Institute of Metabolism and Systems Research, University of Birmingham, Birmingham B15 2TT, UK.
Centre for Endocrinology, Diabetes and Metabolism, Birmingham Health Partners, Birmingham B15 2TT, UK.
J Clin Endocrinol Metab. 2023 Dec 21;109(1):e389-e396. doi: 10.1210/clinem/dgad401.
Pheochromocytomas are increasingly diagnosed in incidentally detected adrenal masses. However, the characteristics of incidental pheochromocytomas are unclear.
We aimed to assess the proportion and clinical, biochemical, radiological, genetic, histopathological, and follow-up characteristics of incidental pheochromocytomas.
A retrospective review was conducted of patients with pheochromocytoma seen between January 2010 and October 2022 at a large UK tertiary care center. The diagnosis was confirmed histologically or by the combined presence of increased plasma and/or urinary metanephrines (MN), indeterminate adrenal mass on cross-sectional imaging, and metaiodobenzylguanidine avidity.
We identified 167 patients with pheochromocytoma; 144 (86.2%) underwent adrenalectomy, for 23 (13.8%) surgery was either awaited, deemed unsuitable due to frailty or other metastatic malignancy, or declined by the patients. Excluding pheochromocytomas diagnosed via screening genetically predisposed individuals (N = 20), 37 of 132 (28.0%) presented with adrenergic symptoms and/or uncontrolled hypertension, while 91 of 132 (69.0%) patients presented with an incidentally detected adrenal mass. Incidentally detected patients were older (median age 62 years) than those detected due to clinical suspicion (aged 42 years) or after genetic screening (aged 33 years) (all P < .05). Incidentally detected pheochromocytomas were smaller (median 42 mm) than tumors detected due to adrenergic symptoms/uncontrolled hypertension (60 mm), but larger than tumors identified by genetic screening (30 mm) (all P < .05). Increased MN excretion showed a similar pattern (symptomatic/uncontrolled hypertension > incidental > genetic screening) (all P < .05). Hereditary predisposition was detected in 20.4% of patients (incidental, 15.3%; symptomatic/uncontrolled hypertension, 42.9%).
The majority of pheochromocytomas are diagnosed incidentally and have distinct clinical, radiological, biochemical, and genetic features. Their detection at older age but smaller size may point to a different underlying tumor biology.
嗜铬细胞瘤越来越多地在偶然发现的肾上腺肿块中被诊断出来。然而，偶然发现的嗜铬细胞瘤的特征尚不清楚。
我们旨在评估偶然发现的嗜铬细胞瘤的比例以及临床、生化、影像学、遗传、组织病理学和随访特征。
对 2010 年 1 月至 2022 年 10 月在英国一家大型三级保健中心就诊的嗜铬细胞瘤患者进行回顾性分析。诊断通过组织学或血浆和/或尿液间甲肾上腺素（MN）升高的联合存在、横断面成像上不确定的肾上腺肿块以及间碘苄胍摄取来确认。
我们共发现 167 例嗜铬细胞瘤患者；144 例（86.2%）接受了肾上腺切除术，23 例（13.8%）因体弱或其他转移性恶性肿瘤而等待手术，或被患者拒绝。排除通过遗传筛查筛查出的遗传性易患个体的嗜铬细胞瘤（N=20）后，37 例（28.0%）因肾上腺素能症状和/或未控制的高血压而就诊，91 例（69.0%）因偶然发现的肾上腺肿块就诊。偶然发现的患者比因临床怀疑（42 岁）或遗传筛查（33 岁）而发现的患者年龄更大（中位年龄 62 岁）（均 P<.05）。偶然发现的嗜铬细胞瘤比因肾上腺素能症状/未控制的高血压而发现的肿瘤（60 毫米）更小，但比通过遗传筛查发现的肿瘤（30 毫米）更大（均 P<.05）。MN 排泄增加也呈现出类似的模式（肾上腺素能症状/未控制的高血压>偶然发现>遗传筛查）（均 P<.05）。在 20.4%的患者中发现了遗传易感性（偶然发现，15.3%；有症状/未控制的高血压，42.9%）。
大多数嗜铬细胞瘤是偶然发现的，具有明显的临床、放射学、生化和遗传特征。它们在年龄较大时被发现，但体积较小，这可能表明肿瘤的生物学基础不同。
