International Registry for PPB and DICER1-Related Conditions

This study is an international registry for people with Pleuropulmonary Blastoma (PPB), a rare lung cancer in children, and other conditions linked to a gene change called DICER1. The registry aims to understand how these tumors and conditions develop, how they progress, and what treatments work best. You might be able to join if you have known or suspected PPB, certain sex-cord stromal tumors (like Sertoli-Leydig cell tumor), or other conditions related to DICER1, such as cystic nephroma or Wilms tumor. This study is observing participants from birth to 100 years old. The main goal is to track how long people live without certain events (like the cancer returning) over 7 years.

Study design
This is an observational study planning to include 3400 participants. It is not testing a specific intervention but rather collecting information.
What's involved
Not specified in the trial record.
Compensation
Not stated in the trial record.
Follow-up
Participants' event-free survival will be measured at 7 years.

AI-generated from the public study record. Only the study team can confirm whether you're eligible — confirm details with them before making decisions.

NCT03382158

International PPB/DICER1 Registry

Recruiting
Not specifiedAges 0+Observational
Children's Hospitals and Clinics of Minnesota
~3,400 participants
Updated 2025-01-30 on ClinicalTrials.gov

At a glance

Recruiting sites
1 of 1 listed site is recruiting right now
RecruitingSuspended, closed, or not yet open
What they're measuring
Event-free survival
Measured over 7 years
Pleuropulmonary Blastoma
Sertoli-Leydig Cell Tumor
DICER1 Syndrome
Cystic Nephroma
Wilms Tumor
Pineoblastoma
Renal Sarcoma
Nodular Hyperplasia of Thyroid
Nasal Chondromesenchymal Hamartoma
Ciliary Body Medulloepithelioma
Neuroblastoma
Pituitary Cancer
Embryonal Rhabdomyosarcoma
Ovarian Sarcoma
Gynandroblastoma
Thyroid Carcinoma
Embryonal Rhabdomyosarcoma of Vagina (Diagnosis)
Embryonal Rhabdomyosarcoma of Uterus (Diagnosis)
Embryonal Rhabdomyosarcoma of Cervix
1 sites across 1 states
Minnesota1
  • Kris Ann P Schultz, MD · PRINCIPAL_INVESTIGATOR · Children's Minnesota

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  • Event-free survival7 years

    The primary endpoint for statistical analysis will be time from start treatment to an event, defined as the occurrence of progression or recurrence of PPB, occurrence of a second malignant neoplasm, or death from any cause that is at least possibly related to the original disease or treatment.