International Ovarian & Testicular Stromal Tumor (OTST) Registry

Since the inception of the OTST Registry, more than 200 children and adults have enrolled from 39 U.S. states and 15 countries. We would like to send a huge shout out to our participants and their families!

We will be gathering virtually over Teams on Monday, August 19th, from 12:00 to 12:20 PM central time for the International PPB/DICER1 & OTST Registries patient and parent advisory board meeting. If you are interested in joining but haven't received the Teams link yet, you can request the link by emailing [email protected].

Those who attended the PPB/DICER1/OTST Family Meeting had the opportunity to hear about Ansley's amazing journey from PPB patient to high school senior and aspiring peds oncology PA. Only a few moments later, we received a message from one of our collaborating pediatric oncology PAs, offering to serve as a resource and mentor to Ansley along her journey.

There is good everywhere and so many beautiful connections and advances happening thanks to all of you!

Thanks to Ansley and her mom, Amy, for sharing their story and to Adina for her wonderful offer of mentorship!

Our PPB/DICER1/OTST Patient/Parent Advisory Group is expanding! We hope that this will build community and advance research and care. Indicate your interest and availability by completing the Doodle: https://doodle.com/meeting/participate/id/dyMlNvWd

Thank you to the many kids, grownups, families and clinicians worldwide who joined us this week for the International PPB/DICER1/OTST Registries 2024 Family Meeting! Attendees joined in person in St. Paul, Minnesota as well as virtually from across the world including Brazil, Chile, Belarus, Germany, UAE, Australia and New Zealand. We are very grateful for your collaboration as we work together to advance outcomes for these rare tumors.

We value your feedback! Please complete the attendee survey by Friday, August 9th, 2024. You can access the survey here: https://us06web.zoom.us/survey/U9npX27LjSIM4K52OPCGTEgrd-IYqF9p0eMZtM2W5Gyr6Si8Saw.8xqIR6rUneBRSTjN/view?id=qow8hBnJRdyG_O4mqoPJ_g #/sharePreview

Tomorrow is the day! Join us from 9:00am to 1:00pm central time for the International PPB/DICER1/OTST Registries 2024 Family Meeting. If you plan on attending virtually, now is a great time to locate the Zoom meeting link emailed to you following your registration. Also, check out our Instagram page () for the finalized agenda or keep an eye out for a copy via email today!

Last call to register to join virtually via Zoom by clicking this link: https://us06web.zoom.us/webinar/register/WN_21x2OOA7TGmORfuzVTb9HQ

Please contact us at [email protected] with any questions or concerns. We are so excited to share the latest updates on PPB, DICER1-associated tumors, and ovarian and testicular stromal tumors!

Hundreds of tennis players will soon hit the courts to raise money for children battling cancer at the Pine Tree Apple Tennis Classic. All proceeds from this event will support childhood cancer research at Children’s Minnesota.🎾

Dr. Kris Ann Schultz, oncologist and cancer researcher, joined Cheryl Yasis, Pine Tree Apple Classic Fund Board Member, to talk about the impact this tournament and its proceeds has on cancer research — and our patients — at Children's Minnesota. More: https://cbsn.ws/3WoNY5B

The International PPB/DICER1/OTST Registries 2024 Family Meeting is only 2 weeks away!

Register for the Family Meeting here: https://us06web.zoom.us/webinar/register/WN_21x2OOA7TGmORfuzVTb9HQ

RSVP for the Meet-&-Greet here: https://forms.office.com/pages/responsepage.aspx?id=OpIKj12BPUaNw4KG0pTxPCZ15_1WQD5Cj-p4rPqGaVJUQUFKQ0lMUktGMDYyMlBWTUE0OTNGV1Y4RiQlQCN0PWcu

If you plan on joining us in-person: The Family meeting and optional Meet-&-Greet events are free, but please RSVP to both by Monday, July 22, 2024 so that we can plan for your attendance.

