The clinical characteristics of patients with normotension in pheochromocytomas and paragangliomas.

PURPOSE

The predominant symptom of pheochromocytomas and paragangliomas (PPGLs) is variable hypertension (HTN); however, not all patients with PPGLs develop HTN. Studies of the clinical characteristics of normotensive patients and which patients are more likely to develop HTN in PPGLs are rare. This study was performed to identify the clinical features to better understand this clinical entity.

METHODS

All consecutive adult patients with PPGLs were retrospectively enrolled from January 2018 to June 2020. The clinical characteristics of the patients were investigated. Multivariate logistic regression analysis was used to identify risk factors and receiver operating characteristic curves were used to evaluate the diagnostic performance of the variables.

RESULTS

There were 203 patients in the analysis, including 115 patients with normotension. Fewer patients with normotensive PPGLs had diabetes (p < 0.001) and clinical symptoms (p < 0.001). The 24-h urinary epinephrine (p = 0.002) and 24-h urinary norepinephrine (24hU-NE) concentrations (p < 0.001) were lower, and tumor diameter (p < 0.001) was smaller in patients with normotensive PPGLs. Multivariate logistic regression analysis showed that diabetes and 24hU-NE concentration were independent and negative risk factors for normotensive PPGLs. The area under the curve of 24hU-NE concentration and diabetes for predicting normotensive PPGLs was 0.788 and 0.634, respectively. Combining diabetes and 24hU-NE concentration into one model, the area under the curve of the model for predicting normotensive PPGLs was 0.817.

CONCLUSIONS

Normal blood pressure could not exclude PPGLs. Identifying the clinical characteristics of normotensive and hypertensive patients in PPGLs is helpful for risk stratification and individualized assessment and treatment.