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Cited by 775Adrenal and Paraganglionic TumorsAdrenal Hormones and DisordersNeuroendocrine Tumor Research Advances

Adrenocortical Carcinoma: Clinical Update

Allolio, Bruno, Fassnacht, Martin·2006·The Journal of Clinical Endocrinology & Metabolism
DOI 10.1210/jc.2005-2639
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Abstract

CONTEXT: Adrenocortical carcinoma (ACC) is a rare and heterogeneous malignancy with incompletely understood pathogenesis and poor prognosis. Patients present with hormone excess (e.g. virilization, Cushing's syndrome) or a local mass effect (median tumor size at diagnosis > 10 cm). This paper reviews current diagnostic and therapeutic strategies in ACC. EVIDENCE ACQUISITION: Original articles and reviews were identified using a PubMed search strategy (http://www.ncbi.nlm.nih.gov/entrez/query.fcgi) covering the time period up until November 2005. The following search terms were used in varying combinations: adrenal, adrenocortical, cancer, carcinoma, tumor, diagnosis, imaging, treatment, radiotherapy, mitotane, cytotoxic, surgery. EVIDENCE SYNTHESIS: Tumors typically appear inhomogeneous in both computerized tomography and magnetic resonance imaging with necroses and irregular borders and differ from benign adenomas by their low fat content. Hormonal analysis reveals evidence of steroid hormone secretion by the tumor in the majority of cases, even in seemingly hormonally inactive lesions. Histopathology is crucial for the diagnosis of malignancy and may also provide important prognostic information. In stages I-III open surgery by an expert surgeon aiming at an R0 resection is the treatment of choice. Local recurrence is frequent, particularly after violation of the tumor capsule. Surgery also plays a role in local tumor recurrence and metastatic disease. In patients not amenable to surgery, mitotane (alone or in combination with cytotoxic drugs) remains the treatment of choice. Monitoring of drug levels (therapeutic range 14-20 mg/liter) is mandatory for optimum results. In advanced disease, the most promising therapeutic options (etoposide, doxorubicin, cisplatin plus mitotane, and streptozotocin plus mitotane) are currently being compared in an international phase III trial (www.firm-act.org). Adjuvant treatment options after complete tumor removal (e.g. mitotane, radiotherapy) are urgently needed because postoperative disease-free survival at 5 yr is only around 30%, but options have still not been convincingly established. National registries, international cooperations, and trials provide important new structures for patients but also for researchers aiming at systematic and continuous progress in ACC. However, future advances in the management of ACC will mainly depend on a better understanding of the molecular pathogenesis facilitating the use of modern cancer treatments (e.g. tyrosine kinase inhibitors).

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Crossref · 10.1210/jc.2005-2639

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156 of 156 references verified0 citation errors · 0 not checkable

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  • Between 40% and 60% of adrenocortical carcinomas are functional and may present with symptoms of hormonal hypersecretion.

    “Between 40% and 60% of ACCs are functional and may present with symptoms of hormonal hypersecretion (126,127,128,129), most commonly with Cushing’s syndrome (estimated 45%).”

    From Adrenal incidentaloma (2020) · cites this paper as [126]

  • Primary adrenocortical carcinoma is rare, with an estimated population incidence of 1 to 2 per million per year.

    “Primary ACC is rare, with an estimated population incidence of 1 to 2 per million per year (126,127).”

    From Adrenal incidentaloma (2020) · cites this paper as [126]

  • Cushing's syndrome is the most common presentation of hormonal hypersecretion in functional adrenocortical carcinoma, estimated at 45%.

    “most commonly with Cushing’s syndrome (estimated 45%).”

    From Adrenal incidentaloma (2020) · cites this paper as [126]

  • Adrenocortical carcinoma is rare and aggressive and can occur at any age, but peak incidence tends to be before age 5 and between ages 40 and 60.

    “ACC is rare and aggressive and can occur at any age. However, peak incidence tends to be before the age of 5 and between the ages of 40 and 60 years of age (126,127).”

    From Adrenal incidentaloma (2020) · cites this paper as [126]

  • ACC incidence is believed to be 1 to 2 per million per year, but valid data are lacking.

    “The incidence is believed to be 1 to 2 per million per year, but valid data are lacking (3).”

    From Adrenocortical carcinoma (2014) · cites this paper as [3]

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Authors
Allolio, B., & Fassnacht, M.
Affiliations
Universitätsklinikum Würzburg (DE)
Corresponding author
Allolio, Bruno
ORCID
Fassnacht, Martin
Venue
The Journal of Clinical Endocrinology & Metabolism
Publisher
The Endocrine Society
Published
2006-06-01
Volume
91
Issue
6
Pages
2027-2037
ISSN
  • 0021-972X (ISSN-L)
  • 1945-7197
DOI
10.1210/jc.2005-2639
OpenAlex
W2035178765
Cited by
775
References
156
Citations by year
2026: 16 · 2025: 22 · 2024: 25 · 2023: 27 · 2022: 35 · 2021: 39 · 2020: 37 · 2019: 39 · 2018: 38 · 2017: 37 · 2016: 42 · 2015: 47 · 2014: 47 · 2013: 43 · 2012: 46
FWCI
7.52
Type
journal article
Language
en
Open access
closed
Locations
  • The Journal of Clinical Endocrinology & Metabolism (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, adrenal imaging, histopathology, adrenalectomy, mitotane, adjuvant therapy, chemotherapy, radiotherapy, local recurrence, metastatic disease, adjuvant radiotherapy, tyrosine kinase inhibitors, molecular pathogenesis, adrenal adenoma
MeSH terms
Adrenal Cortex Neoplasms, Follow-Up Studies, Humans, Neoplasm Staging, Prognosis

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