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Cited by 214Adrenal and Paraganglionic TumorsAdrenal Hormones and DisordersNeuroendocrine Tumor Research Advances

Management of adrenocortical carcinoma

Allolio, Bruno, Hahner, Stefanie, Weismann, Dirk, Fassnacht, Martin·2004·Clinical Endocrinology
DOI 10.1046/j.1365-2265.2003.01881.x
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Abstract

Adrenocortical carcinoma (ACC) is a rare neoplasm with poor prognosis. Patients present with signs of steroid hormone excess (e.g. Cushing's syndrome, virilization) or an abdominal mass. Tumour size at presentation (mean diameter at diagnosis > 10 cm) is the most important indicator of malignancy. In addition, computed tomography (CT) typically demonstrates an inhomogeneous adrenal lesion with irregular margins and variable enhancement of solid components after intravenous contrast media. Magnetic resonance imaging (MRI) is equally effective as CT and is particularly helpful to visualize invasion into large vessels. Complete tumour removal (R0 resection) offers by far the best chance for long-term survival and therefore surgery is the treatment of choice in stage I-III ACC. Despite tumour resection for cure most patients will eventually develop local recurrence or distant metastases. Thus adjuvant treatment options need to be evaluated in high-risk patients (e.g. radiation therapy of the tumour bed and/or chemotherapy). In tumour recurrence re-operation should always be considered. In metastatic disease (stage IV ACC) not amenable to surgery mitotane (o,p'DDD) remains the first-line therapy. Drug monitoring is needed for effective treatment aiming at concentrations between 14 and 20 mg/l. Patients not responding to mitotane may benefit from cytotoxic chemotherapy (23% partial remissions, 4% complete remissions). Only large prospective multicentre trials comparing different treatment options will allow to make systematic progress in the management of ACC.

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Crossref · 10.1046/j.1365-2265.2003.01881.x

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  • The most common metastatic sites are lung (40%-80%), liver (40%-90%), and bone (5%-20%).

    “The most common metastatic sites are lung (40%–80%), liver (40%–90%), and bone (5%–20%) (92).”

    From Adrenocortical carcinoma (2014) · cites this paper as [92]

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Authors
Allolio, B., Hahner, S., Weismann, D., & Fassnacht, M.
Affiliations
University of Würzburg (DE)
Corresponding author
Allolio, Bruno
ORCID
Venue
Clinical Endocrinology
Publisher
Wiley
Published
2003-11-24
Volume
60
Issue
3
Pages
273-287
ISSN
  • 0300-0664 (ISSN-L)
  • 1365-2265
DOI
10.1046/j.1365-2265.2003.01881.x
OpenAlex
W2044828697
Cited by
214
References
157
Citations by year
2026: 1 · 2025: 2 · 2024: 2 · 2023: 3 · 2022: 8 · 2021: 7 · 2020: 7 · 2019: 3 · 2018: 8 · 2017: 3 · 2016: 8 · 2015: 8 · 2014: 18 · 2013: 9 · 2012: 8
FWCI
2.49
Type
journal article
Language
en
Open access
closed
Licence
onlinelibrary.wiley.com/termsAndConditions#vor (vor)
Locations
  • Clinical Endocrinology (publishedVersion)
  • PubMed (publishedVersion)
Topics
  • Health Sciences › Medicine › Surgery › Adrenal and Paraganglionic Tumors
  • Health Sciences › Medicine › Endocrinology, Diabetes and Metabolism › Adrenal Hormones and Disorders
  • Health Sciences › Medicine › Epidemiology › Neuroendocrine Tumor Research Advances
Keywords
adrenocortical carcinoma, Cushing's syndrome, masculinization, R0 resection, adjuvant radiotherapy, adjuvant chemotherapy, mitotane, drug monitoring, metastatic disease, tumor recurrence, prognosis
MeSH terms
Adrenal Cortex Neoplasms, Antineoplastic Agents, Carcinoma, Humans, Magnetic Resonance Imaging, Neoplasm Staging, Prognosis, Tomography, X-Ray Computed, Cytokines

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