RecruitingNCT03382158

International PPB/DICER1 Registry

International Pleuropulmonary Blastoma/DICER1 Registry (for PPB, DICER1 and Associated Conditions)

Sponsor

Children's Hospitals and Clinics of Minnesota

Enrollment

3,400 participants

Start Date

Dec 6, 2016

Study Type

OBSERVATIONAL

Conditions

DICER1 Syndrome Neuroblastoma Thyroid Carcinoma Pleuropulmonary Blastoma Wilms Tumor Sertoli-Leydig Cell Tumor Cystic Nephroma Pineoblastoma Renal Sarcoma Nodular Hyperplasia of Thyroid Nasal Chondromesenchymal Hamartoma Ciliary Body Medulloepithelioma Pituitary Cancer Embryonal Rhabdomyosarcoma Ovarian Sarcoma Gynandroblastoma Embryonal Rhabdomyosarcoma of Vagina (Diagnosis)Embryonal Rhabdomyosarcoma of Uterus (Diagnosis)Embryonal Rhabdomyosarcoma of Cervix

Summary

Pleuropulmonary blastoma (PPB) is a rare malignant neoplasm of the lung presenting in early childhood. Type I PPB is a purely cystic lesion, Type II is a partially cystic, partially solid tumor, Type III is a completely solid tumor. Treatment of children with PPB is at the discretion of the treating institution. This study builds off of the 2009 study and will also seek to enroll individuals with DICER1-associated conditions, some of whom may present only with the DICER1 gene mutation, which will help the Registry understand how these tumors and conditions develop, their clinical course and the most effective treatments.

Eligibility

Min Age: 0 MinutesMax Age: 100 Years

Plain Language Summary

Simplified for easier understanding

This trial is an international registry collecting clinical and genetic data from patients with a rare childhood lung tumor called pleuropulmonary blastoma (PPB) and related rare tumors linked to a gene called DICER1, to better understand these conditions and improve future treatments. **You may be eligible if...** - You have a known or suspected pleuropulmonary blastoma or related chest tumor - You have a known or suspected sex-cord stromal tumor (such as Sertoli-Leydig cell tumor) - You have another known or suspected DICER1-related condition (such as kidney cysts, ovarian sarcoma, pineoblastoma, or pituitary blastoma) - You carry a DICER1 gene mutation, even if you have not developed a related tumor - Informed consent has been provided by the patient or their parent or guardian **You may NOT be eligible if...** - You are unwilling or unable to provide informed consent - You do not have a DICER1-related condition or gene mutation Talk to your doctor to see if this trial is right for you.

This summary was AI-generated to explain the trial in plain language. It is not medical advice. Always discuss eligibility with your doctor before enrolling in a clinical trial.

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