eMedicine Specialties > Pediatrics: General Medicine > Oncology

Adrenal Carcinoma: Differential Diagnoses & Workup

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

Workup

Laboratory Studies

  • Laboratory studies of adrenocortical carcinoma include determinations of serum glucose, serum cortisol, serum adrenal androgen, urine adrenal hormone, urine vanillylmandelic acid (VMA), and urine homovanillic acid (HVA) levels.
  • Laboratory studies enable the physician to distinguish between functioning and nonfunctioning adrenal neoplasms. The results may also help in distinguishing between a neoplasm of the adrenal cortex and a neuroblastoma. Adrenocortical tumors should not be confused with adrenal medullary tumors, also known as pheochromocytomas, which secrete catecholamines, similar to neuroblastomas.

Imaging Studies

  • Imaging studies are the best nonoperative methods to predict which adrenal tumors are affecting the patient.
  • Abdominal and retroperitoneal ultrasonography is usually followed with abdominal CT and MRI.
  • Chest CT should also be performed when metastatic disease is present. Affected lung parenchyma strongly suggests an adrenocortical carcinoma over a neuroblastoma.
  • Bone scanning should also be performed to detect metastatic disease. However, the presence of bone disease does not allow for the differential diagnosis of malignancies.
  • In a recent comparison of imaging findings in pediatric patients with adrenal carcinoma, carcinoma was highly suspected when adrenal lesions had a thin tumoral capsule, a stellate zone of central necrosis, and evidence of the production of adrenocortical hormone. Surgical biopsy or removal should be performed following definitive imaging (see Surgical Care ).

Histologic Findings

  • Histologic findings include numerous mitoses, scant cytoplasm, and none of the rosettes observed in neuroblastoma.
  • The histologic features are characteristic and usually not confused with those of neuroblastoma.
  • Differentiation of adenoma and adenocarcinoma may be difficult. In addition, standard histopathologic staging scales (eg, the Weiss scale) may not be effective in predicting outcome in pediatric adrenocortical tumors.

Staging

  • Staging for adrenocortical carcinoma follows the stage I-IV pattern for most solid tumors.
  • Stages are defined as follows:
    • Stage I is an encapsulated tumor of less than 5 cm that is completely removed.
    • Stage II is a tumor of more than 5 cm that is completely removed.
    • Stage III is a tumor associated with local invasion or with positive lymph nodes but no distant metastasis.
    • Stage IV is a solid tumor with local invasion and positive lymph nodes or metastasis to the liver, lung, or bone.
  • Given the key role of resectability in prognostication and given the lack of consistent efficacy with adjuvant chemotherapy, current staging may best be divided as stage I and II (resectable lesions) and stage III and IV for (unresectable lesions).

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].

  14. 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].

  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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