Pheochromocytoma/paraganglioma crisis: case series from a tertiary referral center for pheochromocytomas and paragangliomas

Anouk C Meijs, Marieke Snel, Eleonora P M Corssmit, Anouk C Meijs, Marieke Snel, Eleonora P M Corssmit

Abstract

Pheochromocytoma/paraganglioma (PPGL)-induced catecholamine crisis is a rare endocrine emergency leading to life-threatening hemodynamic instability causing end-organ damage or dysfunction. As it is associated with a significant mortality rate of approximately 15%, recognizing the signs and symptoms and making the appropriate diagnosis are critical. For this purpose, we report the clinical course of the crisis in four out of a total of six patients with a PPGL crisis from a cohort of 199 PPGL patients of a single tertiary referral center for PPGL patients in the Netherlands diagnosed between 2002 and 2020. Successful treatment of a PPGL crisis demands prompt diagnosis, vigorous pharmacological therapy, and emergency tumor removal if the patient continues to deteriorate.

Keywords: Catecholamines; Critical care medicine; Metanephrines; Paraganglioma; Pheochromocytoma.

Conflict of interest statement

The authors declare no conflict of interest.

Figures

Fig. 1
Fig. 1
Abdominal CT scan demonstrating a hypervascular para-aortic mass of 7 cm with central necrosis, located caudal to the left kidney
Fig. 2
Fig. 2
X-ray of the left femur demonstrating destruction of left femoral cortex due to bone metastasis
Fig. 3
Fig. 3
Treatment in patients with PPGL crisis. Type A crisis: hemodynamic instability and end-organ damage in one or more organs. Type B crisis: sustained hypotension, shock, and multiple organ dysfunction (two or more organ systems). Abbreviations: aICU intensive care unit, bIABP intra-aortic balloon pump, cECMO extracorporeal membrane oxygenation

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