Lima José Viana, Scalissi Nilza M, Goldman Suzan M, Kater Claudio E
Divisão de Endocrinologia e Metabolismo, Departamento de Medicina, Faculdade de Medicina da Universidade Federal de São Paulo (EPM/UNIFESP), São Paulo, SP, Brasil.
Divisão de Endocrinologia, Departamento de Medicina, Faculdade de Ciências Médicas da Santa Casa de São Paulo, São Paulo, SP, Brasil.
Int Braz J Urol. 2025 Nov-Dec;51(6). doi: 10.1590/S1677-5538.IBJU.2025.0094.
Pheochromocytomas (Pheo) and paragangliomas (PGL) are catecholamine-secreting tumours, whose functionality is confirmed by elevated plasma (Pl) and/or 24-h urinary (Ur) metanephrines (MN). Relevance of prior background features were reviewed in a large cohort of patients with Pheo and PGL.
We reviewed clinical, hormonal, and imaging aspects of 116 patients studied prospectively: 93 Pheo; 22 PGL; one Pheo plus PGL.
Twenty-five % PPGL were discovered incidentally. Systemic arterial hypertension (SAH) was present in 81% (43% on stage 3), whereas 9.5% were prehypertensive and 9.5%, normotensive. SAH plus paroxysms occurred in 31 (32.9%) patients, being exclusively sustained in the remaining; 26 (28%) had resistant SAH. Orthostatic hypotension was seen in 65% of patients. Pl/Ur MN and normetanephrine (NMN) were compared to those of a positive (56 functioning PPGL) and a negative control group (654 subjects with normal MN/NMN). Total and fractionated Ur MN were elevated in 94% PPGL patients. Cut-off values of 885 mcg/24-h for Ur MN, and of 1.5 nmol/L for Pl MN identified functioning lesions with 100%/100% sensitivity and 93%/97% specificity, respectively. MRI detected 56% right-side Pheo, 25% on the left, and 19% bilateral; PGL were 56.5% (13/23) retroperitoneal and 43.5%, cervical (10/23). Right-side Pheo were larger (5.8 cm) than left-side ones (3.7 cm), but retroperitoneal (6.5 cm) and neck PGL (6.9 cm) were similar. Tumour size positively correlated with total Ur MN.
in this large cohort of PPGL patients we highlighted relevant aspects of SAH, the frequently overlooked manifestation of orthostatic hypotension, common incidental presentation, significant tumour size/hormonal production.
嗜铬细胞瘤（Pheo）和副神经节瘤（PGL）是分泌儿茶酚胺的肿瘤，其功能通过血浆（Pl）和/或24小时尿（Ur）间甲肾上腺素（MN）升高得以证实。我们在一大群嗜铬细胞瘤和副神经节瘤患者中回顾了既往背景特征的相关性。
我们回顾了116例前瞻性研究患者的临床、激素和影像学方面情况：93例嗜铬细胞瘤；22例副神经节瘤；1例嗜铬细胞瘤合并副神经节瘤。
25%的副神经节瘤-嗜铬细胞瘤（PPGL）是偶然发现的。81%的患者存在系统性动脉高血压（SAH）（43%处于3期），而9.5%为高血压前期，9.5%血压正常。31例（32.9%）患者出现SAH加阵发性发作，其余患者仅为持续性发作；26例（28%）患者存在顽固性SAH。65%的患者出现体位性低血压。将Pl/Ur MN和去甲变肾上腺素（NMN）与阳性对照组（56例有功能的PPGL）和阴性对照组（654例MN/NMN正常的受试者）进行比较。94%的PPGL患者尿总MN和分段MN升高。尿MN的截断值为885 mcg/24小时，血浆MN的截断值为1.5 nmol/L，分别以100%/100%的敏感性和93%/97%的特异性识别有功能的病变。MRI检测到56%的右侧嗜铬细胞瘤，25%在左侧，19%为双侧；副神经节瘤56.5%（13/23）位于腹膜后，43.5%位于颈部（10/23）。右侧嗜铬细胞瘤（5.8 cm）比左侧嗜铬细胞瘤（3.7 cm）大，但腹膜后副神经节瘤（6.5 cm）和颈部副神经节瘤（6.9 cm）大小相似。肿瘤大小与尿总MN呈正相关。
在这一大群PPGL患者中，我们强调了系统性动脉高血压的相关方面、常被忽视的体位性低血压表现、常见的偶然发现情况、显著的肿瘤大小/激素产生情况。
