Ovarian Sex-cord Stromal TumorOTSTNCT01970696
International Ovarian & Testicular Stromal Tumor Registry
An observational study of Ovarian Sex-cord Stromal Tumor and Testicular Stromal Tumors, run by Children's Hospitals and Clinics of Minnesota at 1 site in United States.
Trial 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). Read more about this study
- Who is it for?
- Any age
- Conditions the study is for
- Ovarian Sex-cord Stromal Tumor, Testicular Stromal Tumors, Ovarian Small Cell Carcinoma
- How many people
- 500 participants
- Where
- 1 siteUnited States
- Study dates
- 8 Dec 2011to 31 Dec 2035
- How long will I take part?
- 10 Years
- Phase
- Observational
Key requirements
Who can take part, as listed by the sponsor. Only the study team can confirm whether you are eligible, so take this list to your doctor.
- Ages Any age
- All sexes
- No healthy volunteers
- Child, Adult, Older adult
Study population: The International OTST Registry is established in collaboration with Children's Minnesota.
All patients with a suspected gonadal stromal tumor or a similar tumor. International enrollment is encouraged. Patients of any age are eligible, but based on epidemiology and referral patterns, most participants are expected to be under age 20.
Trial locations and contacts
1 site in 1 country. Contact the study team or a site near you to ask about taking part.
United States1 site, 1 recruiting
- Recruiting
Children's Minnesota
Minneapolis, Minnesota, 55404
Kris Ann P Schultz, MD, MS, 612-813-5940, OTST@childrensMN.org
Emily Armstrong, BS, 612-813-7116, emily.armstrong@childrensmn.org
About this study
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
Read the detailed description
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.
- Keywords
- ovarian, testicular, stromal, sertoli, leydig, juvenile granulosa cell tumor, gynandroblastoma
- Medical subject headings
- Sex Cord-Gonadal Stromal Tumors
What participants receive
What participants receive. In a randomised study each person is assigned to one group by chance.
| Study group | Role | What they receive |
|---|---|---|
| Ovarian Stromal Tumors | Not described | |
| Testicular Stromal Tumors | Not described | |
| Ovarian Small Cell Carcinoma | Not described |
What is being measured
The primary outcome is the main question the study is designed to answer.
DICER1 mutations in ovarian and testicular stromal tumors
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.
Measured: 3 years
Secondary outcomes (2)
Clinical factors associated with outcome in ovarian and testicular stromal tumors
We will analyze the clinical records to determine which factors influence prognosis
Measured: 10 years
Pathologic and Genetic Correlations
We will correlate the pathologic and genetic data with clinical data to describe factors associated with poor prognosis.
Measured: 5 years
Timeline
From the start of the study to its completion; the mark is today. Estimated dates are the sponsor’s plan.
- First posted28 Oct 2013 (estimated)
- Study start8 Dec 2011
- Primary completion31 Dec 2030 (estimated)
- Study completion31 Dec 2035 (estimated)
Resources
- Official record on ClinicalTrials.govThe registry entry this page is built from, with its full change history.
- Publications (27)Papers the sponsor has linked to this study.
- Other trials for Ovarian Sex-cord Stromal TumorStudies of the same condition, open and under way.
- What the trial phases meanHow a treatment moves from first tests in people to approval.
More details
For doctors and researchers: how the study is designed, who runs it, and its registry IDs.
Study design
- Study type
- Observational
- Observational model
- Other
- Time perspective
- Other
- Enrolment
- 500 participants (estimated)
- Target duration
- 10 Years
Sponsor and investigators
- Lead sponsor
- Children's Hospitals and Clinics of Minnesota
- Sponsor type
- University, hospital or other
- Collaborators
- Children's National 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 Colorado, Rutgers University, Massachusetts General Hospital, Klinikum Dortmund Wirbelsäulenchirurgie, ResourcePath, LLC, Allina Health System, Phoenix Children's Hospital, University of Cambridge
- Responsible party
- Principal investigator, Kris Ann Schultz, Children's Hospitals and Clinics of Minnesota
- Investigators
- Kris Ann P Schultz, MD, Principal investigator, Children's Minnesota
IDs, dates and publications
- ClinicalTrials.gov ID
- NCT01970696
- Sponsor's protocol ID
- International OTST Registry
- Acronym
- OTST
- Status last verified
- Oct 2026
- First submitted
- 23 Oct 2013
- Last updated
- 23 Oct 2026
- Results posted
- No
- Data monitoring committee
- No
- FDA-regulated drug
- No
- FDA-regulated device
- No
Publications
Schultz KA, Schneider DT, Pashankar F, Ross J, Frazier L. Management of ovarian and testicular sex cord-stromal tumors in children and adolescents. J Pediatr Hematol Oncol. 2012 May;34 Suppl 2:S55-63. doi: 10.1097/MPH.0b013e31824e3867.
PubMed 22525408Schultz KA, Sencer SF, Messinger Y, Neglia JP, Steiner ME. Pediatric ovarian tumors: a review of 67 cases. Pediatr Blood Cancer. 2005 Feb;44(2):167-73. doi: 10.1002/pbc.20233.
PubMed 15490488Schultz KA, Ness KK, Nagarajan R, Steiner ME. Adnexal masses in infancy and childhood. Clin Obstet Gynecol. 2006 Sep;49(3):464-79. doi: 10.1097/00003081-200609000-00007.
