Neoplasms

All Conditions

Symptoms and General Pathology

Behaviors and Mental Disorders

Skin and Connective Tissue Diseases

Rare Diseases

Sarcoma

Recurrence

Fibromatosis, Aggressive

Rhabdomyosarcoma

Desmoplastic Small Round Cell Tumor

Chordoma

Rhabdomyosarcoma, Embryonal

Aggression

Fibroma

Disease Attributes

Neoplasms, Connective and Soft Tissue

Neoplasms by Histologic Type

Myosarcoma

Neoplasms, Fibrous Tissue

Neoplasms, Connective Tissue

Neoplasms, Germ Cell and Embryonal

Soft Tissue Sarcoma

Desmoid Tumor

Surgical tissue, bone marrow, and blood specimens are collected at diagnosis (initial or relapse) and, if applicable, at the development of a second primary tumor. Specimens are used for research purposes. A certificate of confidentiality protecting the identity of research participants in this project has been issued by the National Cancer Institute.

Cytology Specimen Collection Procedure

Laboratory Biomarker Analysis

Stephen Skapek

The purpose of this study is to collect and store tumor tissue, blood, and bone marrow samples from patients with soft tissue sarcoma that will be tested in the laboratory. Collecting and storing samples of tumor tissue, blood, and bone marrow from patients to test in the laboratory may help the study of cancer.

PRIMARY OBJECTIVES:

I. Collect human tumor tissue and other biological specimens (blood, serum, and bone marrow) from patients with rhabdomyosarcoma or non-rhabdomyosarcoma soft tissue sarcoma diagnosed and/or treated at a Children's Oncology Group (COG) member institution.

II. Provide a repository for storage of tissue and other biological specimens collected by COG investigators from these patients.

III. Make these specimens available for approved projects by laboratory-based investigators.

IV. Collect clinical data on these patients who are not being treated on a COG therapeutic study.

V. Define and compare the clinical features of patient subgroups with alveolar rhabdomyosarcoma whose tumors carry the t(2;13), t(1;13) or neither translocation.

VI. Investigate the relationship between evidence of submicroscopic disease and response rate (CR/PR), failure-free survival, and survival of patients with alveolar rhabdomyosarcoma, as determined by positive or negative reverse transcriptase-polymerase chain reaction (RT-PCR) assay for the t(2:13) and t(1:13) on peripheral blood and bone marrow specimens obtained at diagnosis.

VII. Compare the clinical, cytogenetic, and molecular biologic features of patient subgroups with anaplastic rhabdomyosarcoma and other subtypes of rhabdomyosarcoma.

OUTLINE:

Surgical tissue, bone marrow, and blood specimens are collected at diagnosis (initial or relapse) and, if applicable, at the development of a second primary tumor. Specimens are used for research purposes. A certificate of confidentiality protecting the identity of research participants in this project has been issued by the National Cancer Institute.

Patients who are not enrolled on a Children's Oncology Group treatment trial are followed every 6 months for at least 10 years or until disease progression or development of a second malignancy.

Collecting and Storing Tissue, Blood, and Bone Marrow Samples From Patients With Rhabdomyosarcoma or Other Soft Tissue Sarcoma

A COG Soft Tissue Sarcoma Diagnosis, Biology and Banking Protocol

National Cancer Institute (NCI)

Children's Oncology Group

Antineoplastic Agents

Antirheumatic Agents

All Drugs and Chemicals

Anti-Infective Agents

Cyclophosphamide

Doxorubicin

Liposomal doxorubicin

Ifosfamide

Lenograstim

Thalidomide

Dacarbazine

Isophosphamide mustard

Imidazole

Mechlorethamine

Anti-Bacterial Agents

Antibiotics, Antitubercular

Enzyme Inhibitors

Adjuvants, Immunologic

Immunologic Factors

Antineoplastic Agents, Alkylating

Alkylating Agents

Immunosuppressive Agents

Angiogenesis Inhibitors

Growth Inhibitors

Patients receive doxorubicin, ifosfamide, and dacarbazine IV continuously on days 1-3, 22-24, and 43-45. Patients receive G-CSF subcutaneously beginning on days 4, 25, and 46 and continuing until blood counts recover. Patients undergo radiotherapy once daily on days 7-11, 14-18, 21, 28-32, 35-39, and 42. Patients receive oral thalidomide once daily on days 7-21 and 26-42. Patients undergo surgical resection between days 84 and 98. Beginning 2 weeks after surgery, patients receive oral thalidomide once daily for 12 months in the absence of unacceptable toxicity.

