Abstract

BackgroundDespite an improved understanding of pheochromocytoma and extra-adrenal sympathetic parganglioma (PPGL), including diagnosis and management, some PPGLs are postoperatively diagnosed. Clinical characteristics and intraoperative haemodynamic instability (HI) in postoperatively diagnosed PPGL patients have been poorly defined. Thus, we investigated the clinical characteristics and HI in patients with postoperatively diagnosed PPGLs compared to patients with preoperatively diagnosed PPGLs.MethodsWe obtained clinical and haemodynamic data from the electronic medical records of 256 patients with pathologically confirmed PPGLs at our institution from January 2005 to December 2019. We assessed the intraoperative HI (systolic blood pressure [SBP]>160 mmHg (min) or mean blood pressure [MBP]<60 mmHg (min)) over time.ResultsTwenty-nine patients (11.3%) were diagnosed with PPGLs postoperatively. Hypertension (34.5% vs. 63.0%, P=0.006) and pheochromocytoma (17.2% vs. 81.1%, P<0.001) case rates were lower in postoperatively diagnosed patients than in preoperatively diagnosed patients. Preoperative SBP in the ward was similar between groups, but the use of α-blockers and β-blockers was more frequent in preoperatively diagnosed patients (89.0% vs. 3.4%, P<0.001; 36.3% vs. 6.9%, P=0.003). Considering intraoperative HI, postoperatively diagnosed patients demonstrated a similar percentage of time with SBP>160 mmHg (median [IQR]; 7.9% [2.5; 11.9] % vs. 4.6% [0.0; 11.9], P=0.088) but a significantly lower percentage of time with MBP<60 mmHg (0.0% [0.0; 3.0] vs. 5.6% [0.0, 12.6], P=0.002) compared with preoperatively diagnosed patients.ConclusionsPatients diagnosed with PPGLs postoperatively may have no further higher risk of intraoperative hypertension than those diagnosed preoperatively despite insufficient preoperative management for PPGLs. Further study will be needed to ascertain intrinsic tumour characteristics, and need for universal preoperative use of α- and β-blockers in PPGL patients postoperatively diagnosed or without typical symptoms related PPGLs.

Highlights

  • Pheochromocytoma and paraganglioma (PPGL) are rare catecholamine-secreting tumours originating from chromaffin cells of the adrenal medulla and extra-adrenal paraganglia

  • The proportion of pheochromocytoma was lower in postoperatively diagnosed patients than in preoperatively diagnosed patients (17.2% vs. 81.1%, P

  • Preoperative SBP in the ward was similar between groups, but the use of a-blockers and b-blockers was more frequent in preoperatively diagnosed patients to reduce severe hypertension and tachyarrhythmia during the operation

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Summary

Introduction

Pheochromocytoma and paraganglioma (PPGL) are rare catecholamine-secreting tumours originating from chromaffin cells of the adrenal medulla and extra-adrenal paraganglia. Typical presentations that occur due to the release of catecholamines include headache, sweating, palpitation, and hypertension [2]. Excessive catecholamine release from PPGLs can induce life-threatening complications such as myocardial infarction, heart failure, cardiomyopathy, shock, arrhythmias, and stroke [3, 4]. The clinical presentation is highly variable, from completely asymptomatic to life-threatening complications. Some PPGLs are diagnosed postoperatively in pathologic reports. In these cases, the preoperative medical preparation for PPGLs can be insufficient. Despite an improved understanding of pheochromocytoma and extra-adrenal sympathetic parganglioma (PPGL), including diagnosis and management, some PPGLs are postoperatively diagnosed.

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