BACKGROUND: Acromegaly is so rare that its natural history, including incidence, risk of cancers, and mortality rates remain elusive. This natural study utilized a nationwide database to provide a better understanding of acromegaly's disease course. METHODS: A cohort of 1,195 acromegaly patients were identified and followed-up from 1997 to 2013. Incidence, operation, and re-operation rates were calculated. Excessive mortality and cancer risk related to acromegaly were estimated by standardized mortality ratio (SMR) and standardized incidence ratio (SIR). RESULTS: The incidence was 2.78 per million-person-years, with little gender predominance (female vs male, 49.5% vs 50.5%, respectively). There was female predominance only among 50 and 60 year olds (incidence rate ratio: 1.37 and 1.43, p<0.001 and p=0.002). Among them, 673 (56.3%) had hypophysectomy surgery, and the young-onset (<40 years) patients had more re-operations (15.5%, p=0.01). The overall mortality rate was 22.3 per 1,000-person-years, with a median survival of 4.67 years (with no gender differences, p=0.38). The overall SMR of acromegaly patients was 1.41 and the onset-age-specific SMRs of the early- and middle-onset patients were higher than for those with late-onset. There were 87 newly-diagnosed cancers in the cohort, with an incidence rate of 10.6 per 1,000-person-years (median: 5.4 years). The overall SIR of cancers was 1.91, and there were no differences among gender, onset-age, and disease duration (all SIR>1, approximately 2). CONCLUSION: Acromegaly is associated with an excessive risk of mortality and two-fold higher risk of cancers. Patients with acromegaly should be managed appropriately after the diagnosis.
BACKGROUND:Acromegaly is so rare that its natural history, including incidence, risk of cancers, and mortality rates remain elusive. This natural study utilized a nationwide database to provide a better understanding of acromegaly's disease course. METHODS: A cohort of 1,195 acromegalypatients were identified and followed-up from 1997 to 2013. Incidence, operation, and re-operation rates were calculated. Excessive mortality and cancer risk related to acromegaly were estimated by standardized mortality ratio (SMR) and standardized incidence ratio (SIR). RESULTS: The incidence was 2.78 per million-person-years, with little gender predominance (female vs male, 49.5% vs 50.5%, respectively). There was female predominance only among 50 and 60 year olds (incidence rate ratio: 1.37 and 1.43, p<0.001 and p=0.002). Among them, 673 (56.3%) had hypophysectomy surgery, and the young-onset (<40 years) patients had more re-operations (15.5%, p=0.01). The overall mortality rate was 22.3 per 1,000-person-years, with a median survival of 4.67 years (with no gender differences, p=0.38). The overall SMR of acromegalypatients was 1.41 and the onset-age-specific SMRs of the early- and middle-onset patients were higher than for those with late-onset. There were 87 newly-diagnosed cancers in the cohort, with an incidence rate of 10.6 per 1,000-person-years (median: 5.4 years). The overall SIR of cancers was 1.91, and there were no differences among gender, onset-age, and disease duration (all SIR>1, approximately 2). CONCLUSION:Acromegaly is associated with an excessive risk of mortality and two-fold higher risk of cancers. Patients with acromegaly should be managed appropriately after the diagnosis.
Authors: Maria Fleseriu; Beverly M K Biller; Pamela U Freda; Monica R Gadelha; Andrea Giustina; Laurence Katznelson; Mark E Molitch; Susan L Samson; Christian J Strasburger; A J van der Lely; Shlomo Melmed Journal: Pituitary Date: 2020-10-20 Impact factor: 4.107