PubMed 16885654Rio Frio T, Bahubeshi A, Kanellopoulou C, Hamel N, Niedziela M, Sabbaghian N, Pouchet C, Gilbert L, O'Brien PK, Serfas K, Broderick P, Houlston RS, Lesueur F, Bonora E, Muljo S, Schimke RN, Bouron-Dal Soglio D, Arseneau J, Schultz KA, Priest JR, Nguyen VH, Harach HR, Livingston DM, Foulkes WD, Tischkowitz M. DICER1 mutations in familial multinodular goiter with and without ovarian Sertoli-Leydig cell tumors. JAMA. 2011 Jan 5;305(1):68-77. doi: 10.1001/jama.2010.1910.
PubMed 21205968Schultz KAP, Nelson AT, Kamihara J, Bauer AJ, Hatton J, Merideth MA, Stratton P, Harris AK, Field A, Dehner LP, Stewart DR, Hill DA, Messinger YH. DICER1-Related Tumor Predisposition. 2014 Apr 24 [updated 2026 Mar 25]. In: Adam MP, Bick S, Mirzaa GM, Wallace SE, Amemiya A, editors. GeneReviews(R) [Internet]. Seattle (WA): University of Washington, Seattle; 1993-2026. Available from http://www.ncbi.nlm.nih.gov/books/NBK196157/
PubMed 24761742Pappo AS, Furman WL, Schultz KA, Ferrari A, Helman L, Krailo MD. Rare Tumors in Children: Progress Through Collaboration. J Clin Oncol. 2015 Sep 20;33(27):3047-54. doi: 10.1200/JCO.2014.59.3632. Epub 2015 Aug 24.
PubMed 26304909Pashankar F, Bisogno G, Ribeiro R, Messinger Y, Schultz KA, Rodriguez-Galindo C. The role of registries and tumor banking in rare pediatric tumors. Curr Pediatr Rep 2015:1-9. Avail online 15 Mar 2015. DOI 10.1007/s40124-015-0077-9.
Schultz KAP and Miller R. (2014) Adnexal Masses in Infancy and Childhood. Coomarasamy A, Davuka W, Shafi M, Chan C (eds): Gynecology and Obstetric Surgery: Challenges and Management Options. Wiley-Blackwell. [BOOK.] [In press; avail may 2015].
Schultz KAP, Frazier L, Schneider DT. (2014) Ovarian and Testicular Sex Cord Stromal Tumors. Frazier, AL, Amatruda JF (eds): Textbook Pediatric Germ Cell Tumors, Pediatric Oncology 1. Berlin Heidelberg. Springer-Verlag. [BOOK.].
Schneider DT, Orbach D, Ben-Ami T, Bien E, Bisogno G, Brecht IB, Cecchetto G, Ferrari A, Godzinski J, Janic D, Lopez Almaraz R, Pourtsidis A, Roganovic J, Schultz KAP, Stachowicz-Stencel T, Fresneau B. Consensus recommendations from the EXPeRT/PARTNER groups for the diagnosis and therapy of sex cord stromal tumors in children and adolescents. Pediatr Blood Cancer. 2021 Jun;68 Suppl 4:e29017. doi: 10.1002/pbc.29017. Epub 2021 Mar 24.
PubMed 33760357Doros L, Yang J, Dehner L, Rossi CT, Skiver K, Jarzembowski JA, Messinger Y, Schultz KA, Williams G, Andre N, Hill DA. DICER1 mutations in embryonal rhabdomyosarcomas from children with and without familial PPB-tumor predisposition syndrome. Pediatr Blood Cancer. 2012 Sep;59(3):558-60. doi: 10.1002/pbc.24020. Epub 2011 Dec 16.
PubMed 22180160Schultz KA, Pacheco MC, Yang J, Williams GM, Messinger Y, Hill DA, Dehner LP, Priest JR. Ovarian sex cord-stromal tumors, pleuropulmonary blastoma and DICER1 mutations: a report from the International Pleuropulmonary Blastoma Registry. Gynecol Oncol. 2011 Aug;122(2):246-50. doi: 10.1016/j.ygyno.2011.03.024. Epub 2011 Apr 17.
PubMed 21501861Schultz KA, Harris A, Williams GM, Baldinger S, Doros L, Valusek P, Frazier AL, Dehner LP, Messinger Y, Hill DA. Judicious DICER1 testing and surveillance imaging facilitates early diagnosis and cure of pleuropulmonary blastoma. Pediatr Blood Cancer. 2014 Sep;61(9):1695-7. doi: 10.1002/pbc.25092. Epub 2014 May 13.
PubMed 24821309Schultz KA, Harris A, Messinger Y, Sencer S, Baldinger S, Dehner LP, Hill DA. Ovarian tumors related to intronic mutations in DICER1: a report from the international ovarian and testicular stromal tumor registry. Fam Cancer. 2016 Jan;15(1):105-10. doi: 10.1007/s10689-015-9831-y.
PubMed 26289771Rutter MM, Jha P, Schultz KA, Sheil A, Harris AK, Bauer AJ, Field AL, Geller J, Hill DA. DICER1 Mutations and Differentiated Thyroid Carcinoma: Evidence of a Direct Association. J Clin Endocrinol Metab. 2016 Jan;101(1):1-5. doi: 10.1210/jc.2015-2169. Epub 2015 Nov 10.
PubMed 26555935