Patients receive oral thalidomide once daily beginning on day 1 and continuing until 1 week before surgery. Patients undergo radiotherapy once daily, 5 days a week, on weeks 1-5. Patients undergo surgical resection between days 77 and 91. Beginning 2 weeks after surgery, patients receive oral thalidomide once daily for 6 months in the absence of unacceptable toxicity.

Doxorubicin Hydrochloride

Filgrastim

Radiation Therapy

Therapeutic Conventional Surgery

Burton Eisenberg

Thalidomide may stop the growth of soft tissue sarcoma by stopping blood flow to the tumor. Radiation therapy uses high-energy x-rays to damage tumor cells. Drugs used in chemotherapy, such as doxorubicin, ifosfamide, and dacarbazine, work in different ways to stop tumor cells from dividing so they stop growing or die. Giving thalidomide together with radiation therapy and/or chemotherapy before surgery may shrink the tumor so that it can be removed. This phase II trial is studying how well giving preoperative (before surgery) thalidomide together with radiation therapy works in treating patients with low-grade primary soft tissue sarcoma, and how well giving thalidomide together with radiation therapy, doxorubicin, ifosfamide, and dacarbazine works in treating patients with high-grade or intermediate-grade primary soft tissue sarcoma of the arm, leg, chest wall, or abdominal wall.

OBJECTIVES:

I. Determine the treatment delivery and toxicity of the combination of thalidomide and radiotherapy in patients with low-grade primary soft tissue sarcoma of the extremity or body wall.

II. Determine the treatment delivery and toxicity of the combination of thalidomide and doxorubicin, ifosfamide, dacarbazine, and radiotherapy in patients with high- or intermediate-grade primary soft tissue sarcoma of the extremity or body wall and compare these results with those of patients treated on RTOG-9514.

III. Determine the feasibility of using specific tissue and circulating biomarkers of antiangiogenic response in patients treated with these regimens, in a multi-institutional setting.

IV. Determine the quantitative changes and patient variabilities of these biomarkers before, during, and after therapy with these regimens.

V. Determine the baseline data sets of biomarkers, particularly circulating endothelial cells, in patients treated with these regimens.

VI. Determine the tolerance to long-term post-operative thalidomide in these patients.

VII. Determine the clinical response to pre-operative therapy in these patients.

VIII. Correlate local control and disease-free survival with surrogate biological endpoints in patients treated with these regimens.

OUTLINE: This is a pilot, cohort study. Patients with high- or intermediate-grade tumors \>= 8 cm in diameter are assigned to cohort A and patients with low-grade tumors \> 5 cm in diameter are assigned to cohort B.

Cohort A: Patients receive doxorubicin, ifosfamide, and dacarbazine IV continuously on days 1-3, 22-24, and 43-45. Patients receive filgrastim (G-CSF) subcutaneously beginning on days 4, 25, and 46 and continuing until blood counts recover. Patients undergo radiotherapy once daily on days 7-11, 14-18, 21, 28-32, 35-39, and 42. Patients receive oral thalidomide once daily on days 7-21 and 26-42. Patients undergo surgical resection between days 84 and 98. Beginning 2 weeks after surgery, patients receive oral thalidomide once daily for 12 months in the absence of unacceptable toxicity.

Cohort B: Patients receive oral thalidomide once daily beginning on day 1 and continuing until 1 week before surgery. Patients undergo radiotherapy once daily, 5 days a week, on weeks 1-5. Patients undergo surgical resection between days 77 and 91. Beginning 2 weeks after surgery, patients receive oral thalidomide once daily for 6 months in the absence of unacceptable toxicity.

