Treatment Options

First Line Chemotherapy
1. P6 Protocol established by Memorial Sloan-Kettering Cancer Center.


 * The P6 protocol consists of seven courses of Chemotherapy.


 * Courses 1, 2, 3, and 6 include 6-hour infusions of cyclophosphamide on days 1 and 2 for a total of 4,200 mg/m2 per course (140 mg/kg per course for patients < 10 years old), plus 72-hour infusions of doxorubicin 75 mg/m2 and vincristine 2.0 mg/m2 beginning on day 1 (HD-CAV).


 * Courses 4, 5, and 7 consist of 1-hour infusions of ifosfamide 1.8 g/m2/d and etoposide (VP-16) 100 mg/m2/d, for 5 days.


 * Granulocyte colony-stimulating factor (G-CSF) and mesna are used.


 * Courses start after neutrophil counts reach 500/microL and platelet counts reach 100,000/uL.


 * Surgical resection follows course 3 and radiotherapy follows completion of all chemotherapy. Pubmed Abstract

2. Variations of the P6 protocol


 * A. Kushner's Variation P6 Protocol
 * Courses 1, 2, 3, HD-VAC = cyclophosphamide, doxorubicin , vincristine
 * If significant shrinkage. Surgery.
 * Courses 4 & 5 = Topotecan and Cytoxan
 * More surgery if needed
 * Courses 6 - HD-VAC = cyclophosphamide, doxorubicin , vincristine

B. Ewing's Sarcoma (Ewings Sarcoma has been indicated as being the bone version of DSRCT. DSRCT shares similar genetic features with Ewings and Wilms Tumor). Uses the same 5 chemicals as P6 in less amount but more frequently over 8-9 or more cycles.

Patient Groups by Chemotherapy

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Secondary Chemotherapy
Chemotherapy used after first line fails or is stopped. Some chemo used alone, or may be tried in combination with other chemo drugs. May also involve a Clinical Trial.

1. Irinotecan and Temodar

2. ET-743

3. EPOCH : Etoposide, Doxorubicin, Vinristine, Cyclophosphamide, Prednisolone, G-CSF

Surgery
1. Debulking surgery
 * A major operation where the surgeon removes as many tumours as he can without disturbing/harming surrounding organs and tissue. In DSRCT the objective should be 90-100% surgical removal, if possible. This often requires 2 or more operations.

2. Cytoreductive surgery and Intraperitoneal Hyperthermic chemoperfusion
 * The total removal of all visible tumor, removal of all diseased organs (hysterectomy, colostomy, splenectomy, etc) followed by heating chemo and pumping it into the abdomen for a 90 minute wash.

PubMed

Webcast of IPHC Procedure : Cytoreductive Surgery with Intra-Peritoneal Hyperthermic Chemotherapy - Graphic surgical video provides look into the removal of all abdominal tumor and the 90 minutes abdominal bath for one patient. Caution - this is a real operation, so be prepared! Not a DSRCT page, but procedure is the same.

Roswell Park video abou Hyperthermic chemoperfusion

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Radiation
1. Radiation

2. Radioablation

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Stem Cell
There are two main types of blood stem cell transplants:


 * 1. Bone Marrow Transplant - bone marrow is the soft, sponge-like material found inside bones. The bone marrow contains immature cells that make up the blood-forming (hematopoietic) stem cells (this is different than embryonic stem cells which can develop into any cell within the body).


 * 2. Peripheral Blood Stem Cell Transplant -blood forming or hematopoietic stem cells can also be found in the blood

Both transplants, BMT or PBSCT restore stem cells that have been destroyed purposely by radiation or high dose chemotherapy. This is also called Stem Cell Rescue.

There are three types of stem cell rescues:


 * A. Autologous transplants where patients receive their own stem cells.


 * B. Syngeneic transplants where patients receive stem cells from their identical twin.


 * C. Allogeneic transplants where patients receive stem cells from their brother, sister, or parent.
 * It is also possible to use an unrelated matched donor.


