International Pediatric Adrenocortical Tumor Registry

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Brief Title

International Pediatric Adrenocortical Tumor Registry

Official Title

International Pediatric Adrenocortical Tumor Registry

Brief Summary

      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.

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


Primary Outcome

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


Adrenocortical Tumor

Study Arms / Comparison Groups

Description:  Any participant who meets eligibility criteria and consents to participate in the trial.


* Includes publications given by the data provider as well as publications identified by National Clinical Trials Identifier (NCT ID) in Medline.

Recruitment Information

Estimated Enrollment


Start Date

October 1, 2001

Completion Date

December 2040

Primary Completion Date

December 2040

Eligibility Criteria


          -  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

          -  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




N/A - 21 Years

Accepts Healthy Volunteers



Raul C Ribeiro, MD, 866-278-5833, [email protected]

Location Countries

United States

Location Countries

United States

Administrative Informations



Organization ID


Responsible Party


Study Sponsor

St. Jude Children's Research Hospital

Study Sponsor

Raul C Ribeiro, MD, Principal Investigator, St. Jude Children's Research Hospital

Verification Date

August 2021