Pediatric Adrenal Carcinoma Clinical Presentation

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

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.
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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.[2]
  • 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.[3]
  • In rare cases, feminization may occur.
    • Findings in male patients include gynecomastia and hypertension.
    • Findings in female patients include precocious sexual development and hypertension.
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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.
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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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  2. Chen QL, Su Z, Li YH, Ma HM, Chen HS, Du ML. Clinical characteristics of adrenocortical tumors in children. J Pediatr Endocrinol Metab. 2011;24(7-8):535-41. [Medline].

  3. Shah NS, Lila A. Childhood Cushing disease: a challenge in diagnosis and management. Horm Res Paediatr. 2011;76 Suppl 1:65-70. [Medline].

  4. Balassy C, Navarro OM, Daneman A. Adrenal masses in children. Radiol Clin North Am. Jul 2011;49(4):711-27, vi. [Medline].

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  9. 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].

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

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  12. 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].

  13. 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].

  14. 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.

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

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

  17. Rodriguez-Galindo C, Figueiredo BC, Zambetti GP, Ribeiro RC. Biology, clinical characteristics, and management of adrenocortical tumors in children. Pediatr Blood Cancer. Sep 2005;45(3):265-73. [Medline].

  18. 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].

  19. 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].

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  21. 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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