A rare pheochromocytoma complicated by cardiogenic shock and posterior reversible encephalopathy syndrome: case report

Abstract

Background: Pheochromocytoma is a rare catecholamine-producing tumour that classically displays clinical manifestations related to alpha-adrenergic stimulation, including paroxysmal or sustained hypertension. However, it may occasionally be complicated by life-threatening crisis, leading to refractory acute heart dysfunction in the most severe cases.

Case summary: A 28-year-old woman was admitted to intensive care unit due to hypertensive crisis causing pulmonary oedema, Takotsubo cardiomyopathy, and metabolic acidosis. Due to cardiogenic shock, she required venoarterial extracorporeal membrane oxygenation and IMPELLA implantation. A computed tomography scan revealed a 5 cm tumour of the left adrenal gland compatible with pheochromocytoma The clinical course was complicated by acute kidney injury requiring renal replacement therapy and posterior reversible encephalopathy syndrome (PRES). Pharmacological treatment with alpha lityc agents (including urapidil, dexmedetomidine, and doxazosin at maximum daily dose) and beta blockers, together with left videolaparoscopic adrenalectomy, led to progressive blood pressure control and resolution of the neurological symptoms.

Discussion: Pheochromocytoma crisis turned into a potential catastrophic scenario, characterized by refractory cardiogenic shock requiring circulatory supportive devices and PRES. Alpha-antagonists and beta-blockers were the gold standard pharmacological treatment. A multidisciplinary decision-algorithm was necessary to successfully manage this complex clinical setting.

Keywords: Alpha lityc treatment; Cardiogenic shock; Case report; Pheocromocytoma; Posterior reversible encephalopathy syndrome.

Figure 1

Chest X-ray showing bilateral symmetrical and diffuse alveolar opacities.

Figure 2

Cerebral computed tomography scan showing hypodense (ischaemic) bilateral parieto-occipital areas and a hypodense subcortical area in the left frontal lobe.

Figure 3

Abdominal computed tomography scan revealing a 5 cm tumour of the left adrenal gland compatible with pheochromocytoma.

Figure 4

Magnetic resonance imaging (T1 and T2 sequences; diffusion MR study) showing bilateral parieto-occipital ischaemic lesions of recent onset. Ischaemic lesions of the right cerebellum, right centrum semiovale and left frontal subcortical region can also be observed. Vasogenic oedema accompanies these lesions. These radiological findings, together with a right hemiplegia of new onset, are suggestive of posterior reversible encephalopathy syndrome (PRES).