 * Autologous stem cell rescue requires a patient to 'harvest' his own stem cells from either the bone marrow or blood. He is than given high dose chemotherapy in order to kill off any remaining cancer cells within the body and hopefully leave a disease free environment for the new stem cells to grow back in. The 'harvested' stem cells are than 'given back' to the patient via infusion (similar to a blood transfusion). Because the patients immune system is compromised they are required to remain in isolation for a month while waiting for the new 'rescued' stem cells to 'graft'.

Requires a donor (sibling, parent, unknown matched donor) to harvest stem cells from either bone marrow or blood. The donors stem cells are than given to the patient who has already undergone high dose chemo to provide a disease free environment. Because the patients immune system is compromised they are required to remain in isolation for a month while waiting for the new 'rescued' stem cells to 'graft'.
 * Myeloablative Allogeneic Transplants


 * Nonmyeloablative Allogeneic Transplants is the same principle as myeloablative but with low dose or standard chemotherapy for those patients unable to go through high dose. Benefits are still uncertain with this approach and considered experimental.


 * Graft vs Host disease - the process in which the patients body rejects the stem cell transplant. Medications are given and the patient watched closely for signs of the disease.

More details about stem cell transplants can be found at the National Cancer Institute's page about stem cells: Understanding Stem Cells

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Clinical Trials

 * 17-AAG Clinical Trial


 * AP23573 Clinical Trial - Rapamycin, mTOR inhibitor -FDA placed as orphan drug and put on fast track for bone and soft tissue sarcoma.

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Experimental Treatment
Lupron


 * Lupron Shots - Boost and/or control hormones, testosterone and/or estrogen. Often used for Prostrate cancer.

See DSRCT Research

Dendritic Vaccine
 * Dendritic cells are the ones which contain/stimulate the killer cells which kill what goes wrong in our body, including tumor cells. When the dendritic cells either don’t identify or don’t send the message identifying a “stranger” in the body the tumor cells are allowed to grow.


 * A few places around world, including New York, are testing dendritic vaccines. The original one was created in Germany but different trials are going on. The beauty of this process is that immunotherapy is not harmful to the body and can be safely tried.


 * The objective of the vaccine is twofold: (1) to increase the number of dendritic cells in the body; and (2) to make the dendritic cells identify the tumor cells they failed to identify in the beginning.


 * In order to use the dendritic vaccine the patient must be in remission, which means: (1) being free of visible tumors; and (2) not being treated with chemotherapy.


 * The dendritic vaccine therapy my son is using consists of the following:


 * On day 1 either I or my son donate blood (harvested for 1 ½ hour in order to select by centrifugation the parts of the body which contain the dendritic cells). Actually I donated my blood for the first three times and after that my son has been donating to himself.

On the same day the pathologist starts the preparation of the vaccine, mixing the blood donated plus the frozen sample of the tumor extracted during surgery plus a few other compounds. It takes one week to conclude the process. On day 5 my son gets a very mild dose of chemotherapy (Cyclophosphamide), 15% of the dose he got during his treatment. On day 8 my son gets the vaccine plus a very mild dose of Interleukin. On days 9, 10, 11 and 12 he gets other shots of mild Interleukin He did not have any side effects and the process was repeated monthly for the first six months and is being now extended to every 6 weeks then every 9 weeks.
 * The pathologist is able to have an idea, on the microscope, of the number of dendritic cells being developed and its “quality”. After the fifth shot he concluded that the quality of the dendritic cells is better than that of human beings who never had cancer. He is certain that my son’s immune system is reacting very positively to the vaccine. -

Marcio Rio de Janeiro, Brazil

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Alternative Medicine
See Alternative Medicine

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Maintenance Drugs
1. Cox-2 Inhibitors
 * Vioxx
 * Celebrex

2. Etoposide (VP-16)

3. Unknown
 * Low dose Thalidomide
 * Vinorelbine and low-dose Cyclophosphamide

'Disclaimer: This site is for informational and idea exchange among patients only. We are not doctors and do not offer medical advice. Please check with your own physician if you have any unusual symptoms or questions concerning treatment.' [BACK]