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(Radiographics. 1999;19:1605-1632.)
© RSNA, 1999


CONTINUING EDUCATION

From the Archives of the AFIP1

Paragangliomas of the Head and Neck: Radiologic-Pathologic Correlation

Archana B. Rao, MD, Kelly K. Koeller, CDR, MC, USN and Carol F. Adair, LTC, MC, USA

1 From the Departments of Radiologic Pathology (A.B.R., K.K.K.) and Otolaryngic and Endocrine Pathology (C.F.A.), Armed Forces Institute of Pathology, 14th St at Alaska Ave, Bldg 54, Rm M-121 Washington, DC 20306-6000 and the Departments of Radiology and Nuclear Medicine (K.K.K.) and Pathology (C.F.A.), Uniformed Services University of the Health Sciences, Bethesda, Md. Received May 5, 1999; revision requested May 20 and received June 21; accepted June 21. Address reprint requests to K.K.K.

Paragangliomas of the head and neck are ubiquitous in their distribution, originating from the paraganglia or glomus cells within the carotid body, vagal nerve, middle ear, jugular foramen, and numerous other locations. The typical patient is middle-aged and presents late in the course of the disease, with a painless slow-growing mass. Clinical manifestations include hoarseness 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 results in recurrence, residual tumor, and neurovascular compromise when in the advanced stage. Pathologic examination reveals a characteristic biphenotypic cell line, composed of chief cells and sustentacular cells with a peripheral fibrovascular stromal layer that are organized into a whorled pattern ("zellballen"). Imaging hallmarks 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 at standard spin-echo magnetic resonance imaging; and an intense blush at angiography. Imaging studies depict the location and extent of tumor involvement, help determine the surgical approach, and help predict operative morbidity and mortality. Surgical treatment is definitive. Radiation treatment is included as a palliative adjunct for the exceptional paraganglioma not amenable to surgery.

Index Terms: Head and neck neoplasms, 127.3642, 21.369, 276.369 • Paraganglioma, 127.3642, 21.369, 276.369




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