Evolving Clinical Presentation and Assessment of Pheochromocytoma: A Review


Pheochromocytoma (PHEO) is a neuroendocrine lesion in the adrenal medulla composed of chromaffin cells producing excess amount of catecholamines. These tumoral cells have the property to synthesize, metabolize, store, and secrete catecholamines and their metabolites. The clinical symptomatology is derived from the peripheral tissue effect of norepinephrine, epinephrine, and their by-products. Morbidity and mortality is increased due to the delay in the diagnosis and treatment. A high index of suspicion leads to testing for PHEO through biochemical, imaging, and genetic studies. Dilemma in its assessment comes about when the clinical picture is beset by too much catecholamine secretory periodicity, too little catecholamine secretion, in lesions less than 1 cm, in exclusively dopamine-secreting tumors, and in the unavailability of biochemical tests and imaging.

The aim of this review is to focus on the progress in the approach of early diagnosis of pheochromocytoma through improved clinical, biochemical, and imaging modalities. Emphasis is made on the early recognition of evolving clinical presentations, with the introduction of cardiovascular imaging, 2D echocardiogram, and cardiac MRI in the early diagnosis of patients with no risk factors and with equivocal biochemical and imaging results yet present with cardiovascular events. From the data reviewed and presented, several algorithms are proposed by the authors as an easy guide for clinicians in the diagnostic approach of pheochromocytoma.

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