The DOI matches its Crossref record; 104 of the 106 references it cites were found in a registry or an index.
Clinical management of adrenocortical carcinoma
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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“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]
In the citing paper
“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]
In the citing paper
“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]
In the citing paper
“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]
In the citing paper
“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]
In the citing paper
“Hypercortisolism is the most common presentation of patients presenting with hormone excess (50%–80% of hormone-secreting ACCs), causing classic symptoms including plethora, diabetes mellitus, muscle weakness/atrophy, and osteoporosis.”
From Adrenocortical carcinoma (2014) · cites this paper as [9]
In the citing paper
“Although earlier studies found 49% of patients with metastatic disease (stage 4) at presentation, currently only 25% to 30% of patients present with metastatic disease (9, 13, 25).”
From Adrenocortical carcinoma (2014) · cites this paper as [9]
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58-Year-Old Man With Hypertension and Diffuse Swelling
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Genetic Disorders of Adrenocortical Function
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CUSHING SENDROMU İLE ORTAYA ÇIKAN METASTATİK DEV ADRENOKORTİKAL KARSİNOM OLGUSU
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Análise dos genes ATRX e ZNRF3 como fatores de prognóstico do carcinoma adrenocortical
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Metadata
- 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)
- 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
- 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 › Oncology › 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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