The DOI matches its Crossref record; 155 of the 157 references it cites were found in a registry or an index.
Management of adrenocortical carcinoma
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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“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]
Citations
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Else, T., Kim, A. C., Sabolch, A., Raymond, V. M., Kandathil, A., Caoili, E. M., Jolly, S., Miller, B. S., Giordano, T. J., & Hammer, G. D. (2014) · Endocrine Reviews · doi:10.1210/er.2013-1029 · Cited by 892
Adrenocortical Carcinoma: Clinical Update
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Extent of disease at presentation and outcome for adrenocortical carcinoma: have we made progress?
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Metadata
- Authors
- Allolio, B., Hahner, S., Weismann, D., & Fassnacht, M.
- Affiliations
- University of Würzburg (DE)
- Corresponding author
- Allolio, Bruno
- Venue
- Clinical Endocrinology
- Publisher
- Wiley
- Published
- 2003-11-24
- Volume
- 60
- Issue
- 3
- Pages
- 273-287
- ISSN
- 0300-0664 (ISSN-L)
- 1365-2265
- 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
- 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 › Oncology › 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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