Department of Endocrinology and Internal Medicine, Aarhus University Hospital, Aarhus, Denmark.
Department of Clinical Medicine, Aarhus University, Aarhus, Denmark.
J Clin Endocrinol Metab. 2021 Apr 23;106(5):e2251-e2261. doi: 10.1210/clinem/dgaa965.
Pheochromocytoma and sympathetic paraganglioma (PPGL) are rare catecholamine-secreting tumors but recent studies suggest increasing incidence. Traditionally, PPGL are described to present with paroxysmal symptoms and hypertension, but existing data on clinical presentation of PPGL come from referral centers.
We aimed to describe time trends in clinical presentation and incidence of PPGL in a population-based study.
We conducted a nationwide retrospective cohort study of a previously validated cohort of 567 patients diagnosed with PPGL in Denmark 1977-2015. We collected clinical data from medical records of a geographic subcohort of 192 patients. We calculated age-standardized incidence rates (SIRs) and prevalence for the nationwide cohort and descriptive statistics on presentation for the subset with clinical data.
SIRs increased from 1.4 (95% CI 0.2-2.5) per million person-years in 1977 to 6.6 (95% CI 4.4-8.7) per million person-years in 2015, corresponding to a 4.8-fold increase. The increase was mainly due to incidentally found tumors that were less than 4 cm and diagnosed in patients older than 50 years with no or limited paroxysmal symptoms of catecholamine excess. On December 31, 2015, prevalence of PPGL was 64.4 (CI 95% 57.7-71.2) per million inhabitants. Of 192 patients with clinical data, 171 (89.1%) had unilateral pheochromocytoma, while unilateral paraganglioma (n = 13, 6.8%) and multifocal PPGL (n = 8, 4.2%) were rare.
Incidence of PPGL has increased 4.8-fold from 1977 to 2015 due to a "new" group of older patients presenting with smaller incidentally found PPGL tumors and few or no paroxysmal symptoms.
嗜铬细胞瘤和副神经节瘤（PPGL）是罕见的儿茶酚胺分泌肿瘤，但最近的研究表明其发病率正在增加。传统上，PPGL 表现为阵发性症状和高血压，但关于 PPGL 临床表现的现有数据来自转诊中心。
我们旨在描述一项基于人群的研究中 PPGL 的临床表现和发病率的时间趋势。
我们对丹麦 1977 年至 2015 年确诊的 567 例 PPGL 患者进行了一项全国性回顾性队列研究。我们从地理亚组的 192 名患者的病历中收集了临床数据。我们计算了全国性队列的年龄标准化发病率（SIR）和患病率，并对具有临床数据的亚组进行了描述性统计。
SIR 从 1977 年的每百万人口 1.4（95%CI 0.2-2.5）增加到 2015 年的每百万人口 6.6（95%CI 4.4-8.7），增加了 4.8 倍。这种增加主要是由于偶然发现的肿瘤，这些肿瘤小于 4 厘米，并且在没有或仅有有限的儿茶酚胺过多阵发性症状的 50 岁以上患者中诊断出来。截至 2015 年 12 月 31 日，PPGL 的患病率为每百万居民 64.4（95%CI 57.7-71.2）。在有临床数据的 192 名患者中，171 名（89.1%）患有单侧嗜铬细胞瘤，而单侧副神经节瘤（n=13，6.8%）和多灶性 PPGL（n=8，4.2%）则较为罕见。
由于一组新的年龄较大的患者表现为较小的偶然发现的 PPGL 肿瘤，且阵发性症状较少或没有，1977 年至 2015 年 PPGL 的发病率增加了 4.8 倍。
