Symptoms and General Pathology

All Conditions

Neoplasms

Rare Diseases

Recurrence

Sarcoma

Desmoplastic Small Round Cell Tumor

Neoplasms, Connective and Soft Tissue

Neoplasms by Histologic Type

Soft Tissue Sarcoma

DNA analysis

RNA analysis

gene expression analysis

mutation analysis

nucleic acid sequencing

polymerase chain reaction

laboratory biomarker analysis

pharmacogenomic studies

Pooja Hingorani, MD

RATIONALE: Studying samples of blood and tumor tissue from patients with cancer in the laboratory may help doctors learn more about changes that occur in DNA and identify biomarkers related to cancer.

PURPOSE: This research study is studying genes in samples from younger patients with desmoplastic small round cell tumor registered on COG-D9902 or COG-ABTR01B1.

OBJECTIVES:

* To perform whole-exome sequencing on desmoplastic small round cell tumor (DSRCT) samples and their available matched normal samples to identify novel mutations, single nucleotide polymorphisms, or copy number changes associated with these tumors.
* To identify regions of interest that may be involved in the pathogenesis (including the region of EWSR1-WT1 translocation) from the whole-exome sequencing and perform detailed resequencing and transcriptone sequencing to further define the molecular aberrations at the RNA level.

OUTLINE: This is a multicenter study.

Archived tumor tissue samples are analyzed for DNA sequencing, effects of RNA on gene expression levels, novel RNA isoforms, splice variants, and translocations.

Studying Genes in Samples From Younger Patients With Desmoplastic Small Round Cell Tumor Registered on COG-D9902 or COG-ABTR01B1

Comprehensive Genome Sequencing of Desmoplastic Small Round Cell Tumors

National Cancer Institute (NCI)

Children's Oncology Group

Nervous System Diseases

Skin and Connective Tissue Diseases

Diseases and Abnormalities at or Before Birth

Liposarcoma

Fibrosarcoma

Hemangiosarcoma

Fibromatosis, Aggressive

Leiomyosarcoma

Sarcoma, Synovial

Neurofibrosarcoma

Chordoma

Sarcoma, Alveolar Soft Part

Mesenchymoma

Neoplasms, Adipose Tissue

Neoplasms, Connective Tissue

Neoplasms, Fibrous Tissue

Neoplasms, Germ Cell and Embryonal

Neoplasms, Vascular Tissue

Neurofibromatoses

Neurofibroma

Nerve Sheath Neoplasms

Neoplasms, Nerve Tissue

Nervous System Neoplasms

Peripheral Nervous System Neoplasms

Peripheral Nervous System Diseases

Neuromuscular Diseases

Desmoid Tumor

Synovial Sarcoma

Malignant Peripheral Nerve Sheath Tumor

Epithelioid Sarcoma

Alveolar Soft Part Sarcoma

Malignant Mesenchymoma

Neurofibromatosis

Archived DNA tissue samples are analyzed for frequency of genetic mutations, including SNPs, SNVs, and small deletions and/or insertions, by PCR and mass spectometry (Sequenom MassARRAY). Results are then analyzed to determine whether specific mutations correlate with patient or disease features such as tumor stage, histological grade, or outcome.

Steve Skapek, MD

This research trial studies genes in tissue samples from younger and adolescent patients with soft tissue sarcomas. Studying samples of tumor tissue from patients with cancer in the laboratory may help doctors learn more about changes that occur in DNA and identify biomarkers related to cancer. It may also help doctors find better ways to treat cancer

Study Subtype: Ancillary/Correlative Observational Study Model: Cohort Time Perspective: Retrospective Biospecimen Retention: Samples With DNA Biospecimen Description: Tissue Study Population Description: Existing NRSTS samples from the COG D9902/ARST0332 studies Sampling Method: Non-Probability Sample

OBJECTIVES:

I. To determine the frequency with which actionable mutations are found in archived non-rhabdomyosarcoma soft tissue sarcoma (NRSTS) tumor specimens using mass spectrometry (MS) analysis of tumor-derived deoxyribonucleic acid (DNA).

OUTLINE:

Archived DNA tissue samples are analyzed for frequency of genetic mutations, including single nucleotide polymorphisms (SNPs), single nucleotide variants (SNVs), and small deletions and/or insertions, by polymerase chain reaction (PCR) and mass spectometry (Sequenom MassARRAY). Results are then analyzed to determine whether specific mutations correlate with patient or disease features such as tumor stage, histological grade, or outcome.

Studying Genes in Tissue Samples From Younger and Adolescent Patients With Soft Tissue Sarcomas

Observational - Potentially Actionable Mutations in Archived Non-Rhabdomyosarcoma Soft Tissue Sarcomas (NRSTS)

Behaviors and Mental Disorders

Rhabdomyosarcoma

Rhabdomyosarcoma, Embryonal

Aggression

Fibroma

Disease Attributes

Myosarcoma

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

Study title	NCT ID	Conditions	Interventions	Eligibility	Organization
Studying Genes in Samples From Younger Patients With Desmoplastic Small Round Cell Tumor Registered on COG-D9902 or COG-ABTR01B1	NCT01430637	Sarcoma	DNA analysis RNA analysis gene expression... mutation analys... nucleic acid se... polymerase chai... laboratory biom... pharmacogenomic...	- 50 Years	Children's Oncology Group
Studying Genes in Tissue Samples From Younger and Adolescent Patients With Soft Tissue Sarcomas	NCT01567046	Childhood Alveo... Childhood Angio... Childhood Desmo... Childhood Epith... Childhood Fibro... Childhood Leiom... Childhood Lipos... Childhood Malig... Childhood Neuro... Childhood Synov... Chordoma Desmoid Tumor Metastatic Chil... Nonmetastatic C... Recurrent Child...	laboratory biom...	- 30 Years	Children's Oncology Group
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
Studying Genes in Tissue Samples From Younger and Adolescent Patients With Soft Tissue Sarcomas	NCT01567046	Childhood Alveo... Childhood Angio... Childhood Desmo... Childhood Epith... Childhood Fibro... Childhood Leiom... Childhood Lipos... Childhood Malig... Childhood Neuro... Childhood Synov... Chordoma Desmoid Tumor Metastatic Chil... Nonmetastatic C... Recurrent Child...	laboratory biom...	- 30 Years	Children's Oncology Group

Spots Global Cancer Trial Database for childhood desmoplastic small round cell tumor