Pheochromocytoma Characteristics and Behavior Differ Depending on Method of Discovery
Pheochromocytoma Characteristics and Behavior Differ Depending on Method of Discovery
复制标题
DOI：
10.1210/jc.2018-01707
复制
发表时间：
2019-05-01
影响因子：
5.8
通讯作者：
Bancos, Irina
中科院分区：
文献类型：
--
作者：
Gruber, Lucinda M.;Hartman, Robert P.;Bancos, Irina
内容：现代嗜铬细胞瘤（PHEOs）往往是偶然发现的横截面成像或基于突变的遗传病例检测测试。关于这些PHEOs的表现知之甚少。目的：描述通过成像或基于突变的遗传病例检测检测偶然发现的PHEOs的特征和行为。设计：回顾性研究。设置：转诊中心。患者：2005年至2016年接受治疗的经病理证实的PHEOs的并发患者。主要结局指标：肿瘤大小，血浆/尿分级metanephrines和儿茶酚胺，和术前management.Results：二百七十一例（52%的妇女，中位年龄52.0岁）提出了296 PHEO。发现方法最常见的是横截面成像的偶然发现（61%），而不是PHEO相关症状（27%）或基于突变的病例检测测试（12%）。偶然发现PHEO的患者比有症状和基于突变的病例检测检测患者年龄大（中位年龄56.6岁vs 43岁vs 35岁，P < 0.0001）。基于突变的病例检测PHEO小于因症状发现的PHEO（中位尺寸29.0 vs 50.5 mm，P = 0.0027）。因症状而发现PHEO的患者24小时尿变肾上腺素和总血浆变肾上腺素的中位浓度最高（P < 0.0001）。这些患者需要更高的累积酚苄明剂量比偶然或病例检测PHEO患者（中位数450 vs 375 vs 270 mg，P = 0.029）。结论：PHEO主要是由于偶然发现的横断面成像，而不是PHEO相关的症状。与因症状发现的PHEO相比，通过基于突变的遗传病例检测试验发现的PHEO较小，术前需要的α-肾上腺素能阻滞较少，这支持对有PHEO遗传易感性的患者进行常规病例检测试验。
Context: Modern pheochromocytomas (PHEOs) are often discovered by incidental finding on cross-sectional imaging or mutation-based genetic case detection testing. Little is known about how these PHEOs behave.Objective: To describe the characteristics and behavior of PHEOs discovered incidentally on imaging or through mutation-based genetic case detection testing.Design: Retrospective study.Setting: Referral center.Patients: Consecutive patients with pathology-confirmed PHEOs, treated from 2005 to 2016.Main Outcome Measure(s): Tumor size, plasma/urine fractionated metanephrines and catecholamines, and preoperative management.Results: Two hundred seventy-one patients (52% women, median age 52.0 years) presented with 296 PHEOs. Discovery method was most often incidental finding on cross-section imaging (61%) rather than PHEO-related symptoms (27%) or mutation-based case detection testing (12%). Patients with incidentally discovered PHEOs were older than symptomatic and mutation-based case detection testing patients (median age 56.6 vs 43 vs 35 years, P < 0.0001). Mutation-based case detection PHEOs were smaller than those discovered due to symptoms (median size 29.0 vs 50.5 mm, P = 0.0027). Patients with PHEOs discovered due to symptoms had the highest median concentration of 24-hour urinary metanephrines and total plasma metanephrines (P < 0.0001). These patients required a higher cumulative phenoxybenzamine dose than patients with incidental or case detection PHEO (median 450 vs 375 vs 270 mg, P = 0.029).Conclusions: PHEOs are primarily discovered due to incidental finding on cross-sectional imaging rather than PHEO-related symptoms. PHEOs discovered through mutation-based genetic case detection testing were smaller and required less a-adrenergic blockade preoperatively compared with PHEOs found due to symptoms, which supports routine case detection testing for patients genetically predisposed for PHEOs.