A pelvic paraganglioma presenting as a hypertensive emergency in pregnancy

Abstract

Paraganglia are neuroendocrine organs that originate from the neural crest cells that are closely associated with the autonomic nervous system. 1 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. 1 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). 1 Other extra-adrenal sites that have been described include the bladder, pelvis, prostate, ovaries and thorax. 1 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. 1 We describe a case of extra-adrenal pelvic paraganglioma that caused hypertension in pregnancy.