Pihlblad Vincent E D, Calissendorff Jan, Falhammar Henrik
Department of Molecular Medicine and Surgery, Karolinska Institutet, Stockholm, Sweden.
Department of Endocrinology, Karolinska University Hospital, Stockholm, Sweden.
Clin Endocrinol (Oxf). 2025 Jul 10. doi: 10.1111/cen.70002.
Pheochromocytomas and sympathetic paragangliomas (PPGLs) are similar in most aspects. However, they differ in genetic etiology, hormonal secretion, and associated neoplasms. This study aimed to investigate differences in clinical presentation between PPGLs.
This study employs a retrospective cohort design.
The cohort consists of 201 patients with PPGLs, including 196 with data on symptoms, treated at a major tertiary centre between 2005 and 2024.
Data on age at diagnosis, sex, tumour size, metastases, genetic profiles, mode of discovery, pathology, biochemistry, symptoms, co-morbidities and blood pressure were collected from patients' medical records and compared between patients with pheochromocytomas and paragangliomas.
Women were less prevalent in the pheochromocytoma group compared to the paraganglioma group (51.5% vs. 71.1%, p = 0.03). Pheochromocytomas produced more epinephrine (p < 0.001), though 25.0% of paragangliomas were also producing increased epinephrine concentrations. Most symptoms were similar between groups, but pheochromocytomas were associated with higher prevalence of palpitations (48% vs. 29%, p = 0.04) and anxiety (37% vs. 17%, p = 0.03) than paragangliomas. The pheochromocytoma group reported more symptoms (3 vs. 2, p = 0.01). Pre-diabetes/diabetes was more prevalent in patients with pheochromocytoma (45% vs. 24%, p = 0.02). The classic triad (palpitations, sweating, headaches) occurred in 12% of all patients, and hypertension in 82% of all patients, both being similar in prevalence between the PCC and PGL groups.
Pheochromocytomas and paragangliomas exhibit some notable differences in symptoms and hormone profiles but generally share many features. These distinctions are, however, insufficient for clinicians to differentiate the two tumours based solely on clinical presentation or biochemical data.
嗜铬细胞瘤和交感神经节细胞瘤（PPGLs）在大多数方面相似。然而，它们在遗传病因、激素分泌和相关肿瘤方面存在差异。本研究旨在调查PPGLs之间临床表现的差异。
本研究采用回顾性队列设计。
该队列包括201例PPGLs患者，其中196例有症状数据，于2005年至2024年在一家大型三级中心接受治疗。
从患者病历中收集诊断时的年龄、性别、肿瘤大小、转移情况、基因谱、发现方式、病理、生物化学、症状、合并症和血压等数据，并在嗜铬细胞瘤和副神经节瘤患者之间进行比较。
与副神经节瘤组相比，嗜铬细胞瘤组女性患病率较低（51.5%对71.1%，p = 0.03）。嗜铬细胞瘤产生更多肾上腺素（p < 0.001），不过25.0%的副神经节瘤也产生升高的肾上腺素浓度。两组之间大多数症状相似，但嗜铬细胞瘤与心悸（48%对29%，p = 0.04）和焦虑（37%对17%，p = 0.03）的患病率高于副神经节瘤。嗜铬细胞瘤组报告的症状更多（3种对2种，p = 0.01）。糖尿病前期/糖尿病在嗜铬细胞瘤患者中更常见（45%对24%，p = 0.02）。经典三联征（心悸、出汗、头痛）在所有患者中发生率为12%，高血压在所有患者中发生率为82%，在嗜铬细胞瘤和副神经节瘤组中的患病率相似。
嗜铬细胞瘤和副神经节瘤在症状和激素谱方面存在一些显著差异，但总体上有许多共同特征。然而，这些差异不足以让临床医生仅根据临床表现或生化数据来区分这两种肿瘤。