Patients are followed every 3 months for 2 years and then every 6 months for 4 years.

PROJECTED ACCRUAL: A total of 44 patients (22 per cohort) will be accrued for this study within 17 months.

Preoperative Thalidomide With Radiation Therapy For Patients With Low-Grade Primary Soft Tissue Sarcoma or Thalidomide With Radiation Therapy and Chemotherapy For Patients With High-Grade or Intermediate-Grade Primary Soft Tissue Sarcoma of the Arm, Leg, or Body Wall

A Pilot Phase II Study of Pre-Operative Radiation Therapy and Thalidomide (IND 48832; NSC 66847) for Low Grade Primary Soft Tissue Sarcoma or Pre-Operative MAID/Thalidomide/Radiation Therapy for High/Intermediate Grade Primary Soft Tissue Sarcoma of the Extremity or Body Wall

Was to be estimated using a binomial distribution and accompanied by the associated 95% confidence interval. Due to early study closure, this endpoint could not be fully evaluated per the protocol plan.

Will be estimated using a binomial distribution and accompanied by the associated 95% confidence interval.

Radiation Therapy Oncology Group

Per the protocol, toxicity data was collected via CTCAE3.0 then mapped to CTCAE 4.0 for reporting on this website. Subjects experiencing more than one of a given SAE are counted only once for that SAE. Subjects experiencing more than one of a given "Other (Not Including Serious)" AE are counted only once for that "Other (Not Including Serious)" AE.

Patients receive doxorubicin, ifosfamide, and dacarbazine IV continuously on days 1-3, 22-24, and 43-45. Patients receive G-CSF subcutaneously beginning on days 4, 25, and 46 and continuing until blood counts recover. Patients undergo radiotherapy once daily on days 7-11, 14-18, 21, 28-32, 35-39, and 42. Patients receive oral thalidomide once daily on days 7-21 and 28-42. Patients undergo surgical resection between days 84 and 98. Beginning 2 weeks after surgery, patients receive oral thalidomide once daily for 12 months in the absence of unacceptable toxicity.

Cohort A (Chemotherapy, Radiation, Thalidomide, Surgery)

Cohort B (Thalidomide, Radiation, Surgery)

Total

Age, Continuous

Sex: Female, Male

All eligible patients who started treatment.

This study closed early due to unacceptably high rate of thromboembolic events in Cohort A and due to low accrual in Cohort B. For this reason efficacy endpoints other than response to pre-operative therapy were not reported.

Wendy Seiferheld

Not Compliant

Compliant

Eligible patients who started study treatment.

Treatment Delivery With Compliance Defined as Receiving at Least 95% of the Pre-operative Protocol Dose of RT, All 3 Cycles of MAID (if Applicable), and Receive Thalidomide on 75% of the Days During Radiation

This analysis was not carried out due to the small number of patients accrued to the study. See section "Limitations and Caveats".

Wound Complication (Grades 2, 3, 4, and 5) as Measured by CTCAE v3.0

Response to Pre-operative Therapy Assessed Using RECIST Criteria

Overall Study

Rhabdomyosarcoma, Alveolar

Rhabdomyosarcoma Embryonal

Rhabdomyosarcoma Alveolar

Sarcoma Botryoides

Analgesics

Central Nervous System Depressants

Irinotecan

Morphine

Vincristine

Camptothecin

Dactinomycin

Cactinomycin

Topoisomerase I Inhibitors

Tubulin Modulators

Antimitotic Agents

Patients receive VAC chemotherapy comprising vincristine IV over 1 minute on day 1 of weeks 1-13, 16, 19-25, 28, 31-37, and 40; dactinomycin IV over 1-5 minutes on day 1 of weeks 1, 4, 13, 16, 19, 22, 25, 28, 31, 34, 37,and 40; and cyclophosphamide IV over 1 hour on day 1 of weeks 1, 4, 7, 10, 13, 16, 19, 22, 25, 28, 31, 34, 37, and 40. Patients may also undergo radiotherapy 5 days a week for 4-6 weeks beginning in week 4.

