Cited by 396Adrenal and Paraganglionic TumorsNeuroendocrine Tumor Research AdvancesAdrenal Hormones and Disorders

Adrenocortical carcinoma: a clinician’s update

Fassnacht, Martin, Libé, Rossella, Kroiss, Matthias, Allolio, Bruno·2011·Nature Reviews Endocrinology

Abstract

Adrenocortical carcinoma is a rare heterogeneous neoplasm with an incompletely understood pathogenesis and a poor prognosis. Previous studies have identified overexpression of insulin-like growth factor 2 (IGF-2) and constitutive activation of β-catenin as key factors involved in the development of adrenocortical carcinoma. Most patients present with steroid hormone excess, for example Cushing syndrome or virilization, or abdominal mass effects, but a growing proportion of patients with adrenocortical carcinoma (currently >15%) is initially diagnosed incidentally. No general consensus on the diagnostic and therapeutic measures for adrenocortical carcinoma exists, but collaborative efforts, such as international conferences and networks, including the European Network for the Study of Adrenal Tumors (ENSAT), have substantially advanced the field. In patients with suspected adrenocortical carcinoma, a thorough endocrine and imaging work-up is recommended to guide the surgical approach aimed at complete resection of the tumor. To establish an adequate basis for treatment decisions, pathology reports include the Weiss score to assess malignancy, the resection status and the Ki67 index. As recurrence is frequent, close follow-up initially every 3 months is mandatory. Most patients benefit from adjuvant mitotane treatment. In metastatic disease, mitotane is the cornerstone of initial treatment, and cytotoxic drugs should be added in case of progression. Results of a large phase III trial in advanced adrenocortical carcinoma are anticipated for 2011 and will hopefully establish a benchmark therapy. New targeted therapies, for example, IGF-1 receptor inhibitors, are under investigation and may soon improve current treatment options.

Integrity signals

Registry

Crossref · 10.1038/nrendo.2010.235

Cites

147 of 147 references verified0 citation errors · 0 not checkable

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  • Elevated sex steroids are highly suspicious for ACC and urgent diagnostic evaluation is required.

    “Although benign cortisol-secreting adenomas may very rarely produce androgens (286), elevated sex steroids are highly suspicious for ACC and urgent diagnostic evaluation is required (287-289).”

    From Adrenal incidentaloma (2020) · cites this paper as [287]

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Authors
Fassnacht, M., Libé, R., Kroiss, M., & Allolio, B.
Affiliations
  • University of Würzburg (DE)
  • Hôpital Cochin (FR)
Corresponding author
Fassnacht, Martin
Venue
Nature Reviews Endocrinology
Published
2011-03-08
Volume
7
Issue
6
Pages
323-335
ISSN
  • 1759-5029 (ISSN-L)
  • 1759-5037
OpenAlex
W2068845486
Cited by
396
References
147
Citations by year
2026: 9 · 2025: 21 · 2024: 10 · 2023: 16 · 2022: 22 · 2021: 28 · 2020: 31 · 2019: 19 · 2018: 32 · 2017: 21 · 2016: 18 · 2015: 43 · 2014: 36 · 2013: 38 · 2012: 32
FWCI
7.29
Type
journal article
Language
en
Open access
closed
Locations
  • Nature Reviews Endocrinology (publishedVersion)
  • PubMed (publishedVersion)
Funders
Deutsche Krebshilfe
Topics
  • Health Sciences › Medicine › Surgery › Adrenal and Paraganglionic Tumors
  • Health Sciences › Medicine › Epidemiology › Neuroendocrine Tumor Research Advances
  • Health Sciences › Medicine › Endocrinology, Diabetes and Metabolism › Adrenal Hormones and Disorders
Keywords
adrenocortical carcinoma, pathogenesis, insulin-like growth factor II, β-catenin activation, Cushing's syndrome, masculinization, incidental diagnosis, Weiss score, Ki-67 proliferation index, mitotane, adjuvant therapy, metastatic disease, cytotoxic chemotherapy, targeted therapy, recurrence, prognosis
MeSH terms
Adrenal Cortex Neoplasms, Algorithms, Education, Medical, Continuing, Endocrinology, Humans, Models, Biological, Oncogenes, Genes, Tumor Suppressor, Adrenocortical Carcinoma, Clinical Laboratory Techniques

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