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Treatment Options

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DSRCT Treatment Protocol

There is no established protocol, but most patients based on the stage of their disease, age, and overall health are starting with the following treatments:

Stage I and Stage II DSRCT:

  • Complete surgical resection recommended
  • Chemotherapy - P6 recommened

Stage III and Stage IV DSRCT:

  • First Line Chemotherapy - 7 to 9 months of Vincristine, Adriamycin, Cytoxan, and Etoposide, Ifosimide recommended
  • Debulking surgery at 3 to 4 months determined by response to First Line Chemotherapy recommended
  • Stem Cell Harvest recommended
  • Continue with First Line Chemotherapy recommended
  • Second surgery to remove all visible disease.
    • If malignant abdmoninal seeding suspected: Intra-peritoneal hyperthermic chemoperfusion (option)
  • Continue with First Line Chemotherapy recommended
  • Stem Cell Transplant (option)
  • Radiation (option)
  • Maintenance: VP-16; Vioxx; Celebrex; Rapamycin; low dose Irinotecan and Temodar (option)
  • Second Line Chemotherapy: Irinotecan and Temodar (option)
  • Second Line Chemotherapy: Topetecan and Cytoxan (option)
  • Relapse Options: Radiofrequency ablation; Gamma Knife; Clinical Trials; Other chemotherapy off trial options; Photon Therapy; Complementary and Integrative medicine; Bone Marrow Transplant; 2nd Stem Cell Transplant; experimental treatments
    • New therapies and treatments are desperately needed for this aggressive disease. If patients fail in the above approach please consider entering a Clinical Trial. Clinical Trials can establish which treatments are working and which treatments that do not work. A clinical trial will give the patient access to new drugs, new treatment options, and access to those in the medical field pursuing research into treatments and cures for sarcoma and eventually a cure for DSRCT.

First Line Chemotherapy

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

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.

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Second Line Chemotherapy

Chemotherapy used after first line fails or is stopped because patient has reached lifetime limits. Some chemo agents are used alone, or may be tried in combination with other chemo drugs. May also involve a Clinical Trial. Ask your physician.

Note: Despite medical reports that DSRCT does not metastasize to the brain or spinal column, there are reports from patients of this happening. Temodar(temozolomide) is a drug used for brain cancer and has been noted to cross the 'brain-blood' barrier. This is a drug to consider if metastasis has occured or if you need a second line chemotherapy.


1. Irinotecan and Temodar

A. Clinical Trial for Pediatric: Phase 1 - Temozolomide, Vincristine, Irinotecan
B. Phase II study of a protracted irinotecan schedule

2. ET-743 - Yondelis or the common sea squirt is behind this novel chemotherapeutic agent. At the moment it is undergoing Clincial Trials and there are a few DSRCT patients included.

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. The following chemicals are used separately or in combination depending upon the patient and disease: 5-fluorouracil; mitomycin C; cisplatin; doxorubicin

Management of Peritoneal Surface Malignancy using Intraperitoneal Chemotherapy and Cytoreductive Surgery

PubMed Journal

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 Therapy Principles - Detailed information provided by the American Cancer Society about the benefits and risks associated with using radiation to fight cancer. In the cases of DSRCT radiation therapy is often difficult because of widespred abdominal disease. When a cancer is localized than the radiation can be pinpointed in that one area, but when the cancer is diffuse there becomes a risk to healthy organs. Please discuss the situation thoroughly with your physician.

2. Total Body Irradiation - is radiation used to deliver a wide field of radiation to the whole body. This type of treatment is often used prior to and to prepare the body for a bone marrow transplant.