Patients receive VAC chemotherapy alternating with VI chemotherapy comprising vincristine IV over 1 minute on day 1 of weeks 1-13, 16, 17, 19, 20, 22-26, 28, 31-34, 37, 38, and 40; dactinomycin IV over 1-5 minutes on day 1 of weeks 1, 13, 22, 28, 34, and 40; cyclophosphamide IV over 1 hour on day 1 of weeks 1,10, 13, 22, 28, 34, and 40; and irinotecan hydrochloride IV over 1 hour on days 1-5 of weeks 4, 7, 16, 19, 25, 31, and 37. Patients may also undergo radiotherapy 5 days a week for 4-6 weeks beginning in week 4.

Irinotecan Hydrochloride

Questionnaire Administration

Vincristine Sulfate

Douglas S Hawkins

This randomized phase III trial is studying two different combination chemotherapy regimens to compare how well they work when given together with radiation therapy in treating patients with newly diagnosed rhabdomyosarcoma. Drugs used in chemotherapy, such as vincristine sulfate, dactinomycin, cyclophosphamide, and irinotecan hydrochloride, work in different ways to stop the growth of tumor cells, either by killing the cells or by stopping them from dividing. Radiation therapy uses high-energy x-rays to kill tumor cells. Giving combination chemotherapy together with radiation therapy may kill more tumor cells. It is not yet known which combination chemotherapy regimen is more effective when given together with radiation therapy in treating patients with rhabdomyosarcoma.

PRIMARY OBJECTIVES:

I. To compare the early response rates, failure-free survival (FFS), and survival of patients with intermediate-risk rhabdomyosarcoma (RMS) treated with surgery, radiotherapy, and vincristine (vincristine sulfate), dactinomycin and cyclophosphamide (VAC) or VAC alternating with vincristine, irinotecan (irinotecan hydrochloride) (VI).

SECONDARY OBJECTIVES:

I. To compare FFS, local control, and survival of patients with intermediate-risk RMS treated with VAC and early (week 4) radiotherapy vs delayed (week 10) radiotherapy, using data from Intergroup Rhabdomyosarcoma Study (IRS)-IV for historic comparison.

II. To compare the acute and late effects of VAC to VAC alternating with VI, including the toxicity associated with concurrent VI and radiotherapy.

III. To compare the acute and late effects of VAC as delivered on this study to D9803 VAC.

IV. To correlate change in fludeoxyglucose F-18 positron emission tomography (FDG-PET) maximum standard uptake value (SUVmax) from week 1 to week 4 and 15 with FFS.

V. For VI treated patients, to correlate patient UDP glucuronosyltransferase 1 family, polypeptide A1 (UGT1A1) genotype with VI toxicity. VI. To correlate cytochrome P450, family 2, subfamily B, polypeptide 6 (CYP2B6), cytochrome P450, family 2, subfamily C, polypeptide 9 (CYP2C9), and glutathione S-transferase alpha 1 (GSTA1) genotypes with VAC toxicity.

VII. To prospectively evaluate and validate gene expression values with the intent to define the best diagnostic predictors and more powerful prognostic classifiers.

VIII. To assess the frequency of bladder dysfunction in patients with bladder, prostate, and pelvic sites of RMS 3-6 years after study enrollment.

OUTLINE: Patients are randomized to 1 of 2 treatment arms within 42 days of initial surgery or biopsy.

ARM I (VAC): Patients receive VAC chemotherapy comprising vincristine sulfate IV over 1 minute on day 1 of weeks 1-13, 16, 19-25, 28, 31-37, and 40; dactinomycin IV over 1-5 minutes on day 1 of weeks 1, 4, 13, 16, 19, 22, 25, 28, 31, 34, 37, and 40; and cyclophosphamide IV over 1 hour on day 1 of weeks 1, 4, 7, 10, 13, 16, 19, 22, 25, 28, 31, 34, 37, and 40.

