Temporal Bone Paraganglioma

Abstract

Temporal bone paragangliomas (TBPs) are slow-growing, highly vascular primary neoplasms of the middle ear and lateral skull base that are composed of paraganglion cells derived from the neural crest. Paraganglioma can be found throughout the body, though in the temporal bone, two forms predominate: tympanic paraganglioma (TP) that originate in the middle ear cleft along the tympanic plexus associated with Jacobson’s (IX) and Arnold’s (X) nerves and jugular paraganglioma (JP) derived from chief cells located in the adventitia of the jugular bulb (Moore et al, Otolaryngol – Head Neck Surg: Off J Am Acad Otolaryngol-Head Neck Surg 54(4):597–605, 2016; Carlson et al, Otolaryngol – Head Neck Surg: Off J Am Acad Otolaryngol-Head Neck Surg 152(1):98–105, 2015; Wanna et al, Otolaryngol – Head Neck Surg: Off J Am Acad Otolaryngol-Head Neck Surg 151(6):991–995, 2014; Langerman et al, Arch Otolaryngol – Head Neck Surg 138(4):341–345, 2012; Jackson et al, Arch Otolaryngol;108(7):401–410, 1982). Most TBP are solitary, non-biochemically secreting and benign, while less than 5% are associated with catecholamine release or malignant character; up to 17% of patients display multiple paragangliomas (Jackson et al, Am J Surg 59(4):389–393, 1990). TBP exhibit a female predominance and most commonly manifest during the fourth to fifth decades of life with symptoms of unilateral pulsatile tinnitus, conductive hearing loss, and less commonly lower cranial neuropathy. Owing to the rare prevalence and typical insidious growth, many patients endorse a long duration of symptoms at diagnosis (Moore et al, Otolaryngol – Head Neck Surg: Off J Am Acad Otolaryngol-Head Neck Surg 54(4):597–605, 2016; Carlson et al, Otolaryngol – Head Neck Surg: Off J Am Acad Otolaryngol-Head Neck Surg 152(1):98–105, 2015).