From the archives of the AFIP - Paragangliomas of the head and neck: Radiologic-pathologic correlation

Paragangliomas of the head and neck are ubiquitous in their distribution, o riginating from the paraganglia or glomus cells within the carotid body, va gal nerve, middle ear, jugular foramen, and numerous other locations. The t ypical patient is middle-aged and presents late in the course of the diseas e, with a painless slow-growing mass. Clinical manifestations include hoars eness of voice, lower cranial nerve palsies, pulsatile tinnitus, and other neuro-otologic symptoms. The overall prognosis of patients with a cervical paraganglioma is favorable, whereas its temporal bone counterpart often res ults in recurrence, residual tumor, and neurovascular compromise when in th e advanced stage. Pathologic examination reveals a characteristic biphenoty pic cell line, composed of chief cells and sustentacular cells with a perip heral fibrovascular stromal layer that are organized into a whorled pattern ("zellballen"). Imaging half marks of paragangliomas of the head and neck include an enhancing soft-tissue mass in the carotid space, jugular foramen , or tympanic cavity at computed tomography; a salt-and-pepper appearance a t standard spin-echo magnetic resonance imaging; and an intense blush at an giography. Imaging studies depict the location and extent of tumor involvem ent, help determine the surgical approach, and help predict operative morbi dity and mortality. Surgical treatment is definitive. Radiation treatment i s included as a palliative adjunct for the exceptional paraganglioma not am enable to surgery.