We have some great presentations lined up for this year's International PPB/DICER1/OTST Registries Family Meeting. We are still finalizing details and more talks may be added but check out the attached image for a sneak peek! The finalized agenda will be shared closer to the meeting date.

Register now for the International PPB/DICER1/OTST Registries 2024 Family Meeting on Thursday, August 1, 2024. The meeting will be a hybrid event with the options to attend in person in St. Paul, Minnesota or virtually via Zoom. Click on this link to register: https://us06web.zoom.us/webinar/register/WN_21x2OOA7TGmORfuzVTb9HQ

Also, if you plan on joining us in person, please RSVP for the outdoor, evening Meet-&-Greet event on August 1 from 5-7pm central time. The International PPB/DICER1/OTST Registries together with the Pine Tree Apple Classic Fund will be hosting this event at Life Time in White Bear Lake, MN. RSVP here: https://forms.office.com/pages/responsepage.aspx?id=OpIKj12BPUaNw4KG0pTxPCZ15_1WQD5Cj-p4rPqGaVJUQUFKQ0lMUktGMDYyMlBWTUE0OTNGV1Y4RiQlQCN0PWcu

Sertoli-Leydig cell tumors (SLCT) and gynandroblastomas are rare s*x cord stromal tumors, representing

Save the date! The 2024 International PPB/DICER1/OTST Registries Family Meeting will be held on Thursday, August 1, 2024 starting at 9:00am central time. Following the hybrid meeting, there will be a further opportunity to connect at an evening Meet-&-Greet with food, kids’ arts and crafts, and we hope, some Minnesota summer sunshine!

Please watch your email and our social media pages for more information on how to register in the upcoming weeks. We look forward to connecting with you!

We are grateful for all past and present participants, friends, and families dedicated to improving outcomes and learning more about these rare tumors. Over the last few days, we performed some random acts of kindness in honor of

Today we celebrate National Cancer Survivor’s Day while also holding a spot in our hearts for those who lost their courageously fought battles. Through the contributions of past and current patients and families like you, much has been learned about these rare tumors but there is still much to learn. Together we can find a cure and improve outcomes for those with ovarian and testicular stromal tumors.

As we move into stay tuned for updates on how we are creating a space to bring researchers, clinicians, genetic counselors, and other healthcare professionals together to discuss the latest updates in OTST and DICER1-related research.

April is testicular cancer awareness month. The International Ovarian and Testicular Stromal Tumor (OTST) Registry’s mission is to learn more about cause and best treatments for ovarian and testicular stromal tumors. Thank you to all of the patients and families who have shared their journey with us in support of this mission as we learn more together.

We invite you to join us in-person or virtually for the 2024 International PPB/DICER1/OTST Registries Family Meeting on Thursday, August 1st. Registration for this event is now open. Follow the link to register: https://us06web.zoom.us/webinar/register/WN_21x2OOA7TGmORfuzVTb9HQ #/registration

Did you receive a copy of our PPB/DICER1/OTST Registries Newsletter? It was filled with recent research advancements, upcoming opportunities to connect, and the 2024 Family Meeting registration link! Please reach out to [email protected] if you have any questions or comments regarding the latest edition or if you would like to be sent a copy.

Happy National Doctor’s Day! Thank you to all of the amazing providers whose intentional care and dedication go into supporting patients and families with rare cancers, and a special thank you to our Registry physicians, Dr. Yoav Messinger and Dr. Kris Ann Schultz, for the care you provide to each of your patients as well as all of your continued efforts in moving this research forward.

March is both Social Work Month and Child Life Month! We want to extend a huge thank you and share our appreciation for the incredible work these Child Life and Social Work professionals do for patients and families every day.

Today is Rare Disease Day! We’ve enjoyed sharing some research highlights with you over these past couple weeks. To learn more about the Registry, our research, and upcoming opportunities to connect, read our latest newsletter issue – just released today. If you aren’t already on our mailing list, you can request to be added by reaching us at [email protected].

