International Ovarian & Testicular Stromal Tumor Registry (OTST)

March 17, 2023 updated by: Kris Ann Schultz, Children's Hospitals and Clinics of Minnesota

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 of 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

Study Overview

Status

Recruiting

Conditions

Detailed Description

The Registry collects and analyzes case-by-case data on ovarian and testicular 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.

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), 3) paraffin blocks and/or scrolls and 4) fresh tissue. In some cases, saliva samples, buccal swabs or urine 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.

Biologic specimens will be banked and stored for future research.

Study Type

Observational

Enrollment (Anticipated)

300

Contacts and Locations

This section provides the contact details for those conducting the study, and information on where this study is being conducted.

Study Contact

Study Contact Backup

Study Locations

  • United States
    • Minnesota
      • Minneapolis, Minnesota, United States, 55404
        • Recruiting
        • Children's Minnesota
        • Contact:
        • Contact:
        • Principal Investigator:
          • Kris Ann P Schultz, MD

Participation Criteria

Researchers look for people who fit a certain description, called eligibility criteria. Some examples of these criteria are a person's general health condition or prior treatments.

Eligibility Criteria

Ages Eligible for Study

1 second to 100 years (Child, Adult, Older Adult)

Accepts Healthy Volunteers

Yes

Genders Eligible for Study

All

Sampling Method

Non-Probability Sample

Study Population

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

Description

Inclusion Criteria:

  • Previous or current diagnosis of an ovarian sex cord stromal including but not limited to: Sertoli-Leydig cell tumor, gynandroblastoma (now enrolling these patients on PPB/DICER1 Registry), 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:

  • Unable to provide informed consent/assent
  • Adult Granulosa cell tumor (unless otherwise specified by Medical Director)

Study Plan

This section provides details of the study plan, including how the study is designed and what the study is measuring.

How is the study designed?

Design Details

  • Observational Models: Other
  • Time Perspectives: Other

Cohorts and Interventions

Group / Cohort
Ovarian Stromal Tumors
Testicular Stromal Tumors
Ovarian Small Cell Carcinoma

What is the study measuring?

Primary Outcome Measures

Outcome Measure
Measure Description
Time Frame
DICER1 mutations in ovarian and testicular stromal tumors
Time Frame: 3 years
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.
3 years

Secondary Outcome Measures

Outcome Measure
Measure Description
Time Frame
Clinical factors associated with outcome in ovarian and testicular stromal tumors
Time Frame: 10 years
We will analyze the clinical records to determine which factors influence prognosis
10 years
Pathologic and Genetic Correlations
Time Frame: 5 years
We will correlate the pathologic and genetic data with clinical data to describe factors associated with poor prognosis.
5 years

Collaborators and Investigators

This is where you will find people and organizations involved with this study.

Sponsor

Children's Hospitals and Clinics of Minnesota

Collaborators

M.D. Anderson Cancer Center
Massachusetts General Hospital
Washington University School of Medicine
Dana-Farber Cancer Institute
Children's Hospital Colorado
University of Texas Southwestern Medical Center
Children's National Research Institute
University of Cambridge
Phoenix Children's Hospital
Rutgers University
Allina Health System
Klinikum Dortmund Wirbelsäulenchirurgie
ResourcePath, LLC

Investigators

  • Principal Investigator: Kris Ann P Schultz, MD, Children's Minnesota

Publications and helpful links

The person responsible for entering information about the study voluntarily provides these publications. These may be about anything related to the study.

General Publications

Helpful Links

Study record dates

These dates track the progress of study record and summary results submissions to ClinicalTrials.gov. Study records and reported results are reviewed by the National Library of Medicine (NLM) to make sure they meet specific quality control standards before being posted on the public website.

Study Major Dates

Study Start (Actual)

December 8, 2011

Primary Completion (Anticipated)

December 1, 2028

Study Completion (Anticipated)

December 1, 2030

Study Registration Dates

First Submitted

October 23, 2013

First Submitted That Met QC Criteria

October 23, 2013

First Posted (Estimate)

October 28, 2013

Study Record Updates

Last Update Posted (Actual)

March 21, 2023

Last Update Submitted That Met QC Criteria

March 17, 2023

Last Verified

March 1, 2023

More Information

Terms related to this study

Keywords

Additional Relevant MeSH Terms

Other Study ID Numbers

  • International OTST Registry

Drug and device information, study documents

Studies a U.S. FDA-regulated drug product

No

Studies a U.S. FDA-regulated device product

No

This information was retrieved directly from the website clinicaltrials.gov without any changes. If you have any requests to change, remove or update your study details, please contact register@clinicaltrials.gov. As soon as a change is implemented on clinicaltrials.gov, this will be updated automatically on our website as well.

Clinical Trials on Ovarian Small Cell Carcinoma

Search Similar Trials

Sponsors and Collaborators

Medical Conditions

Drug Interventions

CROs by country

CROs in Bosnia & Herzegovina

Conditions

Rare Diseases

Drug Interventions

Dietary Supplements

Sponsor/Collaborators

Locations

3
Subscribe