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NCT Number: NCT01970696

International Ovarian & Testicular Stromal Tumor Registry

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

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Key information

Conditions

Ovarian Sex-cord Stromal Tumor Neoplasms Neoplasms by Histologic Type Neoplasms, Gonadal Tissue Ovarian Small Cell Carcinoma Sex Cord-Gonadal Stromal Tumors Testicular Stromal Tumors

Age range

0 year–100 year

Sex eligibility

All sexes

Study type

Observational

Primary location

About this study

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

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

The data collected include:

  • clinical and laboratory findings
  • family history
  • imaging studies
  • surgery records
  • pathology records including review and study of pathology materials
  • treatment (surgery, chemotherapy, radiation)
  • recurrences or metastases
  • 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.

Who can participate

Healthy volunteers accepted: Yes

Only the study team can determine whether someone qualifies for participation.

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)

Treatment and study plan

Primary outcomes

  1. 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.

Secondary outcomes

  1. 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

  2. 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.

Study contacts

Contact information is provided by the study sponsor or research team.

Anne Harris, MPH

CONTACT

[email protected]

612-813-5861

Paige Mallinger, MS

CONTACT

[email protected]

612-813-7121

Sponsors and collaborators

Lead sponsor

Children's Hospitals and Clinics of Minnesota

Other

Collaborators

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

Registry information

Acronym: OTST

Important dates

Study start
2011
Primary completion
2028
Study completion
2030
First posted
Oct 28, 2013
Registry last updated
Aug 22, 2024

OpenTrials presents study information sourced from ClinicalTrials.gov. The official registry record should be consulted for the latest information.

View the official ClinicalTrials.gov record (opens in a new tab)

This listing is for discovery and informational purposes only. It is not medical advice, does not guarantee that a study is recruiting, and does not determine eligibility. Contact the study team and a qualified healthcare professional when considering participation.

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