International Pediatric Adrenocortical Tumor Registry

This study is currently recruiting participants. (see Contacts and Locations)
Verified January 2015 by St. Jude Children's Research Hospital
Information provided by (Responsible Party):
St. Jude Children's Research Hospital Identifier:
First received: June 13, 2008
Last updated: January 7, 2015
Last verified: January 2015

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.

Adrenocortical Tumor

Study Type: Observational
Study Design: Time Perspective: Prospective
Official Title: International Pediatric Adrenocortical Tumor Registry

Resource links provided by NLM:

Further study details as provided by St. Jude Children's Research Hospital:

Primary Outcome Measures:
  • 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: Indefinite ] [ Designated as safety issue: No ]

Biospecimen Retention:   Samples With DNA

Peripheral blood sample with DNA and RNA; Tumor Tissue (of any histology); Adrenocortical tumors;

Estimated Enrollment: 9999
Study Start Date: May 2001
Estimated Study Completion Date: December 2040
Estimated Primary Completion Date: December 2040 (Final data collection date for primary outcome measure)
Any participant who meets eligibility criteria and consents to participate in the trial.

Detailed Description:

Adrenocortical tumors (ACT) is a rare cancer that forms in the outer layer of the adrenal gland and is 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.


Ages Eligible for Study:   up to 21 Years
Genders Eligible for Study:   Both
Accepts Healthy Volunteers:   No
Sampling Method:   Non-Probability Sample
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.



  • Stratum A: participant suspected or confirmed diagnosis of adrenocortical tumor 9ACT)
  • 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

Exclusion Criteria - Stratum R (relative):

  • Any age
  • Diagnosis of malignant tumor
  • Signed informed consent

Inclusion Criteria - Stratum P (parent):

  • Biological parent of Stratum A participant
  Contacts and Locations
Choosing to participate in a study is an important personal decision. Talk with your doctor and family members or friends about deciding to join a study. To learn more about this study, you or your doctor may contact the study research staff using the Contacts provided below. For general information, see Learn About Clinical Studies.

Please refer to this study by its identifier: NCT00700414

Contact: Raul C Ribeiro, MD 1-866-278-5833

United States, California
Stanford University Completed
Stanford, California, United States, 94305
United States, Florida
All Children's Hospital/St. Petersburg Hospital Recruiting
St. Petersburg, Florida, United States, 33701
Contact: Gregory Hale, MD         
Contact: Mike Gates, RN         
Principal Investigator: Gregory Hale, MD         
United States, Ohio
The Children's Medical Center Recruiting
Dayton, Ohio, United States, 45404
Contact: Mukund Dole, MD         
Contact: Jenny Dillon, RN, CCRP         
Principal Investigator: Mukund Dole, MD         
United States, Tennessee
St. Jude Children's Research Hospital Recruiting
Memphis, Tennessee, United States, 38105
Contact: Raul C Ribeiro, MD    866-278-5833   
Principal Investigator: Raul C Ribeiro, MD         
United States, Texas
Cook Children's Medical Center Recruiting
Fort Worth, Texas, United States, 76104
Contact: Kelly Vallance, MD         
Contact: Kathy Contreras         
Principal Investigator: Kelly Vallance, MD         
Sponsors and Collaborators
St. Jude Children's Research Hospital
Principal Investigator: Raul C Ribeiro, MD St. Jude Children's Research Hospital
  More Information

Additional Information:
No publications provided

Responsible Party: St. Jude Children's Research Hospital Identifier: NCT00700414     History of Changes
Other Study ID Numbers: IPACTR
Study First Received: June 13, 2008
Last Updated: January 7, 2015
Health Authority: United States: Institutional Review Board

Additional relevant MeSH terms:
Adrenal Cortex Neoplasms
Adrenal Cortex Diseases
Adrenal Gland Diseases
Adrenal Gland Neoplasms
Endocrine Gland Neoplasms
Endocrine System Diseases
Neoplasms by Site processed this record on July 05, 2015