator: Robert L. Saylors,
Pediatric Oncology Group,
Phone: 501-320-1494

Taxotere ® (Generic Name: Docetaxel)
Manufactured by Aventis Pharmaceuticals www.aventispharma-us.com/
Phone: 800-633-1610
Taxotere Website: www.taxotere.com/

Status: FDA approved in the United States for locally advanced or Metastatic breast cancer. In use as a single agent for relapsed Ewing's Sarcoma by Dr. Doug Hawkins at the University of Washington, Seattle, Phone: 206-526-2106 or (206) 526-2131, email: dhawki@chmc.org and by Dr. Andrew Pendleton at Cabell Huntington Hospital in Huntington, West Virginia, Phone 304-691-1300.

Camptosar ® - http://pharmaciaoncology.com/drugroot/camptosar/pi/public/camptosar.pdf/
(Generic Name: Irinotecan [CPT-11]) - http://pharmaciaoncology.com/drugroot/camptosar/pi/public/camptosar.pdf/
Manufactured by Pharmacia Upjohn Co. http://pharmaciaoncology.com/
Phone: 800-253-8600 x8244

Thalmomid ® (Thalidomide)
Manufactured by Celgene Corporation www.celgene.com/
Phone: 732-271-1001
FAX: 732-271-4184
Email: webmaster@celgene.com

Clinical Study

There is a recently completed Phase 1 clinical study in children with relapsed solid tumours with good results. The study was conducted at St Jude Children's Research Hospital www.stjude.org in Memphis, Tennessee and The University of Tennessee also in Memphis. The Study synopsis appears in the Proceedings of the American Society of Clinical Oncology, Volume 17, 1998, No. 721.

The contact at St. Jude Children's Research Hospital is:

Wayne L. Furman, M.D
Phone: 901-495-2403/3577
Email: wayne.furman@stjude.org

Professor Stuart Siegel, M.D. at the Children's Hospital of Los Angeles www.childrenshospitalla.org/ also reports good preliminary results on patient with relapsed Ewing's Sarcoma, his contact details are:

Professor Stuart Siegel, M.D.
Phone: 323-669-2205
Email: ssiegel@chla.usc.edu.

Status: FDA approved drug but not for Ewing's Sarcoma.


Information Reference:

The information within this page was collated from several different sources by George Quinn.

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