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IPACTR: International Pediatric Adrenocortical Tumor Registry


Tissue Bank

Diseases Treated:

Adrenocortical tumors (carcinoma and adenoma)

Eligibility Overview:

  • Diagnosis of an adrenocortical tumor (ACT)
  • 21 years of age or younger at diagnosis
  • Relatives of patients with ACT
  1. Brief Summary

    Adrenocortical tumor (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, the incidence is 0.3 cases per million people per year or about 25-35 new cases of ACT per year, making this a very rare tumor. However, in southern Brazil, the annual incidence of ACT is 10-15 times that seen in the United States accounting for 3.4 to 4.2 per million per year.

    The creation of a tumor registry provides a mechanism to collect information for this rare disease that cannot be gathered in a single institution. 

    Primary Objectives

    The analysis of the registry data:

    • Will permit an overview of the clinical, epidemiological, current treatment standards, and survival data of these patients and thus create opportunities for research
    • May facilitate the development of treatment consensus among investigators who register their patients and help to design future studies
    • Will allow the combined Children’s Oncology Group (COG) and IPACTR studies to provide meaningful insight into the biology of ACT, including clinical phenotype/genotype relationships and treatment outcome and long-term follow-up data in subjects with this rare tumor
    • Will 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 Outline

    This study aims to collect demographic and medical information including family history of cancer of children and adolescents with adrenocortical tumors (ACT) 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 plan to obtain blood samples from participants with ACT, their biological parents, and relatives for determination of the TP53 germline status. Finally they aim to perform molecular studies of tumor cells to clarify the role of the TP53 gene and other genetic/molecular pathways in these tumors. 

    Study Arms

    • 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

    Eligibility Criteria

    Inclusion Criteria - Stratum A (participant with ACT):
    • 21 years old or younger at diagnosis
    • Suspected or confirmed diagnosis of adrenocortical tumor (adenoma, carcinoma or undefined histology)
    Inclusion Criteria - Stratum R (relative):
    • Any age
    • Diagnosis of malignant tumor
    Inclusion Criteria - Stratum P (parent):
    • Biological parent of Stratum A participant

    Study Design

    Time Perspective: Prospective

    Study Sites

    United States

    St. Jude Children’s Research Hospital
    Memphis, Tennessee

    All Children's Hospital/St. Petersburg Hospital
    St. Petersburg, Florida

    The Children’s Medical Center
    Dayton, Ohio

    Cook Children’s Medical Center
    Fort Worth, Texas

  2. About this observational study

    Adrenocortical tumor (ACT) is a very rare and fast-growing cancer of the adrenal glands, located on the top of both kidneys. The tumor is seen in only about 0.2 percent of all childhood cancers. It affects more girls than boys and most commonly develops in children between ages one and four years.

    In the International Pediatric Adrenocortical Tumor Registry (IPACTR) study, researchers at St. Jude Children’s Research Hospital and at institutions around the world are working together to learn more about the clinical and genetics of adrenocortical tumor. This information may help researchers learn better ways of treating children with these rare tumors.

    Purpose of this observational study

    The main goal of IPACTR is to collect relevant clinical and biological data, including blood and tissue samples, to help researchers better understand the following:

    • Common signs and symptoms of ACT
    • The length of time between the first signs and symptoms and diagnosis of ACT
    • How genetic conditions such as Li-Fraumeni and Beckwith-Wiedemann syndrome make a person more likely to develop ACT
    • Family history of cancer in relatives of children with ACT
    • How children with ACT in different parts of the world are treated
    • How the genetic makeup affects survival in children and relatives with ACT

    Eligibility overview

    • Diagnosis of an adrenocortical tumor (ACT)
    • 21 years of age or younger at diagnosis
    • Relatives of patients with ACT
  3. IPACTR Quick View
    Sponsor St. Jude Children’s Research Hospital identifier NCT00700414
    Trial start date May 2001
    Estimated enrollment Not limited
    Study type Non-therapeutic/Observational

    Adrenocortical tumor (ACT)

    Ages 21 years or younger
    Principal investigator Raul C. Ribeiro, MD
    Study site St. Jude Children’s Research Hospital
    For a consultation or to discuss IPACTR St. Jude Physician/Patient Referral Office

St. Jude Children’s Research Hospital
262 Danny Thomas Place
Memphis, TN 38105  USA
Voice: 1-888-226-4343 or 901-595-4055
24-Hour Emergency Access Pager: 1-800-349-4334

Contact IPACTR

Call: 866-278-5833
Fax: 901-521-9005
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The above information is intended to provide only a basic description about a research protocol that may be currently active at St. Jude. The details made available here may not be the most up-to-date information on protocols used by St. Jude. To receive full details about a protocol and its status and or use at St. Jude, a physician must contact St. Jude directly.