eMedicine Specialties > Pediatrics: General Medicine > Oncology

Adrenal Carcinoma

Author: Lawrence C Wolfe, MD, Professor, Department of Pediatrics, Tufts University School of Medicine; Chief of Transfusion Service, Chief, Division of Pediatric Hematology/Oncology, New England Medical Center, Floating Hospital for Infants and Children
Contributor Information and Disclosures

Updated: Nov 26, 2008

Introduction

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

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.

Clinical

History

  • Children with adrenal tumors often present with a history of adrenocortical hormone production. Virilization due to increased androgen production is most common, whereas cushingoid features are relatively uncommon.
  • In most cases, the patient’s history includes elements of adrenocortical hormone production and a palpable mass. The presence of both findings raises the likelihood of adrenocortical carcinoma because most functioning adrenal tumors with a palpable mass are carcinomas rather than adenomas.
  • Approximately 20-40% of patients with adrenocortical carcinoma present without any history of adrenocortical hormone overproduction.

Physical

  • Physical findings almost always include a palpable mass in the abdomen that usually involves the center of the abdomen rather than the flanks. The mass is hard and nonmovable.
  • Approximately 50-80% of children with adrenocortical carcinoma present with virilization.
    • Findings in males include premature puberty with enlargement of the penis and scrotum, pubic hair, acne, and deepening voice.
    • Findings in females include premature appearance of pubic and axillary hair, clitoral hypertrophy, acne, deepening voice, premature increase in growth velocity, lack of appropriate breast development, and lack of menarche.
  • Signs of Cushing syndrome are present in about 10% of affected children. These signs include a round face, a double chin, buffalo-hump fat distribution, generalized obesity, failure of growth velocity, and hypertension.
  • In rare cases, feminization may occur.
    • Findings in male patients include gynecomastia and hypertension.
    • Findings in female patients include precocious sexual development and hypertension.

Causes

  • When patients present with adrenocortical hormone overproduction, the differential diagnosis usually includes carcinoma, adenoma, and hypothalamic pituitary error.
  • The patient's history and physical findings often indicate functioning or nonfunctioning neuroblastoma, especially in young children.
  • For patients who present with only a palpable mass, the differential diagnoses vary with age, and the range is broad.
  • In children younger than 5 years, neuroblastoma and Wilms tumor are the most likely malignant diagnoses. In older patients, lymphoma, germ cell tumors, sarcoma, undifferentiated tumors, and neuroblastoma are possible.

More on Adrenal Carcinoma

Overview: Adrenal Carcinoma
Differential Diagnoses & Workup: Adrenal Carcinoma
Treatment & Medication: Adrenal Carcinoma
Follow-up: Adrenal Carcinoma
References
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References

  1. Michalkiewicz E, Sandrini R, Figueiredo B, et al. Clinical and outcome characteristics of children with adrenocortical tumors: a report from the International Pediatric Adrenocortical Tumor Registry. J Clin Oncol. Mar 1 2004;22(5):838-45. [Medline].

  2. Hermsen IG, Gelderblom H, Kievit J, Romijn JA, Haak HR. Extremely long survival in six patients despite recurrent and metastatic adrenal carcinoma. Eur J Endocrinol. Jun 2008;158(6):911-9. [Medline].

  3. Hah JO. Intensive chemotherapy with autologous PBSCT for advanced adrenocortical carcinoma in a child. J Pediatr Hematol Oncol. Apr 2008;30(4):332-4. [Medline].

  4. Schulick RD, Brennan MF. Long-term survival after complete resection and repeat resection in patients with adrenocortical carcinoma. Ann Surg Oncol. Dec 1999;6(8):719-26. [Medline].

  5. Tritos NA, Cushing GW, Heatley G, Libertino JA. Clinical features and prognostic factors associated with adrenocortical carcinoma: Lahey Clinic Medical Center experience. Am Surg. Jan 2000;66(1):73-9. [Medline].

  6. Berruti A, Terzolo M, Sperone P, et al. Etoposide, doxorubicin and cisplatin plus mitotane in the treatment of advanced adrenocortical carcinoma: a large prospective phase II trial. Endocr Relat Cancer. Sep 2005;12(3):657-66. [Medline].

