International Ovarian & Testicular Stromal Tumor Registry (OTST)
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| ClinicalTrials.gov Identifier: NCT01970696 |
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Recruitment Status :
Recruiting
First Posted : October 28, 2013
Last Update Posted : May 20, 2019
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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:
- to understand risk factors by studying age, pathologic subtype, histopathologic features, tumor invasiveness, degree of differentiation, presence of metastasis
- 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
- 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
- to determine optimal screening regimens
- to use clinical data obtained through the Registry to refine treatment algorithms
- to establish a collection of annotated biology specimens (tumor tissue and germline DNA) for future research
| Condition or disease |
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| Ovarian Stromal Tumor Testicular Stromal Tumors Ovarian Small Cell Carcinoma DICER1 Gene Mutation |
The Registry collects and analyzes case-by-case data on ovarian 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), and 3) paraffin blocks. 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 [Patient Registry] |
| Estimated Enrollment : | 200 participants |
| Observational Model: | Other |
| Time Perspective: | Other |
| Target Follow-Up Duration: | 10 Years |
| Official Title: | International Ovarian & Testicular Stromal Tumor Registry |
| Actual Study Start Date : | December 8, 2011 |
| Estimated Primary Completion Date : | December 2025 |
| Estimated Study Completion Date : | December 2025 |
| Group/Cohort |
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| Ovarian Stromal Tumors |
| Testicular Stromal Tumors |
| Ovarian Small Cell Carcinoma |
- 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.
- Clinical factors associated with outcome in ovarian and testicular stromal tumors [ Time Frame: 3 years ]We will analyze the clinical records to determine which factors influence prognosis
- Pathologic and Genetic Correlations [ Time Frame: 3 years ]We will correlate the pathologic and genetic data with clinical data to describe factors associated with poor prognosis.
Biospecimen Retention: Samples With DNA
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| Ages Eligible for Study: | up to 100 Years (Child, Adult, Older Adult) |
| Sexes Eligible for Study: | All |
| Accepts Healthy Volunteers: | Yes |
| Sampling Method: | Non-Probability Sample |
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:
- Unable to provide informed consent/assent
To learn more about this study, you or your doctor may contact the study research staff using the contact information provided by the sponsor.
Please refer to this study by its ClinicalTrials.gov identifier (NCT number): NCT01970696
| Contact: Anne Harris, MPH | 612-813-5861 | anne.harris@childrensmn.org |
| United States, Minnesota | |
| Children's Minnesota | Recruiting |
| Minneapolis, Minnesota, United States, 55404 | |
| Contact: Kris Ann P Schultz, MD, MS 612-813-5940 OTST@childrensMN.org | |
| Contact: Anne K Harris 612-813-5861 OTST@childrensMN.org | |
| Principal Investigator: Kris Ann P Schultz, MD | |
| Principal Investigator: | Kris Ann P Schultz, MD | Children's Minnesota |
Additional Information:
Publications of Results:
Other Publications:
| Responsible Party: | Kris Ann Schultz, Kris Ann P. Schultz, MD, MS, Children's Hospitals and Clinics of Minnesota |
| ClinicalTrials.gov Identifier: | NCT01970696 History of Changes |
| Other Study ID Numbers: |
International OTST Registry |
| First Posted: | October 28, 2013 Key Record Dates |
| Last Update Posted: | May 20, 2019 |
| Last Verified: | May 2019 |
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ovarian testicular stromal Sertoli |
Leydig gynandroblastoma juvenile granulosa cell tumor DICER1 |
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Carcinoma, Small Cell Small Cell Lung Carcinoma Neoplasms 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 |