Retroperitoneal Paraganglioma – a rare cause of arterial hypertension

Paragangliomas, as well as pheochromocytomas, are neuroendocrine tumours, which often secrete catecholamines. This hypersecretion causes symptoms that are often difficult to control. Pheochromocytomas originate in the medulla of the adrenal glands and make up 80-85% of cases, whereas paragangliomas originate extra-adrenally and make up 15-20% of cases. Paragangliomas are rare tumours, and although their precise incidence rate is unknown, it is estimated that they occur in 1-2 in 100,000 people and it is believed that many cases go undiagnosed. Up to 25% of paragangliomas are hereditary. The sporadic form is usually diagnosed in patients between the fourth and fifth decades of life, while hereditary forms tend to present at a younger age (before the age of 40). There is no difference in the frequency of incidence between genders. It can occur in any racial group but it is less common amongst black people. Paragangliomas may be derived from the parasympathetic or sympathetic ganglia. Parasympathetic ganglia-derived paragangliomas are located almost exclusively in the base of the neck and the skull, usually arise from the carotid body and jugulotympanic ganglia and are usually non-functional (do not produce catecholamines). In contrast, sympathetic ganglia-derived paragangliomas can arise anywhere along the sympathetic chain from the base of the skull down to the urinary bladder and prostate (with high intra-abdominal prevalence) and in most cases secrete catecholamines. Paragangliomas are a rare, but important cause of hypertension, causing less than 1% of hypertension cases. They can also cause other symptoms associated with a hypermetabolic state and vasoconstriction. Diagnosis is based on the analysis of secreted biochemical products and the evaluation of imaging findings. The only curative treatment is surgery, however, this requires rigorous and long-term