ARM II (VAC/VI): Patients receive VAC chemotherapy alternating with VI chemotherapy comprising vincristine sulfate IV over 1 minute on day 1 of weeks 1-13,16, 17, 19, 20, 22-26, 28, 31-34, 37, 38, and 40; dactinomycin IV over 1-5 minutes on day 1 of weeks 1,13, 22, 28, 34, and 40; cyclophosphamide IV over 1 hour on day 1 of weeks 1,10, 13, 22, 28, 34, and 40; and irinotecan hydrochloride IV over 90 minutes on days 1-5 of weeks 4, 7, 16, 19, 25, 31, and 37.

In both arms, treatment continues in the absence of disease progression or unacceptable toxicity. Patients\* in both arms also undergo radiotherapy 5 days a week for 4-6 weeks beginning in week 4 (except patients with alveolar RMS rendered group I by amputation OR patients needing week 1 emergency radiotherapy for symptomatic spinal cord compression).

NOTE: \*Individualized local control plan that deviates from protocol-mandated radiotherapy allowed for patients =\< 24 months of age.

After completion of study treatment, patients are followed up every 2-4 months for 4 years and then annually for 5-10 years.

Combination Chemotherapy and Radiation Therapy in Treating Patients With Newly Diagnosed Rhabdomyosarcoma

Randomized Study of Vincristine, Dactinomycin and Cyclophosphamide (VAC) Versus VAC Alternating With Vincristine and Irinotecan (VI) for Patients With Intermediate-Risk Rhabdomyosarcoma (RMS)

Probability of no relapse, secondary malignancy, or death after 4 year in the study

Proportion of patients with complete or partial response. Complete Response (CR): Complete disappearance of the tumor confirmed at \> 4 weeks; Partial Response (PR): At least 64% decrease in volume compared to the baseline; Overall Response (OR) = CR + PR.

Probability of being alive after 4 years in the study.

Compare 4-year EFS using eligible participants only to the historical rate of 0.65 with IRSI-V. The 4-year EFS is probability of no relapse, secondary malignancy, or death after 4 years in the study. The Delayed (Week 10) Radiotherapy is from IRSI-V, and the number of participants of IRSI-V is unknown, but we have the rate of 0.65.

Compare 2-year local failure rate to the historical rate of 0.13 with IRSI-V. The Delayed (Week 10) Radiotherapy is from IRSI-V, and the number of participants of IRSI-V is unknown, but we have the rate of 0.13.

Compare 4-year OS using eligible participants only to the historical rate of 0.70 with IRSI-V. The 4-year OS is probability of being alive after 4 years in the study. The Delayed (Week 10) Radiotherapy is from IRSI-V, and the number of participants of IRSI-V is unknown, but we have the rate of 0.70.

Grade 3 or 4 nausea, diarrhea, dehydration, radiation dermatitis, mucositis due to radiation. Severe and undesirable adverse event is considered as grade 3; Life-threatening or disabling adverse event is grade 4. Grade 4 is worse than grade 3.

The toxicity rates will be estimated for each phase and course of treatment, and will be compared to the fixed rates under D9803 using one-sided lower confidence intervals for a single proportion without adjustment for multiple comparisons.

4-year EFS (probability of no relapse, secondary malignancy, or death after 4 years in the study).

4-year EFS (probability of no relapse, secondary malignancy, or death after 4 years in the study)

Severe and undesirable adverse event is considered as grade 3; Life-threatening or disabling adverse event is grade 4. Grade 4 is worse than grade 3.

Incidence of toxicity related to VAC treatment in patients with CYP2B6 genotypes.

Incidence of toxicity related to VAC treatment in patients with GSTA1 and CYP2C9 genotypes.

Number of patients with a summary score greater than 8.5

All eligible patients were considered in the evaluation of adverse events.

