Aldosterone-producing adrenocortical carcinoma: an unusual cause of Conn's syndrome with an ominous clinical course
Open accessAbstract
Aldosterone-producing adrenocortical carcinoma (APAC) is a rare cause of hypertension often diagnosed late because of paucity of information. Thus, we delineated its clinical course and survival rates based on two cases referred to us that featured diverging clinical courses, and on a scrutiny of the literature since 1955 when the first case of APAC was identified. Data on demography, imaging results, hormonal assessment, histology, and clinical course were extracted independently by the investigators. We included in our database 58 cases, most presenting with Conn's syndrome. Plasma aldosterone levels were on average increased 14-fold; plasma renin activity was suppressed in 55% of cases. The tumor showed extremely variable size and weight, and no gender or side preference. Metastases were present in 10% of all cases at initial diagnosis and in an additional 48% of cases at follow-up. Median survival was 546 days (95% confidence interval (CI): 240-851); median time to either recurrence or death was 212 days (95% CI: 29-395). No clinical or histological signs predicted survival with Cox regression analysis. We concluded that, although an ominous course with a poor survival rate is common, no sign accurately predicts the course of APAC. Thus, molecular studies to identify diagnostic markers of survival are mandatory.
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Autonomous aldosterone secretion is rare in ACC.
“Autonomous aldosterone secretion (which classically leads to hypertension and hypokalemia) is rare in ACC (85, 86).”
From Adrenocortical carcinoma (2014) · cites this paper as [86]
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- Authors
- Seccia, T. M., Fassina, A., Nussdorfer, G. G., Pessina, A. C., & Rossi, G. P.
- Affiliations
- University of Bari Aldo Moro (IT)
- University of Padua (IT)
- Venue
- Endocrine Related Cancer
- Publisher
- Bioscientifica
- Published
- 2005-03-01
- Volume
- 12
- Issue
- 1
- Pages
- 149-159
- ISSN
- 1351-0088 (ISSN-L)
- 1479-6821
- OpenAlex
- W2133643770
- Cited by
- 131
- References
- 82
- Citations by year
- 2026: 4 · 2025: 4 · 2024: 6 · 2023: 5 · 2022: 5 · 2021: 3 · 2020: 9 · 2019: 9 · 2018: 10 · 2017: 3 · 2016: 5 · 2015: 11 · 2014: 6 · 2013: 8 · 2012: 12
- FWCI
- 4.44
- Type
- journal article
- Language
- en
- Update notices
- correction (2006-03-01)
- Open access
- bronze
- doi.org
- Locations
- Endocrine Related Cancer (publishedVersion)
- PubMed (publishedVersion)
- Padua Research Archive (University of Padova) (submittedVersion, other-oa)
- INFM-OAR (INFN Catania) (submittedVersion, other-oa)
- Topics
- Health Sciences › Medicine › Surgery › Adrenal and Paraganglionic Tumors
- Health Sciences › Medicine › Endocrinology, Diabetes and Metabolism › Hormonal Regulation and Hypertension
- Health Sciences › Medicine › Endocrinology, Diabetes and Metabolism › Adrenal Hormones and Disorders
- Keywords
- primary aldosteronism, hypertension, survival, metastasis, plasma aldosterone, plasma renin activity, prognostic factors, delayed diagnosis
- MeSH terms
- Adrenal Gland Neoplasms, Adult, Aldosterone, Female, Humans, Middle Aged, Prognosis, Syndrome, Survival Rate, Adrenocortical Carcinoma
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