- ICH GCP
- US Clinical Trials Registry
- Clinical Trial NCT00700414
International Pediatric Adrenocortical Tumor Registry
This study aims to collect demographic and medical information including detailed family history of cancer of children and adolescents with adrenocortical tumors in order to learn more about the clinical and epidemiological aspects, treatment modalities, and outcome of patients with this rare disease, worldwide.
In addition, investigators at St. Jude Children's Research Hospital (SJCRH) plan to perform molecular studies of tumor cells aimed to clarify the role of the TP53 gene and other genetic pathways in these tumors. They aim to obtain relevant biological material from participants with adrenocortical tumor (ACT), their biological parents, and relatives for determination of the TP53 germline status, molecular studies of the TP53 gene, and other molecular pathways.
Study Overview
Status
Conditions
Detailed Description
Adrenocortical tumors (ACT) are rare cancer types that form in the outer layer of the adrenal gland and are very uncommon in children and teenagers. There is variation in pediatric ACT incidence worldwide. In the United States, only about 25 new cases of ACT per million per year, making this a very rare tumor. However, in southern Brazil, the annual incidence of ACT is 15 times that seen in the United States accounting for 3.4-4.2 per million per year.
Molecular studies have revealed that the majority of children with ACT, particularly those younger than 4 years of age, have constitutional TP53 mutations and/or imprinting defects at chromosome 11p as observed in Beckwith Wiedemann syndrome (BWS) patients. Some mutations, as exemplified by the R337H TP53 germline mutation, in which the function of the mutant protein is relatively preserved, the history of cancer in the carriers and their families is relatively unremarkable. In other cases, the TP53 mutated gene encodes a functionally-impaired protein that predicts for a pervasive history of familial cancer (Li-Fraumeni syndrome). Therefore, these observations have implications for genetic counseling of families with childhood ACT and underscore the importance of genotype-phenotype correlations in familial cancer syndromes.
The creation of a rare tumor registry provides a mechanism to collect information that cannot be gathered in a single institution. The analysis of the registry data would permit an overview of the clinical, epidemiological, current treatment standards, and survival data of these patients and thus create opportunities for research. It also may facilitate the development of treatment consensus among investigators who register their patients and help to design future studies. Moreover, the combined Children's Oncology Group (COG) and IPACTR studies are expected to provide meaningful insight into the biology of ACT, including clinical phenotype/genotype relationships, treatment outcome and long-term follow-up data in subjects with this rare tumor. Finally, it would provide data on the long-term consequences of exposure to tumor-secreted androgens (found in more than 80% of the pediatric cases) on children's growth and development.
Study Type
Enrollment (Estimated)
Contacts and Locations
Study Contact
- Name: Raul C Ribeiro, MD
- Phone Number: 866-278-5833
- Email: referralinfo@stjude.org
Study Locations
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California
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Stanford, California, United States, 94305
- Completed
- Stanford University
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Florida
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Saint Petersburg, Florida, United States, 33701
- Completed
- All Children's Hospital/St. Petersburg Hospital
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Ohio
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Dayton, Ohio, United States, 45404
- Recruiting
- The Children's Medical Center
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Contact:
- Mukund Dole, MD
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Contact:
- Jenny Dillon, RN, CCRP
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Principal Investigator:
- Mukund Dole, MD
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Tennessee
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Memphis, Tennessee, United States, 38105
- Recruiting
- St. Jude Children's Research Hospital
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Contact:
- Raul C Ribeiro, MD
- Phone Number: 866-278-5833
- Email: referralinfo@stjude.org
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Principal Investigator:
- Raul C Ribeiro, MD
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Texas
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Fort Worth, Texas, United States, 76104
- Completed
- Cook Children's Medical Center
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Participation Criteria
Eligibility Criteria
Ages Eligible for Study
Accepts Healthy Volunteers
Sampling Method
Study Population
Age ≤ 21 years old at diagnosis of adrenocortical tumor.
Relatives of the ACT patients of any age with a diagnosis of malignant tumor.
Description
STRATIFICATION ASSIGNMENT:
- Stratum A: participant suspected or confirmed diagnosis of adrenocortical tumor (ACT)
- Stratum R: relative of participant with ACT and TP53 mutation who has diagnosis of malignancy
- Stratum P: biological parent of participant with ACT
Inclusion Criteria - Stratum A (participant with ACT):
- Age ≤ 21 years old at diagnosis
- Suspected or confirmed diagnosis of adrenocortical tumor (adenoma, carcinoma or undefined histology).
- Signed informed consent
Inclusion Criteria - Stratum R (relative):
- Any age
- Diagnosis of malignant tumor
- Signed informed consent
Inclusion Criteria - Stratum P (parent):
- Biological parent of Stratum A participant
Study Plan
How is the study designed?
Design Details
Cohorts and Interventions
Group / Cohort |
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Participants
Any participant who meets eligibility criteria and consents to participate in the trial.
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What is the study measuring?
Primary Outcome Measures
Outcome Measure |
Time Frame |
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Collect demographic/medical information, detailed family history of cancer of children/adolescents with adrenocortical tumors, learn more about the clinical and epidemiological aspects, treatment modalities, and outcome of patients
Time Frame: Annually from diagnosis until no longer being followed, defined as up to 5 years from the date of last follow-up
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Annually from diagnosis until no longer being followed, defined as up to 5 years from the date of last follow-up
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Collaborators and Investigators
Investigators
- Principal Investigator: Raul C Ribeiro, MD, St. Jude Children's Research Hospital
Publications and helpful links
Study record dates
Study Major Dates
Study Start (Actual)
Primary Completion (Estimated)
Study Completion (Estimated)
Study Registration Dates
First Submitted
First Submitted That Met QC Criteria
First Posted (Estimated)
Study Record Updates
Last Update Posted (Actual)
Last Update Submitted That Met QC Criteria
Last Verified
More Information
Terms related to this study
Additional Relevant MeSH Terms
Other Study ID Numbers
- IPACTR
Drug and device information, study documents
Studies a U.S. FDA-regulated drug product
Studies a U.S. FDA-regulated device product
This information was retrieved directly from the website clinicaltrials.gov without any changes. If you have any requests to change, remove or update your study details, please contact register@clinicaltrials.gov. As soon as a change is implemented on clinicaltrials.gov, this will be updated automatically on our website as well.
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