Accelerate research

With research comes progress. The Registry is leading research initiatives that can be the motivation for transforming the way PPB/DICER1 is detected and treated.

Our four strategic pillars

The mission of The International PPB/DICER1 Registry is to improve outcomes for children and adults with PPB, and other DICER1-related cancers through four strategic pillars:

Define optimal therapy for PPB, Sertoli-Leydig cell tumor and other DICER1-related cancers

Validate testing and surveillance guidelines for children and adults with germline DICER1 variations

Develop new ways to diagnose and follow children with DICER1-related cancers

Discover new therapies for DICER1-related cancers

How you can help find a cure

We can find a cure for PPB, SLCT and other DICER1-related cancers if we all work together. This includes clinicians, pathologists, geneticists, radiologists, laboratory scientists, coordinators, philanthropic volunteers and donors—and most of all, patients and families. Each research advancement in this area has been made possible by the generous sharing of clinical information and tissue and blood samples from individuals and/or their family members.

To contribute clinical information and/or blood/tumor samples, please consider enrollment in The International PPB/DICER1 Registry.

Eligibility criteria for the Registry:

Individuals of any age, from any state, or country
Known or suspected pleuropulmonary blastoma (PPB)
Known or suspected DICER1-related ovarian tumor
Known or suspected other DICER1-related tumor
Known DICER1 pathogenic variation (also called DICER1 mutation)

Additional information to consider

Information for clinicians

Clinicians are invited to connect with the Registry regarding participation of individual patients, potential collaborations and other questions.

Please see separate sections of the website regarding individual tumor types and information regarding DICER1.

Information for pathologists

Pathologists are invited to connect with the Registry regarding free central pathology review (no charge to institution or patient/family).

Sending slides for central pathology review
Sending blocks and/or scrolls
Sending fresh tissue at time of surgery
Sending frozen tissue

View the specimen transmittal form.

How tissue samples are used

Left over tumor tissue (tumor tissue removed for a clinical reason but not needed for clinical care) is studied for several purposes:

To confirm the diagnosis:
This process, called central review, helps ensure that study results truly represent the tumor that is being studied. This central review is performed through review of slides. Please note that only the slides that are sent can be reviewed, thus if there is a component of the tumor not present on the slides that are sent, that would not be reflected by the review report.
To look for potential targets:
This is to determine if there are any genetic alterations in the tumor that may now or in the future be targetable with certain medications. Analysis for potential targets may be done with scrolls or blocks but frozen tissue is preferred.
To determine impact of certain medications or substances on tumor tissue:
In certain circumstances, fresh tissue (unpreserved) tissue may be available at the time of surgery. This tissue is critical for cutting edge research and may be used in preclinical models to determine sensitivity of some tumors to certain chemotherapy medications. This is one of the reasons that understanding the diagnosis preoperatively is so important; then procedures can be put in place to allow fresh tissue to be sent during or shortly after surgery.

Health professional forms

We encourage all treating physicians and families to consider participating in the Registry to allow sharing of clinical data and tumor tissue.

How to enroll in the registry

We offer free central reviews by Registry pathologists to review/confirm the diagnosis of PPB and all other DICER1 disease states. Please fill out the form(s) and email them to [email protected].

Specimen transmittal form

Radiologists, please fill out the form(s) here and email them to [email protected]

Consultation form