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‘Case of the month’ from UConn Health, Farmington, CT, USA: management of a giant paraganglioma

Paragangliomas (PGLs) are rare neuroendocrine tumours that are derived from chromaffin cells of the autonomic nervous system. They share a close embryonic origin with phaeochromocytomas (PHEOs), which also arise from chromaffin cells but occur within the adrenal medulla [1]. PGLs can originate in a variety of locations from the neck to the pelvis; however, it is estimated that 70–80% occur within infra-diaphragmatic sympathetic ganglia such as the organ of Zuckerkandl [2]. Approximately 40% of PHEO/PGLs (PPGLs) are associated with familial syndromes including multiple endocrine neoplasia type 2, Von Hippel–Lindau, neurofibromatosis 1, and succinate dehydrogenase mutationrelated tumours [3].