Study Results
The study team has not published outcome measurements, participant flow, or safety data for this trial yet. Check back later for updates.
Basic Information
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RECRUITING
9999 participants
OBSERVATIONAL
2001-10-01
2040-12-31
Brief Summary
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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.
Detailed Description
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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.
Conditions
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Study Design
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OTHER
PROSPECTIVE
Study Groups
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Participants
Any participant who meets eligibility criteria and consents to participate in the trial.
No interventions assigned to this group
Eligibility Criteria
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Inclusion Criteria
* Suspected or confirmed diagnosis of adrenocortical tumor (adenoma, carcinoma or undefined histology).
* Signed informed consent
* Any age
* Diagnosis of malignant tumor
* Signed informed consent
* Biological parent of Stratum A participant
21 Years
ALL
No
Sponsors
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St. Jude Children's Research Hospital
OTHER
Responsible Party
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Principal Investigators
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Raul C Ribeiro, MD
Role: PRINCIPAL_INVESTIGATOR
St. Jude Children's Research Hospital
Locations
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Stanford University
Stanford, California, United States
All Children's Hospital/St. Petersburg Hospital
St. Petersburg, Florida, United States
The Children's Medical Center
Dayton, Ohio, United States
St. Jude Children's Research Hospital
Memphis, Tennessee, United States
Cook Children's Medical Center
Fort Worth, Texas, United States
Countries
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Central Contacts
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Facility Contacts
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Mukund Dole, MD
Role: primary
Jenny Dillon, RN, CCRP
Role: backup
Raul C Ribeiro, MD
Role: primary
References
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Pinto EM, Maxwell KN, Halalsheh H, Phillips A, Powers J, MacFarland S, Walsh MF, Breen K, Formiga MN, Kriwacki R, Nichols KE, Mostafavi R, Wang J, Clay MR, Rodriguez-Galindo C, Ribeiro RC, Zambetti GP. Clinical and Functional Significance of TP53 Exon 4-Intron 4 Splice Junction Variants. Mol Cancer Res. 2022 Feb;20(2):207-216. doi: 10.1158/1541-7786.MCR-21-0583. Epub 2021 Oct 21.
Related Links
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St. Jude Children's Research Hospital
Clinical Trials Open at St. Jude
Other Identifiers
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IPACTR
Identifier Type: -
Identifier Source: org_study_id