A pelvic paraganglioma presenting as a hypertensive emergency in pregnancy : case study

Paraganglia are neuroendocrine organs that originate from the neural crest cells that are closely associated with the autonomic nervous system. Paragangliomas and phaeochromocytomas are tumours that arise from these organs. Paragangliomas are extra-adrenal tumours that can be divided into sympathetic and parasympathetic, and are further categorised by site. The term "phaeochromocytoma" is reserved for intra-adrenal tumours. The most common location of these extra-adrenal paragangliomas is the inferior para-aortic region (also known as the organ of Zuckerkandl). Other extra-adrenal sites that have been described include the bladder, pelvis, prostate, ovaries and thorax. According to McNicol, the estimated incidence of paragangliomas is 1:300 000. At least 30% of paragangliomas have a hereditary occurrence. Approximately 10% are syndromic. We describe a case of extra-adrenal pelvic paraganglioma that caused hypertension in pregnancy.