Pediatric Adrenal Carcinoma 

  • Author: Lawrence C Wolfe, MD; Chief Editor: Robert J Arceci, MD, PhD   more...
 
Updated: Nov 22, 2011
 

Background

Adrenocortical carcinoma is a rare tumor in the pediatric population (0-21 y). In a study of the incidence of functioning adrenal tumors in patients aged 4-20 years, 59 were identified at a single referral institution over a period of years. Only 2 of these patients had adrenocortical carcinoma. The authors underreported the overall incidence of adrenocortical carcinoma because 20-40% of patients present with a palpable mass and no symptoms of adrenal hormone hypersecretion. Because of the relative rarity of these tumors, little is known about their cause and the influence of genetic factors, although adrenocortical carcinomas are associated with numerous constitutional syndromes, including Li-Fraumeni syndrome, Beckwith-Wiedemann syndrome, Carney complex, multiple endocrine neoplasia 1, and hemihypertrophy syndrome.

Data have suggested an increased incidence in female individuals, especially at age 0-3 years or after age 13 years. No racial predominance for this diagnosis has been established; however, in southern Brazil, the incidence of adrenal tumors is 10-15 times that of the general population. This incidence is associated with a mutation in the P53 gene. Based on data from the International Pediatric Adrenocortical Tumor Registry, the median age when children develop adrenal carcinomas is 3.2 years; 60% are younger than 4 years, and 14% are older than 13 years.[1]

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Epidemiology

Mortality/Morbidity

The prognosis of patients with adrenocortical carcinoma is always guarded. Cures were reported in patients who underwent complete removal of a small (< 9 cm, < 200 g) encapsulated tumors. Reports of remission of metastatic disease are only anecdotal. Aggressive surgical and medical treatments have prolonged mean survival times by approximately 18 months and, occasionally, by longer than 48 months. Studies of aggressive surgical and early adjuvant therapy are limited by the rarity of this illness in childhood.

Race

No racial predilection has been identified.

Sex

As data accumulate, especially in international registries, the incidence of adrenal tumors in female individuals has risen higher than previously thought.

Age

Adrenocortical carcinoma is a rare tumor among individuals aged 0-21 years.

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Contributor Information and Disclosures
Author

Lawrence C Wolfe, MD  Senior Associate in Pediatric Hematology/Oncology, Schneider Children's Hospital

Lawrence C Wolfe, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Pediatrics, American Association of Blood Banks, American Society of Hematology, Children's Oncology Group, and Eastern Society for Pediatric Research

Disclosure: Nothing to disclose.

Specialty Editor Board

Samuel Gross, MD  Professor Emeritus, Department of Pediatrics, University of Florida; Clinical Professor, Department of Pediatrics, University of North Carolina; Adjunct Professor, Department of Pediatrics, Duke University

Samuel Gross, MD is a member of the following medical societies: American Association for Cancer Research, American Society for Blood and Marrow Transplantation, American Society of Clinical Oncology, American Society of Hematology, and Society for Pediatric Research

Disclosure: Nothing to disclose.

Mary L Windle, PharmD  Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Timothy P Cripe, MD, PhD  Professor of Pediatrics, Division of Hematology/Oncology, Cincinnati Children's Hospital Medical Center; Clinical Director, Musculoskeletal Tumor Program, Co-Medical Director, Office for Clinical and Translational Research, Cincinnati Children's Hospital Medical Center; Director of Pilot and Collaborative Clinical and Translational Studies Core, Center for Clinical and Translational Science and Training, University of Cincinnati College of Medicine

Timothy P Cripe, MD, PhD is a member of the following medical societies: American Association for the Advancement of Science, American Pediatric Society, American Society of Hematology, American Society of Pediatric Hematology/Oncology, and Society for Pediatric Research

Disclosure: Nothing to disclose.

Mary E Cataletto, MD  Director of Children's Sleep Services, Winthrop Sleep Disorders Center, Mineola, NY; Professor of Clinical Pediatrics, State University of New York at Stony Brook, Stony Brook, NY

Mary E Cataletto, MD is a member of the following medical societies: American Academy of Pediatrics and American College of Chest Physicians

Disclosure: Shering Plough Pharmaceuticals Honoraria Consulting

Chief Editor

Robert J Arceci, MD, PhD  King Fahd Professor of Pediatric Oncology, Professor of Pediatrics, Oncology and the Cellular and Molecular Medicine Graduate Program, Kimmel Comprehensive Cancer Center at Johns Hopkins University School of Medicine

Robert J Arceci, MD, PhD is a member of the following medical societies: American Association for Cancer Research, American Association for the Advancement of Science, American Pediatric Society, American Society of Hematology, and American Society of Pediatric Hematology/Oncology

Disclosure: Nothing to disclose.

References

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