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¹ Department of Clinical Methodology and Medical, Surgical Technologies, University of Bari, Piazza G Cesare 11, 70124 Bari, Italy
² Department of Oncological Sciences: Section of Pathology, University of Padova, Via Giustiniani 2, 35128 Padova, Italy
³ Department of Human Anatomy and Physiology (Section of Anatomy), University of Padova, Via Gabelli, 35126 Padova, Italy
⁴ Department of Clinical and Experimental Medicine, Clinica Medica 4, University of Padova, Via Giustiniani 2, 35128 Padova, Italy

(Requests for offprints should be addressed to G P Rossi; Email: gianpaolo.rossi{at}unipd.it)

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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