From the Archives of the AFIP

Abstract

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.

Dumbbell-Shaped Paraganglioma of the Cervical Spine in a Child

“…Extra-adrenal paragangliomas are usually located in the head and neck region, with 90% arising in the carotid body or glomus jugulare [3], though it has been reported in the orbit, paranasal sinuses, larynx, sella turcica, pineal gland, cavernous sinus, thyroid gland, nasopharynx, mandible, soft palate, face, cheek and along the course of the vagus nerve [4]. Spinal involvement is uncommon; a vast majority of these tumors are intradural and found within the cauda equina [3,5,6,7,8,9].…”

“…The most common sites of origin of paragangliomas are the carotid body and glomus jugulare; the tumors arising from the rest of the paraganglion system are uncommon [3, 4]. Paragangliomas involving the spinal canal are rare and mainly take the form of intradural compression of the cauda equina [3,5,6,7,8,9] or intra-/extradural compression of the thoracic cord [10,11,12,13,14].…”

Intracranial Pressure and the Role of the Vagus Nerve: A Hypothesis

“…In the advential layer of the jugular bulb there is a lining of nonchromaffin-staining paraganglian cells that make nerve connections with the glossopharyngeal and vagus nerves [15]. These cells have a well-defined chemoreceptor function [16].…”

Hereditary Paraganglioma/Pheochromocytoma and Inherited Succinate Dehydrogenase Deficiency

“…Before commencing surgery, other sites of PGL or PHEO should be sought by determining 24-hour urinary or plasma metanephrine concentration and radiological methods such as 123 I-metaiodobenzylguanidine (mIBG) scintigraphy and/or octreotide scintigraphy and/or 18 F-DOPA or 18 F-fluorodopamine whole-body positron emission tomography (PET). Finally, angiography is worthwhile to assess the extent of the tumor and for presurgical embolization [19]. In cases of PHEO, surgery must be accompanied by preparatory drug treatment and the assistance of a specialized team of anesthetists [20].…”