Trial record 1 of 175013 for:    ALL
Previous Study | Return to List | Next Study

International Ovarian & Testicular Stromal Tumor Registry (OTST)

This study is currently recruiting participants. (see Contacts and Locations)
Verified June 2014 by Children's Hospitals and Clinics of Minnesota
Sponsor:
Collaborators:
Children's Research Institute
Dana-Farber Cancer Institute
Washington University School of Medicine
University of Texas Southwestern Medical Center
M.D. Anderson Cancer Center
Children's Hospital Medical Center, Cincinnati
Information provided by (Responsible Party):
Kris Ann Schultz, Children's Hospitals and Clinics of Minnesota
ClinicalTrials.gov Identifier:
NCT01970696
First received: October 23, 2013
Last updated: June 25, 2014
Last verified: June 2014
  Purpose

Rare tumors are understudied, yet have the potential to shed light on vast areas of cancer research. Ovarian sex cord-stromal tumors, rare tumors of childhood and young adulthood, have recently been found to be associated with a lung cancer of early childhood called pleuropulmonary blastoma (PPB). The cause of these ovarian tumors is unknown. DICER1 mutations are seen in the majority of children with PPB. Research shows DICER1 mutations are also seen in some patients with ovarian tumors. Like PPB, ovarian stromal tumors are highly curable when found in early stage; however, later forms of the disease are aggressive and often fatal. The International Ovarian Stromal Tumor Registry collects clinical and biologic data to understand why these tumors occur and how to treat them. Current work involves the study the role of DICER1 and miRNA expression in ovarian stromal tumors. Understanding the clinical history, predisposing factors and DICER1 and miRNA expression in these ovarian tumors of childhood will lead to targeted screening and risk stratification for evidence-based treatment and biologically rational therapies. These efforts will improve the lives of children by increasing survival and reducing late effects.

The specific goals of the International Ovarian and Testicular Stromal Tumor Registry are:

  1. to understand risk factors by studying age, pathologic subtype, histopathologic features, tumor invasiveness, degree of differentiation, presence of metastasis
  2. to collect information on personal and family history in order to refine the clinical characteristics of patients and families with and without germline DICER1 mutations and other genetic predisposing factors
  3. to determine whether there is a pattern of gene expression or DNA alterations that correlate with predisposition to ovarian tumors, biologic behavior and clinical outcome
  4. to determine optimal screening regimens
  5. to use clinical data obtained through the Registry to refine treatment algorithms
  6. to establish a collection of annotated biology specimens (tumor tissue and germline DNA) for future research

Condition
Ovarian Stromal Tumor
Testicular Stromal Tumors
Ovarian Small Cell Carcinoma

Study Type: Observational [Patient Registry]
Target Follow-Up Duration: 10 Years
Official Title: International Ovarian & Testicular Stromal Tumor Registry

Resource links provided by NLM:


Further study details as provided by Children's Hospitals and Clinics of Minnesota:

Primary Outcome Measures:
  • DICER1 mutations in ovarian and testicular stromal tumors [ Time Frame: 3 years ] [ Designated as safety issue: No ]
    We will analyze germline DNA from patients with gonadal stromal tumors to determine the presence of DICER1 mutations. The tumor tissue will also be sequenced. mRNA and miRNA expression will be analyzed on tumor tissue. Results of the above will be correlated with clinical outcome.


Secondary Outcome Measures:
  • Clinical factors associated with outcome in ovarian and testicular stromal tumors [ Time Frame: 3 years ] [ Designated as safety issue: No ]
    We will analyze the clinical records to determine which factors influence prognosis

  • Pathologic and Genetic Correlations [ Time Frame: 3 years ] [ Designated as safety issue: No ]
    We will correlate the pathologic and genetic data with clinical data to describe factors associated with poor prognosis. We will look for targetable mutations for later stage and recurrent tumors.


Biospecimen Retention:   Samples With DNA

1) whole blood for DNA extraction and lymphoblastoid cell line generation, 2) stained and unstained slides and snap frozen tumor tissue (if available), and 3) paraffin blocks.


Estimated Enrollment: 100
Study Start Date: December 2011
Estimated Primary Completion Date: December 2015 (Final data collection date for primary outcome measure)
Groups/Cohorts
Ovarian Stromal Tumors
Testicular Stromal Tumors
Ovarian Small Cell Carcinoma

Detailed Description:

The Registry collects and analyzes case-by-case data on ovarian stromal tumors. Cases are identified:

  1. by referrals from clinicians or pathologists
  2. by families initiating contact with the Registry
  3. by Registry requests to authors of published cases to share further details

The data collected include:

  1. clinical and laboratory findings
  2. family history
  3. imaging studies
  4. surgery records
  5. pathology records including review and study of pathology materials
  6. treatment (surgery, chemotherapy, radiation)
  7. recurrences or metastases
  8. long-term follow-up

The demographic and clinical data are abstracted into a database secured by password protection. Each record in the database has a unique Registry number.