  7. Bonfig W, Bittmann I, Bechtold S, et al. Virilising adrenocortical tumours in children. Eur J Pediatr. Sep 2003;162(9):623-8. [Medline].

  8. Bukowski RM, Wolfe M, Levine HS, et al. Phase II trial of mitotane and cisplatin in patients with adrenal carcinoma: a Southwest Oncology Group study. J Clin Oncol. Jan 1993;11(1):161-5. [Medline].

  9. Haak HR, Hermans J, van de Velde CJ, et al. Optimal treatment of adrenocortical carcinoma with mitotane: results in a consecutive series of 96 patients. Br J Cancer. May 1994;69(5):947-51. [Medline].

  10. Hovi L, Wikstrom S, Vettenranta K, et al. Adrenocortical carcinoma in children: a role for etoposide and cisplatin adjuvant therapy? Preliminary report. Med Pediatr Oncol. May 2003;40(5):324-6. [Medline].

  11. Lee P, Witchel SS. Disorders of the adrenal gland. In: Burg FD, Polin RA, Ingelfinger JR, et al, eds. Gellis and Kagan's Current Pediatric Therapy. Philadelphia, PA: WB Saunders; 1995:338-41.

  12. Ribeiro J, Ribeiro RC, Fletcher BD. Imaging findings in pediatric adrenocortical carcinoma. Pediatr Radiol. Jan 2000;30(1):45-51. [Medline].

  13. Ribeiro RC, Figueiredo B. Childhood adrenocortical tumours. Eur J Cancer. May 2004;40(8):1117-26. [Medline].

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  15. Sredni ST, Alves VA, Latorre Mdo R, Zerbini MC. Adrenocortical tumours in children and adults: a study of pathological and proliferation features. Pathology. Apr 2003;35(2):130-5. [Medline].

  16. Stewart JN, Flageole H, Kavan P. A surgical approach to adrenocortical tumors in children: the mainstay of treatment. J Pediatr Surg. May 2004;39(5):759-63. [Medline].

  17. Stratakis C, Chrousos G. Endocrine tumors. In: Pizzo PA, Poplack DG, eds. Principles and Practice of Pediatric Oncology. Philadelphia, PA: Lippincott Williams & Wilkins; 1997:947-76.

  18. Zidan J, Shpendler M, Robinson E. Treatment of metastatic adrenal cortical carcinoma with etoposide (VP-16) and cisplatin after failure with o,p'DDD. Clinical case reports. Am J Clin Oncol. Jun 1996;19(3):229-31. [Medline].

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Further Reading

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Keywords

adrenal carcinoma, adrenal cortical carcinoma, adrenocortical carcinoma, adrenal cancer, abdominal mass, adrenal hormone hypersecretion, Li-Fraumeni complex, Cushingoid features, virilization, androgen production, premature puberty, premature pubic hair, acne, tumor, cancer, P53 gene, Beckwith-Wiedemann syndrome, Carney complex, multiple endocrine neoplasia 1, hemihypertrophy syndrome, hypertension, obesity, gynecomastia, precocious sexual development, neuroblastoma, Wilms tumor

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Contributor Information and Disclosures

Author

Lawrence C Wolfe, MD, Professor, Department of Pediatrics, Tufts University School of Medicine; Chief of Transfusion Service, Chief, Division of Pediatric Hematology/Oncology, New England Medical Center, Floating Hospital for Infants and Children
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.

Medical Editor

Samuel Gross, MD, Professor Emeritus, Department of Pediatrics, University of Florida, Clinical Professor, Department of Pediatrics, UNC, 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.

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine
Disclosure: Pfizer Inc Stock Investment from broker recommendation; Avanir Pharma Stock Investment from broker recommendation

Managing Editor

Timothy P Cripe, MD, PhD, Professor of Pediatric Hematology/Oncology, University of Cincinnati; Director, Translational Research Trials Office, Department of Pediatrics, Cincinnati Children's Hospital Medical Center
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.

CME Editor

Mary E Cataletto, MD, Associate Director, Division of Pediatric Pulmonology, Winthrop University Hospital; Professor of Clinical Pediatrics, State University of New York at Stony Brook; Director of Children's Sleep Services, Winthrop University Hospital
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, Department of Oncology, Division of Pediatric Oncology, 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.

 
 
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