Trial Title:
International Ovarian & Testicular Stromal Tumor Registry
NCT ID:
NCT01970696
Condition:
Ovarian Sex-cord Stromal Tumor
Testicular Stromal Tumors
Ovarian Small Cell Carcinoma
Conditions: Official terms:
Neoplasms
Carcinoma, Small Cell
Small Cell Lung Carcinoma
Sex Cord-Gonadal Stromal Tumors
Conditions: Keywords:
ovarian
testicular
stromal
sertoli
leydig
juvenile granulosa cell tumor
gynandroblastoma
Study type:
Observational [Patient Registry]
Overall status:
Recruiting
Study design:
Time perspective:
Other
Summary:
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
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.
Criteria for eligibility:
Study pop:
Prospectively or retrospectively diagnosed patients with ovarian or testicular stromal
tumors or ovarian small cell carcinoma
Sampling method:
Non-Probability Sample
Criteria:
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)
Gender:
All
Minimum age:
0 Years
Maximum age:
100 Years
Healthy volunteers:
Accepts Healthy Volunteers
Locations:
Facility:
Name:
Children's Minnesota
Address:
City:
Minneapolis
Zip:
55404
Country:
United States
Status:
Recruiting
Contact:
Last name:
Kris Ann P Schultz, MD, MS
Phone:
612-813-5940
Email:
OTST@childrensMN.org
Contact backup:
Last name:
Anne K Harris
Phone:
612-813-5861
Email:
OTST@childrensMN.org
Investigator:
Last name:
Kris Ann P Schultz, MD
Email:
Principal Investigator
Start date:
December 8, 2011
Completion date:
December 2030
Lead sponsor:
Agency:
Children's Hospitals and Clinics of Minnesota
Agency class:
Other
Collaborator:
Agency:
Children's National Research Institute
Agency class:
Other
Collaborator:
Agency:
Dana-Farber Cancer Institute
Agency class:
Other
Collaborator:
Agency:
Washington University School of Medicine
Agency class:
Other
Collaborator:
Agency:
University of Texas Southwestern Medical Center
Agency class:
Other
Collaborator:
Agency:
M.D. Anderson Cancer Center
Agency class:
Other
Collaborator:
Agency:
Children's Hospital Colorado
Agency class:
Other
Collaborator:
Agency:
Rutgers University
Agency class:
Other
Collaborator:
Agency:
Massachusetts General Hospital
Agency class:
Other
Collaborator:
Agency:
Klinikum Dortmund Wirbelsäulenchirurgie
Agency class:
Other
Collaborator:
Agency:
ResourcePath, LLC
Agency class:
Other
Collaborator:
Agency:
Allina Health System
Agency class:
Other
Collaborator:
Agency:
Phoenix Children's Hospital
Agency class:
Other
Collaborator:
Agency:
University of Cambridge
Agency class:
Other
Source:
Children's Hospitals and Clinics of Minnesota
Record processing date:
ClinicalTrials.gov processed this data on November 12, 2024
Source: ClinicalTrials.gov page:
https://clinicaltrials.gov/ct2/show/NCT01970696
http://www.OTSTregistry.org
http://www.PPBregistry.org
