Fulminant pheochromocytoma crisis triggered by glucocorticoids: ultra-early adrenalectomy under ECMELLA support

Figures

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  Figure 1. Pathophysiology of acute catecholamine-induced cardiomyopathy (ACIC) in pheochromocytoma. Schematic illustration demonstrating the mechanism of ACIC. The pheochromocytoma releases a massive surge of catecholamines (epinephrine/norepinephrine), following a triggering stimulus (represented in the figure by lightning bolts), leading to supraphysiological stimulation of cardiac adrenergic receptors. This results in intracellular calcium overload, oxidative stress, and severe coronary microvascular spasm, ultimately causing myocardial stunning and the classic apical Takotsubo pattern observed in this case, characterized by mid-apical akinesis and basal hyperkinesis. Created in BioRender. https://BioRender.com/413uen5.

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  Figure 2. Strategic timeline highlighting ultra-early source control. Timeline of major clinical events illustrating the rapidity of the intervention. Day 1: Corticosteroid trigger leads to cardiogenic shock (pH 6.98) and Impella insertion. Day 3: Onset of electrical storm and hyperpyrexia prompts escalation to ECMELLA. Crucially, Rescue Robotic Adrenalectomy is performed just hours later on the same night, breaking the cycle of catecholamine toxicity. POD 5: ECMO decannulation. POD 14: Evidence of myocardial recovery trend. Created in BioRender. https://BioRender.com/413uen5.

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  Figure 3. Preoperative computed tomography (CT). Axial (A) and coronal (B) CT images demonstrating a heterogeneous 5 cm mass in the right adrenal gland, consistent with a pheochromocytoma, prior to the onset of the adrenergic crisis.

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  Figure 4. Multimodal monitoring and mechanical support during rescue surgery. (A) Chest X-ray of the ECMELLA configuration. Shows the Impella catheter tip properly positioned in the left ventricle (arrowhead) crossing the aortic valve, alongside the peripheral VA-ECMO cannulae. (B) Intraoperative Transesophageal Echocardiography (TEE). Mid-esophageal view demonstrating intermittent aortic valve opening (asterisk) despite retrograde ECMO flow, confirming effective LV venting provided by the Impella. (C) Rotational Thromboelastometry (ROTEM) Analysis. Intraoperative trace showing preserved clot firmness (MCF), guiding a strategy of hemostatic stability without heparin reversal. (D) Intraoperative Lactate Trend. Graph illustrating metabolic status during the surgery. Lactate levels were 2.2 mmol/L at the start under ECMELLA support. A transient spike to 4.1 mmol/L occurred during a hypotensive phase caused by tumor manipulation and mild bleeding, which resolved to 2.9 mmol/L by the end of the procedure following resuscitation.

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  Figure 5. Histological and immunohistochemical features of the adrenal pheochromocytoma: hematoxylin–eosin staining (A, B) showing spindle-cell morphology and a solid–trabecular nested growth pattern; immunohistochemical positivity for synaptophysin (C) and chromogranin (D).