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Cited by 423Adrenal and Paraganglionic TumorsRenal cell carcinoma treatmentAdrenal Hormones and Disorders

Adrenocortical carcinoma: diagnosis, evaluation and treatment

NG, LINDA, LIBERTINO, JOHN M.·2003·Journal of Urology
DOI 10.1016/s0022-5347(05)64023-2
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Abstract

PURPOSE: We describe the presenting features, imaging methods, prognosis of and treatment approach to adrenocortical carcinoma. MATERIALS AND METHODS: A comprehensive literature review of adrenal carcinoma was performed using PubMed and a review of current urology, oncology, radiology and pathology journals. RESULTS: The incidence of adrenocortical tumors has been reported to be approximately 2/1 x 10(6) population, although this value may be an underestimate due to an increase in the incidence of unexpected adrenal masses or incidentalomas in the last decade. There has been a bimodal age incidence of adrenal carcinoma with most patients 40 to 50 years old in the studies reviewed. Overall the prevalence of adrenal tumors was slightly higher in females with most presenting with Cushing's syndrome. Of the 602 adrenal tumors reviewed 62% were functional and 38% were nonfunctional. Modern day imaging, including ultrasound, computerized tomography (CT) and magnetic resonance imaging, have greatly improved the diagnosis and staging of adrenal carcinoma. Surgical removal of a localized tumor and absent metastatic disease were associated with improved survival. Medical therapy with mitotane and its successors in patients with metastatic disease appear to be of little benefit for improving survival. CONCLUSIONS: Based on a review of the literature we recommend that hormone levels should be determined in symptomatic and asymptomatic patients with adrenal masses. CT should be the first study done to define an adrenal mass. All solid incidentalomas greater than 5 cm. on CT or magnetic resonance imaging should be removed surgically. The best chance of survival was achieved by surgical extirpation with the value of adjuvant therapy yet to be determined.

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Crossref · 10.1016/s0022-5347(05)64023-2

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36 of 36 references verified0 citation errors · 0 not checkable

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  • Between 40% and 60% of adrenocortical carcinomas are functional and may present with symptoms of hormonal hypersecretion.

    “Between 40% and 60% of ACCs are functional and may present with symptoms of hormonal hypersecretion (126,127,128,129), most commonly with Cushing’s syndrome (estimated 45%).”

    From Adrenal incidentaloma (2020) · cites this paper as [127]

  • Feminization and hyperaldosteronism are rare in adrenocortical carcinoma, occurring in less than 10% of cases.

    “Feminization and hyperaldosteronism are rare occurring in <10% of cases (127,130).”

    From Adrenal incidentaloma (2020) · cites this paper as [127]

  • Primary adrenocortical carcinoma is rare, with an estimated population incidence of 1 to 2 per million per year.

    “Primary ACC is rare, with an estimated population incidence of 1 to 2 per million per year (126,127).”

    From Adrenal incidentaloma (2020) · cites this paper as [127]

  • Cushing's syndrome is the most common presentation of hormonal hypersecretion in functional adrenocortical carcinoma, estimated at 45%.

    “most commonly with Cushing’s syndrome (estimated 45%).”

    From Adrenal incidentaloma (2020) · cites this paper as [127]

  • Adrenocortical carcinoma is rare and aggressive and can occur at any age, but peak incidence tends to be before age 5 and between ages 40 and 60.

    “ACC is rare and aggressive and can occur at any age. However, peak incidence tends to be before the age of 5 and between the ages of 40 and 60 years of age (126,127).”

    From Adrenal incidentaloma (2020) · cites this paper as [127]

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Authors
NG, L., & LIBERTINO, J. M.
Affiliations
Lahey Hospital and Medical Center (US)
Venue
Journal of Urology
Publisher
Ovid Technologies (Wolters Kluwer Health)
Published
2003-01
Volume
169
Issue
1
Pages
5-11
ISSN
  • 0022-5347 (ISSN-L)
  • 1527-3792
DOI
10.1016/s0022-5347(05)64023-2
OpenAlex
W2105268776
Cited by
423
References
37
Citations by year
2026: 8 · 2025: 10 · 2024: 22 · 2023: 15 · 2022: 17 · 2021: 22 · 2020: 21 · 2019: 22 · 2018: 13 · 2017: 14 · 2016: 24 · 2015: 28 · 2014: 24 · 2013: 13 · 2012: 25
FWCI
3.82
Type
journal article
Language
en
Open access
closed
Licence
www.elsevier.com/tdm/userlicense/1.0/ (tdm)
Locations
  • The Journal of Urology (publishedVersion)
  • PubMed (publishedVersion)
Topics
  • Health Sciences › Medicine › Surgery › Adrenal and Paraganglionic Tumors
  • Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Renal cell carcinoma treatment
  • Health Sciences › Medicine › Endocrinology, Diabetes and Metabolism › Adrenal Hormones and Disorders
Keywords
adrenocortical carcinoma, adrenal tumors, Cushing's syndrome, adrenal incidentaloma, functional adrenal tumors, adrenal imaging, computed tomography, magnetic resonance imaging, mitotane, adjuvant therapy, surgical resection, prognosis
MeSH terms
Adrenal Cortex Neoplasms, Humans, Prognosis, Adrenocortical Carcinoma

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