Enrollment in the OTST Registry is based on local diagnosis, but central pathology review is offered as a part of Registry procedures. DICER1 testing is offered but not required.

For each patient enrolled, the Registry will request 1) whole blood for DNA extraction and lymphoblastoid cell line generation 2) slides or snap frozen tumor tissue (if available), and 3) paraffin blocks. In some cases, saliva samples or buccal swab samples will be obtained for DNA extraction.

Pathology materials are centrally reviewed when available. Any discrepancies in the diagnostic interpretation are discussed with the submitting pathologist or clinician. When the central review pathologist cannot confirm diagnosis of a stromal tumor, the referring physician is notified. The local pathologist retains responsibility for the final pathological diagnosis. It is the responsibility of the referring physician to notify the patient regarding any discrepancy found.

DICER1 kits containing consents, medical testing requisition forms, as well as UN3373 approved specimen shipping containers with two ACD tubes and instructions for collection and mailing are prepared and sent to families. Participants arrange for phlebotomy services with their physicians. Biologic specimens will be banked and stored for future research.

  Eligibility

Genders Eligible for Study:   Both
Accepts Healthy Volunteers:   Yes
Sampling Method:   Non-Probability Sample
Study Population

Prospectively or retrospectively diagnosed patients with ovarian or testicular stromal tumors or ovarian small cell carcinoma

Criteria

Inclusion Criteria:

  • Previous or current diagnosis of an ovarian sex cord stromal including but not limited to: Sertoli-Leydig cell tumor, gynandroblastoma, juvenile granulosa cell tumor, Sertoli cell tumor, sex cord stromal tumor with annular tubules or undifferentiated stromal tumor
  • Previous or current diagnosis of a testicular stromal tumor including but not limited to: juvenile granulosa cell tumor, Sertoli cell tumor, Leydig cell tumor or undifferentiated stromal tumor

Exclusion Criteria:

  • No clinical or biologic material available for review
  • Unable to provide informed consent/assent
  Contacts and Locations
Choosing to participate in a study is an important personal decision. Talk with your doctor and family members or friends about deciding to join a study. To learn more about this study, you or your doctor may contact the study research staff using the Contacts provided below. For general information, see Learn About Clinical Studies.

Please refer to this study by its ClinicalTrials.gov identifier: NCT01970696

Locations
United States, Minnesota
Children's Hospital & Clinics of Minnesota Recruiting
Minneapolis, Minnesota, United States, 55404
Contact: Kris Ann P Schultz, MD    612-813-5940    OTST@childrensMN.org   
Contact: Anne K Harris    612-813-5861    OTST@childrensMN.org   
Principal Investigator: Kris Ann P Schultz, MD         
Sponsors and Collaborators
Children's Hospitals and Clinics of Minnesota
Children's Research Institute
Dana-Farber Cancer Institute
Washington University School of Medicine
University of Texas Southwestern Medical Center
M.D. Anderson Cancer Center
Children's Hospital Medical Center, Cincinnati
Investigators
Principal Investigator: Kris Ann P Schultz, MD Children's Hospital and Clinics of Minnesota
  More Information

Additional Information:
Publications:

Responsible Party: Kris Ann Schultz, Kris Ann P. Schultz, MD, Children's Hospitals and Clinics of Minnesota
ClinicalTrials.gov Identifier: NCT01970696     History of Changes
Other Study ID Numbers: International OTST Registry
Study First Received: October 23, 2013
Last Updated: June 25, 2014
Health Authority: United States: Institutional Review Board

Keywords provided by Children's Hospitals and Clinics of Minnesota:
ovarian
testicular
stromal
Sertoli
Leydig
gynandroblastoma
granulosa cell tumor
DICER1

Additional relevant MeSH terms:
Neoplasms
Carcinoma, Small Cell
Small Cell Lung Carcinoma
Carcinoma
Neoplasms, Glandular and Epithelial
Neoplasms by Histologic Type
Carcinoma, Bronchogenic
Bronchial Neoplasms
Lung Neoplasms
Respiratory Tract Neoplasms
Thoracic Neoplasms
Neoplasms by Site
Lung Diseases
Respiratory Tract Diseases

ClinicalTrials.gov processed this record on September 18, 2014