Trends in incidence and mortality risk for acromegaly in Norway: a cohort study

Abstract

Purpose Recent data have shown a decreasing overall mortality in acromegaly over the last decades. However, cancer incidence and cancer-related mortality still appear to be increased. Our aim was to obtain updated epidemiological data from Norway in a clinically well-defined cohort with complete register-based follow-up. Methods Patients diagnosed with acromegaly from South-Eastern Norway between 1999–2019 (n = 262) and age and sex matched population controls (1:100) were included (n = 26,200). Mortality and cancer data were obtained from the Norwegian Cause of Death and Cancer Registry. Mortality and cancer incidence were compared by Kaplan–Meier analyses and Cox regression; we report hazard ratios (HRs) with 95% confidence intervals (95% CI). Results Median age at diagnosis was 48.0 years (interquartile range (IQR): 37.6–58.0). Mean annual acromegaly incidence rate was 4.7 (95% CI 4.2–5.3) cases/106 person-years, and the point prevalence (2019) was 83 (95% CI 72.6–93.5) cases/106 persons. Overall mortality was not increased in acromegaly, HR 0.8 (95% CI 0.5–1.4), cancer-specific and cardiovascular-specific mortality was also not increased (HR: 0.7 (95% CI 0.3–1.8) and 0.8 (95% CI: 0.3–2.5) respectively). The HR for all cancers was 1.45 (1.0–2.1; p = 0.052). Conclusion In this large cohort study, covering the period 1999–2019, patients were treated with individualized multimodal management. Mortality was not increased compared to the general population and comparable with recent registry studies from the Nordic countries and Europe. Overall cancer risk was slightly, but not significantly increased in the patients.

Changes in acromegaly comorbidities, treatment, and outcome over three decades: a nationwide cohort study

“…However, as patients are followed up closely at highly specialized centers, symptoms suggestive of cancer are likely managed more closely. This is supported by the fact that cancer-specific mortality is not increased, as previously reported from this cohort ( 16 ) and observed in other comparable cohorts ( 6 , 18 , 34 ). The incidence of cancer was not found to be increasing in our cohort across the study periods, despite GH excess being a well-established risk factor for the development of cancer.…”

“…Interestingly, cancer was the only comorbidity for which the risk did not significantly increase over the three decades. Cancer was diagnosed in a total of 72 patients, corresponding to 10% of our study population, which is similar to previous publications ( 6 , 21 , 26 ). The modest cancer risk in our cohort aligns well with findings from recent meta-analyses, where population-based studies displayed lower cancer rates as compared to single-center studies and only modestly increased overall cancer risk [standardized incidence ratio (SIR), 1.45 [1.20; 1.75] and 1.5 [1.2; 1.8], respectively] ( 16 , 17 ).…”

“…Acromegaly is a systemic disease caused by increased growth hormone (GH) secretion, predominantly due to a pituitary adenoma ( 1 ). The disease is rare with incidence rates of 3.1 to 5.3 cases/million/year ( 2 – 8 ) and a reported prevalence (per million) in newer studies ranging from 83 to 137 ( 3 , 4 , 6 , 9 , 10 ). The incidence appears to increase, and a shift toward a milder disease phenotype has been suggested ( 4 , 11 – 13 ).…”

“…The availability of medical alternatives has reduced the use of pituitary tumor irradiation and, in turn, reduced irradiation-induced adverse effects such as secondary intracranial tumors, cerebrovascular disease, and hypopituitarism ( 20 ). The diverse treatment options allow for a more personalized and multimodal treatment approach, leading to disease control in ~60%–90% of patients ( 4 , 6 , 21 , 22 ).…”

High mortality risk among women with acromegaly still persists

“…reported a successful reduction in mortality rates in a retrospective study of 442 patients with acromegaly (65% females) to an SMR of 0.72 ( 6 ). In addition, a recent cohort study of 262 patients with acromegaly diagnosed between 1999–2019 in Norway demonstrated a decreasing trend of risk of mortality from 0.88 in patients diagnosed between 1999–2005 to 0.86 in those diagnosed between 2006–2012 and 0.52 in 2013–2019 ( 20 ).…”