eMedicine Specialties > Pediatrics: General Medicine > Oncology

Adrenal Carcinoma: Follow-up

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

Follow-up

Further Inpatient Care

  • Patients with adrenal carcinomas who undergo complete surgical resection with no evidence of continuing functional hormone production do not require further inpatient care.
  • If the patient has evidence of local or distant metastases during ambulatory follow-up, aggressive attempts at repeat resection should be undertaken. These attempts leads to additional inpatient care.
  • If treatment includes intensive chemotherapy, further inpatient care is necessary to deliver chemotherapy or to treat chemotherapy-related toxicity.
  • If lesions seem particularly sensitive to chemotherapy, with dramatic diminishment of tumoral masses in the chest or elsewhere, autologous transplantation might be a consideration. However, only anecdotal data suggest that transplantation is helpful in managing this disease. One study reported the use of chemotherapy, surgical debulking of lung metastases, and autologous transplantation; 2 years of continuous complete remission was reported.3

Further Outpatient Care

  • Ambulatory follow-up should occur every month for the first 2 years after treatment because repeat resection of locally recurring disease and resection of metastatic lung disease can substantially affect long-term survival.
  • Scanning of the local area in the abdomen or pelvis and of sites of metastatic disease should continue every 3 months for the first 2 years, every 4 months for the next 2 years, and every 6 months during the fifth year.
  • Patients should be monitored for the reappearance of adrenocortical hormone hyperactivity, along with scanning, unless their history suggests that Cushing syndrome or autonomous adrenocortical hormonal production is present. If this is the case, the physician should immediately search for recurrence.

Prognosis

The overall prognosis for patients with adrenal carcinoma is poor.

  • In a study of pediatric patients with adrenocortical tumors, overall survival was 54.2%.1 The investigators did not distinguish adenoma from adenocarcinoma because of the difficulty (even with expert review) of separating these diagnoses at early stages. The excellent survival rate of patients with stage I tumors, the group who have adenomas, affects this rate. However, numerous valuable prognostic indicators have emerged; these serve to comfort patients with completely resected disease and suggest experimental or palliative care for those without. 
    • Stage: By far the most important prognostic variable is the clinical stage. Because adenoma is usually stage I by definition, the observation that stage I survival rates approach 90% because of complete resection is no surprise. However, adenocarcinomas are noted in this group as well, and many investigators believe that a small (<200 g) stage I carcinoma can be associated with a favorable prognosis when it is completely removed without tumor spill. With stage II disease, the survival rate decreases to close to 40%, even with complete resection. Stage III and IV disease result in equally poor survival rates of less than 20%.
    • Age: In the aforementioned study, patients younger than 4 years had an event-free survival rate of more than 70%. The event-free survival rate for patients aged 4-12 years decreased to 30-40%.
    • Function: Tumors that virilize alone or nonfunctioning tumors improve the prognosis compared with tumors that produce Cushing syndrome due to an overproduction of glucocorticoid.
  • In a study of 31 patients at the Lahey Clinic over 30 years, mean survival was 17 months (range, 1-205 mo). The 5-year survival rate was 26%.5
  • As surgical procedures for tumor removal improve, patients' prognoses should also improve.
  • At present, medical therapy has a palliative role. A well-conducted series of clinical trials is needed to prove that adjuvant therapy helps to prolong survival.

Miscellaneous

Medicolegal Pitfalls

  • In general, children older than 4 years present with retroperitoneal tumors in a bulky or advanced form. This presentation may lead to an inappropriate placement of blame on the primary care physician for not detecting the tumor early, especially if the patient has had nonspecific signs (eg, constipation, mild abdominal pain).
  • The prognosis of patients with adrenocortical carcinoma is grave even when the tumor is discovered early.
 


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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