OTST Blog

The 29th annual Pine Tree Apple Tennis Classic raised more than $200,000 for Childhood Cancer Research and the International OTST Registry!!! Thank you to all who supported this amazing event. Stay tuned for our 30th next year!

http://www.childrensmn.org/ptatc/

Exciting News!!! Decemeber 11th marks the International OTST Registry's 2-year anniversary. Since inception, the registry has enrolled 55 patients from 4 continents, 6 countries and 22 states. Over the next year, the OTST Registry will publish and present preliminary clinical and genetic findings, advance pathology knowledge through molecular and genetic studies, raise ovarian and testicular stromal tumor awareness and expand the cohort of OTST Registry patients. We are grateful to our collaborating partners, sponsors and especially patients.  Thanks to all of you our research is thriving, and together we will lead the way for improved care for children, adolescents and adults with ovarian and testicular cancer.

Although most participants in the OTST Registry so far have a diagnosis of ovarian stromal tumor, the Registry is also studying testicular stromal tumors. We are working closely with Dr. Nicholas Cost, a pediatric urologist from Colorado Children’s Hospital to study testicular tumors. Please join us in welcoming Dr. Cost to the OTST Blog.

Testicular Stromal Tumor Blog Post

“Study on Clinical and Pathologic Risk Factors in Stage I Testicular Stromal Tumors”

            Testicular stromal tumors are rare (3-5% of all testicular tumors) and include the following subtypes: Leydig cell tumors, Sertoli Cell Tumors, Granulosa Cell Tumors, Mixed Tumors, and Unclassified Stromal Tumors. Overall, most testicular stromal tumors (90%) will present with clinically localized disease (Stage I), confined to the testicle alone. However, up to 10% are malignant and either present with metastasis or will relapse while on surveillance after orchiectomy (removal of the testis) for what was presumed to be Stage I disease.

Given that 90% of testicular stromal tumors are Stage I, the question that troubles the majority of patients and doctors is how to ideally manage those who initially appear to be cured by simply removing of the testicular tumor? Clearly, treating every Stage I patient with additional surgery or chemotherapy to prevent future recurrence would expose up to 90% of patients to unnecessary therapy. However, there may be a threshold of risk that would make such “prophylactic” treatment worth the potential side-effects. In an ideal world, we would identify which clinical and pathologic criteria would predispose the Stage I patients to later recurrence. Previous investigations have identified that some pathologic information from the testicular tumor can indicate an increased risk for later relapse. 

This existing data is unquestionably important, but only serves as a starting point since we still do not understand how much risk each of these features pose and if they act cumulatively such that more “risk factors” places a patient at more risk for recurrence. We have now started a study designed to review all known published literature of patients with Stage I testicular stromal tumors.  These cases will be reviewed to collect data on the presence or absence of the risk factors and other clinical and pathologic features. We will then compare the cases which did or did not experience disease relapse and analyze which of these risk factors are associated with recurrence and try to determine how much risk is associated with each factor.

If you are interested in learning more about this study of testicular stromal tumors, please contact us. Thank you in advance for your interest in understanding how to best treat patients with these rare tumors!

First off, thank you to all our OTST Registry participants. Thanks to you, the Registry has now enrolled 50 participants with ovarian and testicular stromal tumors from 4 continents, 6 countries and 20 states. We continue to try to learn as much as possible about these rare tumors. Our current research efforts focus on the biology (including cause) and treatment of these tumors.

Our first efforts have focused on determining how often people with ovarian stromal tumor carry DICER1 mutations. Next we will try to understand more about how DICER1 mutations lead to ovarian tumors using mRNA and miRNA analyses.

In the meantime, we're also studying how to best treat these tumors. We're working with Children's Oncology Group and Gynecology Oncology Group. Gynecology Oncology Group has an open clinical trial which offers treatment with one of two regimens for patients who require chemotherapy but have not yet received it. The two treatment regimens are: cisplatin/etoposide/bleomycin (PEB) and carboplatin/paclitaxel. We also continue to study these tumors to figure out which regimens might be best for tumors that recur after initial treatment.