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|NCT03382158 : International PPB Registry for PPB, DICER1 and Associated Conditions|
|Ages||Min: N/A Max: N/A|
1. Pathology Diagnosis: Individuals with newly-diagnosed PPB Types I, II or III.
Diagnosis is made by the local pathologist.
All cases must be submitted for central pathology review. Only centrally-reviewed
cases confirmed as PPB will be analyzed prospectively.
Cases in which the initial diagnosis is "suggestive" or "supportive" of PPB, but not
diagnostic, and in which later resection specimens, including resections following
chemotherapy, confirm a PPB diagnosis will be included. Patients diagnosed by fine
needle aspiration biopsy will be included only if a later resection specimen,
including resection(s) following chemotherapy, is diagnostic of PPB.
2. Prior Therapy: PPB Type I: All patients are eligible and will be followed in the
3. PPB Types II or III: Newly-diagnosed Types II and III PPB patients will be included in
the Treatment and Biology Registry.
4. Prior corticosteroid therapy is allowed.
5. Types II and III PPB patients with PRIOR Type I PPB diagnosis
6. Individuals with DICER1-related conditions including PPB, Sertoli-Leydig cell tumor,
gynandroblastoma, cystic nephroma, renal sarcoma and others.
7. Individuals with the DICER1 gene mutation regardless of whether they have a known
8. Informed consent by patient/ or parent/guardian (also, where appropriate: assent and
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