3. Intensity Modulated Radiotherapy - Involves the changing of the size, shape, and intensity of radiation beam to conform to the size, shape, and location of the patient's tumor. (example: Tomo Therapy )

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Radio Frequency Ablation

RFA uses radiofrequency energy to destroy targeted neoplasms in the liver, breast, and other localized areas. Under guidance (CT) a probe is inserted into the lesion or mass. By exerting radio-energy with frequency around 460 kHz, the tumor mass (via the probe) is heated and destroyed. This is considered a minimally invasive therapy, but may not be suitable for all patients. Please check with your physician about your own situation. RFA for Liver Tumors

RFA has been used successfully on several DSRCT patients with liver metastises.

RFA

Photon Therapy

This has not been tried specifically on patients with DSRCT but has been used on pediatric patients with sarcoma.

Spot-scanning proton therapy for malignant soft tissue tumors in childhood: First experiences at the Paul Scherrer Institute.

  • Timmermann B,
  • Schuck A,
  • Niggli F,
  • Weiss M,
  • Lomax AJ,
  • Pedroni E,
  • Coray A,
  • Jermann M,
  • Rutz HP,
  • Goitein G.
Division of Radiation Medicine, Proton Therapy, Paul Scherrer Institut, Villigen, Switzerland. beate.timmermann@psi.ch
PURPOSE: Radiotherapy plays a major role in the treatment strategy of childhood sarcomas. Consequences of treatment are likely to affect the survivor's quality of life significantly. We investigated the feasibility of spot-scanning proton therapy (PT) for soft tissue tumors in childhood. METHODS AND MATERIALS: Sixteen children with soft tissue sarcomas were included. Median age at PT was 3.3 years. In 10 children the tumor histology was embryonal rhabdomyosarcoma. All tumors were located in the head or neck, parameningeal, or paraspinal, or pelvic region. In the majority of children, the tumor was initially unresectable (Intergroup Rhabdomyosarcoma Study [IRS] Group III in 75%). In 50% of children the tumors exceeded 5 cm. Fourteen children had chemotherapy before and during PT. Median total dose of radiotherapy was 50 cobalt Gray equivalent (CGE). All 16 children were treated with spot-scanning proton therapy at the Paul Scherrer Institute, and in 3 children the PT was intensity-modulated (IMPT). RESULTS: After median follow-up of 1.5 years, local control was achieved in 12 children. Four children failed locally, 1 at the border of the radiation field and 3 within the field. All 4 children died of tumor recurrence. All 4 showed unfavorable characteristic either of site or histopathology of the tumor. Acute toxicity was low, with Grade 3 or 4 side effects according to Radiation Therapy Oncology Group/European Organization for Research and Treatment of Cancer (RTOG/EORTC) criteria occurring in the bone marrow only. CONCLUSIONS: Proton therapy was feasible and well tolerated. Early local control rates are comparable to those being achieved after conventional radiotherapy. For investigations on late effect, longer follow-up is needed.
PMID: 17084557 [PubMed - indexed for MEDLINE]

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'.
    • Myeloablative Allogeneic Transplants

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'.

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

1. Clinical Trials for DSRCT
2.17-AAG Clinical Trials
3. #AP23573 Clinical Trial Rapamycin - mTOR inhibitor -FDA placed as orphan drug and put on fast track for bone and soft tissue sarcoma.
Rapamycin induces apoptosis of JN-DSRCT-1 cells

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Tools

DSRCT and Diagnostics

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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; *(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 consists of the following:
On day 1 donate blood (harvested for 1 ½ hour in order to select by centrifugation the parts of the body which contain the dendritic cells).
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 a very mild dose of chemotherapy (Cyclophosphamide) is given.
On day 8 patient gets the vaccine plus a very mild dose of Interleukin.
On days 9, 10, 11 and 12 Patient gets other shots of mild Interleukin
The process is than repeated monthly for the first six months. Than is 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” and whether the immune system is responding.

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Alternative Medicine

See Alternative Medicine

Burzynski Clinic, Houston, Texas

Antineoplasm & Herceptin treatment

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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

4. Irinotecan and Temodar - Low dose

DSRCT References

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.

Contact: dsrct.wiki@gmail.com

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