Patients receive VAC chemotherapy comprising vincristine IV over 1 minute on day 1 of weeks 1-13, 16, 19-25, 28, 31-37, and 40; dactinomycin IV over 1-5 minutes on day 1 of weeks 1, 4, 13, 16, 19, 22, 25, 28, 31, 34, 37,and 40; and cyclophosphamide IV over 1 hour on day 1 of weeks 1, 4, 7, 10, 13, 16, 19, 22, 25, 28, 31, 34, 37, and 40. Patients may also undergo radiotherapy 5 days a week for 4-6 weeks beginning in week 4.

Dactinomycin: Given IV

Cyclophosphamide: Given IV

Vincristine Sulfate: Given IV

Radiation Therapy: Undergo radiotherapy

Laboratory Biomarker Analysis: Correlative studies

Questionnaire Administration: Ancillary studies

Vincristine, Dactinomycin, Cyclophosphamide (VAC)

Patients receive VAC chemotherapy alternating with VI chemotherapy comprising vincristine IV over 1 minute on day 1 of weeks 1-13, 16, 17, 19, 20, 22-26, 28, 31-34, 37, 38, and 40; dactinomycin IV over 1-5 minutes on day 1 of weeks 1, 13, 22, 28, 34, and 40; cyclophosphamide IV over 1 hour on day 1 of weeks 1,10, 13, 22, 28, 34, and 40; and irinotecan hydrochloride IV over 1 hour on days 1-5 of weeks 4, 7, 16, 19, 25, 31, and 37. Patients may also undergo radiotherapy 5 days a week for 4-6 weeks beginning in week 4.

Irinotecan Hydrochloride: Given IV

Dactinomycin: Given IV

Cyclophosphamide: Given IV

Vincristine Sulfate: Given IV

Radiation Therapy: Undergo radiotherapy

Laboratory Biomarker Analysis: Correlative studies

Questionnaire Administration: Ancillary studies

VAC Alternating With VI

VAC Alternating With Vincristine, Irinotecan (VI)

Ethnicity (NIH/OMB)

Race (NIH/OMB)

Results Reporting Coordinator

Patients receive VAC chemotherapy comprising vincristine IV over 1 minute on day 1 of weeks 1-13, 16, 19-25, 28, 31-37, and 40; dactinomycin IV over 1-5 minutes on day 1 of weeks 1, 4, 13, 16, 19, 22, 25, 28, 31, 34, 37,and 40; and cyclophosphamide IV over 1 hour on day 1 of weeks 1, 4, 7, 10, 13, 16, 19, 22, 25, 28, 31, 34, 37, and 40. Patients may also undergo radiotherapy 5 days a week for 4-6 weeks beginning in week 4.

Dactinomycin: Given IV Cyclophosphamide: Given IV Vincristine Sulfate: Given IV Radiation Therapy: Undergo radiotherapy Laboratory Biomarker Analysis: Correlative studies Questionnaire Administration: Ancillary studies

Patients receive VAC chemotherapy alternating with VI chemotherapy comprising vincristine IV over 1 minute on day 1 of weeks 1-13,16, 17, 19, 20, 22-26, 28, 31-34, 37, 38, and 40; dactinomycin IV over 1-5 minutes on day 1 of weeks 1, 13, 22, 28, 34, and 40; cyclophosphamide IV over 1 hour on day 1 of weeks 1,10, 13, 22, 28, 34, and 40; and irinotecan hydrochloride IV over 1 hour on days 1-5 of weeks 4, 7, 16, 19, 25, 31, and 37. Patients may also undergo radiotherapy 5 days a week for 4-6 weeks beginning in week 4.

Event Free Survival (EFS)

Patients receive VAC chemotherapy comprising vincristine IV over 1 minute on day 1 of weeks 1-13, 16, 19-25, 28, 31-37, and 40;dactinomycin IV over 1-5 minutes on day 1 of weeks 1, 4, 13, 16, 19, 22, 25, 28, 31, 34, 37,and 40; and cyclophosphamide IV over 1 hour on day 1 of weeks 1, 4, 7, 10, 13, 16, 19, 22, 25, 28, 31, 34, 37, and 40. Patients may also undergo radiotherapy 5 days a week for 4-6 weeks beginning in week 4.