Most DICER1 variants are found in the coding region of the DICER1 gene, however, variants in the intronic (noncoding) region of DICER1 can also result in risk for tumor development. These are variants that were likely missed by prior testing. Thank you to the kids and adults in the PPB/DICER1 Registry and their families who made this important development possible! Read more about this research here:

Intronic Germline DICER1 Variants in Patients With Sertoli-Leydig Cell Tumor Sequencing introns in cancer predisposition genes detects novel loss-of-function variants.Germline pathogenic loss-of-function (pLOF) variants in DICER1 are associated with a predisposition for a variety of solid neoplasms, including pleuropulmonary blastoma ...

Thanks to 133 children with Type II or III PPB and their families, we now have a much better understanding of the role of PET imaging and bone scans in these rare cancers. This report discusses the utility and limitations of PET imaging for detection of PPB in the lung and other sites at diagnosis and recurrence.

Assessing the Role of Positron Emission Tomography and Bone Scintigraphy in Imaging of Pleuropulmonary Blastoma (PPB): A Report from the International PPB/DICER1 Registry Pleuropulmonary blastoma (PPB) is the most common primary lung neoplasm of infancy and early childhood. Given the rarity of PPB, the role of positron emission tomography (PET) and bone scintigraphy (bone scans) in diagnostic evaluation and surveillance ...

Type II and Type III PPB are aggressive sarcomas diagnosed primarily in young children under the age of 7, treated with surgery, intensive chemotherapy and sometimes radiation. This report from the Registry, made possible by 314 children and families that have shared their journeys, analyses prognostic factors and outcomes in children with type II and type III PPB.

Outcomes for Children With Type II and Type III Pleuropulmonary Blastoma Following Chemotherapy: A Report From the International PPB/DICER1 Registry Pleuropulmonary blastoma (PPB) is the most common primary lung neoplasm of infancy and early childhood. Type II and type III PPB have historically been associated with a poor prognosis.Patients with known or suspected PPB were enrolled in the International ...

PPB Types I & Ir are both purely cystic lesions but type I PPB has a higher likelihood of progressing to type II or III PPB. In 2023, the Registry published results from the largest ever cohort of children with type I and Ir PPB. Thanks to your collective efforts, there is now much more information about risk factors and how to treat these rare tumors. Learn more about Types I and Ir PPB here:

Type I and Ir Pleuropulmonary blastoma (PPB): A Report from the International PPB/DICER1 Registry Pleuropulmonary blastoma (PPB) is the most common lung cancer of infancy and early childhood. Type I PPB is a purely cystic lesion with a microscopic population of primitive small cells with or without rhabdomyoblastic features which may progress to type ...

As Rare Disease Day approaches, we want to thank all the participants in our PPB/DICER1 and OTST Registries and their families for their ongoing collaboration and support. Over the next couple weeks, we plan to highlight some of the many research advances made possible by your collaboration!

New DICER1 research published in Journal of Clinical Oncology: Precision Oncology
In partnership with kids and families and our collaborators, we've been investigating situations in which the clinical picture suggested DICER1 variation but no changes were identified by routine testing. By analyzing a region within the intronic section of the DICER1 gene, we were able to identify these "hidden" variants, allowing for testing and surveillance in these patients and their relatives. These new research findings may bring clarity to patients and families with negative DICER1 testing despite a history suggestive of a DICER1 variant.

Intronic Germline DICER1 Variants in Patients With Sertoli-Leydig Cell Tumor Sequencing introns in cancer predisposition genes detects novel loss-of-function variants.

Today, we bring awareness around . As National Organization for Rare Disorders, Inc. (NORD) so wonderfully puts it, “Alone we are rare. Together we are strong.”

Childhood Cancer Awareness Month, a time for bringing extra recognition to all of the young heroes in our lives.