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Cited by 349Adrenal and Paraganglionic TumorsNeuroendocrine Tumor Research AdvancesAdrenal Hormones and Disorders

Clinical management of adrenocortical carcinoma

Fassnacht, Martin, Allolio, Bruno·2009·Best Practice & Research Clinical Endocrinology & Metabolism
DOI 10.1016/j.beem.2008.10.008
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Abstract

Adrenocortical carcinoma (ACC) is a rare and heterogeneous malignancy, and most of the diagnostic and therapeutic strategies are not fully established according to criteria of evidence-based medicine. However, recently collaborative efforts (e.g. International Consensus Conference 2003 and networks like the European Network for the Study of Adrenal Tumours (ENSAT)) have significantly advanced the field. This article summarizes current standards in the management of ACC. In patients with suspected ACC a thorough endocrine and imaging work-up is followed by complete (Ro) resection of the tumour by an expert surgeon and initiation of adjuvant mitotane. In advanced disease not amenable to radical resection, cytotoxic drugs will be added to mitotane. The most promising regimens (etoposide, doxorubicin, cisplatin plus mitotane and streptozotocin plus mitotane) are currently compared in an international phase-III trial. Several targeted therapies are under investigation (e.g. IGF-1 inhibitors, sunitinib, sorafenib) and may lead to new treatment options.

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Crossref · 10.1016/j.beem.2008.10.008

Cites

104 of 104 references verified0 citation errors · 0 not checkable

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  • The median age of ACC diagnosis is in the fifth to sixth decade, with the German ACC Registry reporting a median age of 46 years.

    “The median age of diagnosis is in the fifth to sixth decade, with the German ACC Registry reporting a median age at diagnosis of 46 years (9).”

    From Adrenocortical carcinoma (2014) · cites this paper as [9]

  • Another third of ACC patients present with nonspecific symptoms due to local tumor growth, such as abdominal or flank pain, abdominal fullness, or early satiety.

    “Another third present with nonspecific symptoms due to local tumor growth, such as abdominal or flank pain, abdominal fullness, or early satiety (9, 10).”

    From Adrenocortical carcinoma (2014) · cites this paper as [9]

  • For 40% to 60% of ACC patients, the major presenting complaints are symptoms and signs of hormone excess.

    “For 40% to 60% of patients, the major presenting complaints are symptoms and signs of hormone excess (3, 9, 10).”

    From Adrenocortical carcinoma (2014) · cites this paper as [9]

  • Biochemically or clinically apparent adrenocortical hormone production is evident in up to 45% to 70% of ACC patients.

    “Biochemically or clinically apparent adrenocortical hormone production is evident in up to 45% to 70% (9, 10, 85).”

    From Adrenocortical carcinoma (2014) · cites this paper as [9]

  • At the time of presentation, ACCs are generally large tumors, measuring on average 10 to 13 cm.

    “At the time of presentation, ACCs are generally large tumors, measuring on average 10 to 13 cm (9, 85, 89).”

    From Adrenocortical carcinoma (2014) · cites this paper as [9]

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Authors
Fassnacht, M., & Allolio, B.
Affiliations
University of Würzburg (DE)
Corresponding author
Fassnacht, Martin
ORCID
Fassnacht, Martin
Venue
Best Practice & Research Clinical Endocrinology & Metabolism
Publisher
Elsevier BV
Published
2009-04
Volume
23
Issue
2
Pages
273-289
ISSN
1521-690X (ISSN-L)
DOI
10.1016/j.beem.2008.10.008
OpenAlex
W2066923659
Cited by
349
References
106
Citations by year
2026: 8 · 2025: 16 · 2024: 20 · 2023: 24 · 2022: 16 · 2021: 24 · 2020: 20 · 2019: 13 · 2018: 21 · 2017: 12 · 2016: 22 · 2015: 30 · 2014: 15 · 2013: 18 · 2012: 33
FWCI
4.79
Type
journal article
Language
en
Open access
closed
Licence
Locations
  • Best Practice & Research Clinical Endocrinology & Metabolism (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, adrenalectomy, cytotoxic chemotherapy, etoposide, doxorubicin, cisplatin, streptozotocin, targeted therapy, sunitinib, sorafenib, diagnostic workup, advanced disease
MeSH terms
Adrenal Cortex Hormones, Adrenal Cortex Neoplasms, Animals, Humans, Neoplasm Staging, Prognosis, Adrenocortical Carcinoma, Disease Progression

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