Response Rate (RR)

Overall Survival (OS)

17 ineligible participants were excluded.

Event Free Survival (EFS) Probability VAC and Early (Week 4) Radiotherapy Compared to Delayed (Week 10) Radiotherapy, Using IRSIV for Historic Comparison

Local Failure

Overall Survival (OS) Probability VAC and Early (Week 4) Radiotherapy Compared to Delayed (Week 10) Radiotherapy, Using IRSIV for Historic Comparison

Incidence of Toxicity

Compare incidence of Anemia to historical rate of 0.40 from D9803, which is Randomized Study of VCR, Actinomycin-D, and CPM (VAC) vs. VAC alternating with VCR, Topotecan, and CPM (VTC) for Patients with Intermediate Risk RMS. Comparing the incidence of anemia with VAC on ARST0531 to the historical rate of 0.40 with VAC on D9803. The 95% one-sided confidence interval for the incidence of anemia will be calculated and evaluated to see if the interval contains the null rate of 0.40.

Compare incidence of Nausea or Hepatopathy to historical rate of 0.05 from D9803, which is Randomized Study of VCR, Actinomycin-D, and CPM (VAC) vs. VAC alternating with VCR, Topotecan, and CPM (VTC) for Patients with Intermediate Risk RMS. The 95% one-sided confidence interval for the incidence of nausea or hepatopathy will be calculated and evaluated to see if the interval contains the null rate of 0.05.

Compare incidence of Febrile Neutropenia to historical rate of 0.50 from D9803, which is Randomized Study of VCR, Actinomycin-D, and CPM (VAC) vs. VAC alternating with VCR, Topotecan, and CPM (VTC) for Patients with Intermediate Risk RMS. The 95% one-sided confidence interval for the incidence of febrile neutropenia will be calculated and evaluated to see if the interval contains the null rate of 0.50.

Compare incidence of Platelet Count Decreased to historical rate of 0.70 from D9803, which is Randomized Study of VCR, Actinomycin-D, and CPM (VAC) vs. VAC alternating with VCR, Topotecan, and CPM (VTC) for Patients with Intermediate Risk RMS. The 95% one-sided confidence interval for the incidence of platelet count decreased will be calculated and evaluated to see if the interval contains the null rate of 0.70.

Compare incidence of Vomiting to historical rate of 0.15 from D9803, which is Randomized Study of VCR, Actinomycin-D, and CPM (VAC) vs. VAC alternating with VCR, Topotecan, and CPM (VTC) for Patients with Intermediate Risk RMS. The 95% one-sided confidence interval for the incidence of vomiting will be calculated and evaluated to see if the interval contains the null rate of 0.15.

Compare incidence of Anemia to historical rate of 0.40 from D9803, which is Randomized Study of VCR, Actinomycin-D, and CPM (VAC) vs. VAC alternating with VCR, Topotecan, and CPM (VTC) for Patients with Intermediate Risk RMS. 95% one-sided confidence interval for the incidence of anemia will be calculated and evaluated to see if the interval contains the null rate of 0.40.

Anemia

Febrile Neutropenia

Nausea or Hepatopathy

Platelet Count Decreased

Vomiting

VAC (Weeks 1-15)

VAC (Weeks 31 - 43)

Number of patients with specific adverse events.

Acute and Late Effects of VAC as Delivered on This Study to D9803 VAC

% Change in SUVmax From Baseline to Week 4 < 40%

% Change in SUVmax From Baseline to Week 4 >= 40%

452 participants were excluded due to ineligibility or absence of SUVmax evaluation at baseline and week 4.

Compare Event Free Survival (EFS) With Respect to the Level of % Change in FDG PET Maximum Standard Uptake Value (SUVmax) at Week 4

% Change in SUVmax From Baseline to Week 15 < 40%

% Change in SUVmax From Baseline to Week 15 >= 40%

421 participants were excluded due to ineligibility or absence of SUVmax evaluation at baseline and week 15.

Compare Event Free Survival (EFS) With Respect to the Level of % Change in FDG PET Maximum Standard Uptake Value (SUVmax) at Week 15

The incidences of grade 3 or higher neutropenia, with or without fever between UGT1A1 Genotypes (6/6, 6/7, 7/7) were compared by the Fisher's exact test.

The incidences of grade 3 or higher diarrhea between UGT1A1 Genotypes (6/6, 6/7, 7/7) were compared by Fisher's exact test.

Neutropenia, with or without Fever

Diarrhea

UGT1A1 Genotype 6/6

UGT1A1 Genotype 6/7

UGT1A1 Genotype 7/7

Ineligible patients are excluded. Only patients tested for the UGT1A1 genotypes are reported and included in this analysis.

Incidence of Toxicity Related to VI Treatment in Patients With UGT1A1 Genotype

The analysis was abandoned due to low incidence of toxicity. Data were not collected.

Toxicity With CYP2B6 Genotypes

Toxicity With GSTA1 and CYP2C9 Genotypes

PAX3

PAX7

Only eligible patients who were tested for their fusion status and PAX partners.

Event Free Survival (EFS) by PAX Status

470 participants were excluded due to ineligibility or absence of the dysfunctional voiding and incontinence symptoms questionnaire.

Study title	NCT ID	Conditions	Interventions	Eligibility	Organization
Collecting and Storing Tissue, Blood, and Bone Marrow Samples From Patients With Rhabdomyosarcoma or Other Soft Tissue Sarcoma	NCT00919269	Adult Rhabdomyo... Childhood Desmo... Chordoma Desmoid-Type Fi... Metastatic Chil... Non-Metastatic ... Previously Trea... Recurrent Adult... Recurrent Child... Recurrent Child... Rhabdomyosarcom... Stage I Adult S... Stage II Adult ... Stage III Adult... Stage IV Adult ... Untreated Child...	Cytology Specim... Laboratory Biom...	- 50 Years	Children's Oncology Group
Preoperative Thalidomide With Radiation Therapy For Patients With Low-Grade Primary Soft Tissue Sarcoma or Thalidomide With Radiation Therapy and Chemotherapy For Patients With High-Grade or Intermediate-Grade Primary Soft Tissue Sarcoma of the Arm, Leg, or Body Wall	NCT00089544	Recurrent Adult... Stage I Adult S... Stage II Adult ... Stage III Adult...	Dacarbazine Doxorubicin Hyd... Filgrastim Ifosfamide Laboratory Biom... Radiation Thera... Thalidomide Therapeutic Con...	16 Years -	National Cancer Institute (NCI)
Preoperative Thalidomide With Radiation Therapy For Patients With Low-Grade Primary Soft Tissue Sarcoma or Thalidomide With Radiation Therapy and Chemotherapy For Patients With High-Grade or Intermediate-Grade Primary Soft Tissue Sarcoma of the Arm, Leg, or Body Wall	NCT00089544	Recurrent Adult... Stage I Adult S... Stage II Adult ... Stage III Adult...	Dacarbazine Doxorubicin Hyd... Filgrastim Ifosfamide Laboratory Biom... Radiation Thera... Thalidomide Therapeutic Con...	16 Years -	National Cancer Institute (NCI)
Combination Chemotherapy and Radiation Therapy in Treating Patients With Newly Diagnosed Rhabdomyosarcoma	NCT00354835	Adult Rhabdomyo... Childhood Alveo... Childhood Botry... Childhood Embry... Localized Child... Rhabdomyosarcom... Sarcoma Stage I Adult S... Stage II Adult ... Stage III Adult...	Cyclophosphamid... Dactinomycin Irinotecan Hydr... Laboratory Biom... Questionnaire A... Radiation Thera... Vincristine Sul...	- 49 Years	Children's Oncology Group

Spots Global Cancer Trial Database for stage iii adult soft tissue sarcoma ajcc v7