Jugular paragangliomas are generally benign slow-growing tumors that can cause pulsatile tinnitus, hearing loss, and cranial nerves neuropathy. Progressive growth can also lead to intracranial extension. Historically, the treatment of choice for these lesions has been gross total resection. However, over the last 15 years, many groups have adopted less invasive management strategies including stereotactic radiation therapy, subtotal resection, and primary observation in order to reduce treatment-associated morbidity. The focus of this article is to review the modern management of jugular paraganglioma, highlighting the evolving treatment paradigm at the Otology Group of Vanderbilt.
Key points
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Jugular paragangliomas are the most common tumors of the jugular foramen.
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The management of jugular paragangliomas is challenging because of their close proximity to cranial nerves (CN) and the internal carotid artery.
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Surgery, radiation, and observation are all viable management options and should be individualized to the patient.
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At the authors’ center, there has been a paradigm shift toward conservatism in selected cases in order to minimize morbidity.
Introduction
Jugular paragangliomas (JPs) are the most common primary neoplasms of the jugular foramen, arising from the paraganglion cells within the adventitia of the jugular bulb. They are slow-growing, highly vascularized tumors that are usually diagnosed during the fourth to fifth decades of life, affecting women 3 times more frequently than men. Although considered histologically benign, the management of jugular paragangliomas is challenging because of their infiltrative nature and close proximity to the facial nerve and lower cranial nerves (CN), carotid canal, posterior fossa meninges, and otic capsule. Historically, gross total microsurgical resection was considered the treatment of choice, offering complete eradication of disease; however, this strategy may cause significant morbidity, even in the hands of experienced surgeons.
In an effort to explore less invasive treatment methods, stereotactic radiosurgery began gaining popularity in the early 1990s, and today has become the primary treatment modality of choice for many centers. The primary benefit of radiation therapy is a lower risk of up-front cranial neuropathy compared with gross total resection; however, tumor control and length of follow-up in these studies are variable.
More recently, observation has been considered for select patients such as those with small tumors and few attributable symptoms, those with multicentric disease and contralateral lower cranial neuropathy, or elderly and infirm patients without brainstem compression. The data concerning observation for JP are scarce, and few centers have looked into the clinical course of untreated JP. Such data are needed in order to compare against outcomes with radiation therapy. For example, if it was demonstrated that a large number of tumors do not grow for extended periods of observation, it could be argued that radiation therapy should be reserved until there is definitive evidence of growth.
The Otology Group of Vanderbilt has over 40 years of experience with JP. In the authors’ practice, most tumors are managed with microsurgery; however, over the last decade, the authors’ group has adopted a less aggressive approach in select patients in order to minimize cranial nerve morbidity. In this article, the authors report their experience managing JP, highlighting the paradigm shift in treatment at the authors’ center.
Introduction
Jugular paragangliomas (JPs) are the most common primary neoplasms of the jugular foramen, arising from the paraganglion cells within the adventitia of the jugular bulb. They are slow-growing, highly vascularized tumors that are usually diagnosed during the fourth to fifth decades of life, affecting women 3 times more frequently than men. Although considered histologically benign, the management of jugular paragangliomas is challenging because of their infiltrative nature and close proximity to the facial nerve and lower cranial nerves (CN), carotid canal, posterior fossa meninges, and otic capsule. Historically, gross total microsurgical resection was considered the treatment of choice, offering complete eradication of disease; however, this strategy may cause significant morbidity, even in the hands of experienced surgeons.
In an effort to explore less invasive treatment methods, stereotactic radiosurgery began gaining popularity in the early 1990s, and today has become the primary treatment modality of choice for many centers. The primary benefit of radiation therapy is a lower risk of up-front cranial neuropathy compared with gross total resection; however, tumor control and length of follow-up in these studies are variable.
More recently, observation has been considered for select patients such as those with small tumors and few attributable symptoms, those with multicentric disease and contralateral lower cranial neuropathy, or elderly and infirm patients without brainstem compression. The data concerning observation for JP are scarce, and few centers have looked into the clinical course of untreated JP. Such data are needed in order to compare against outcomes with radiation therapy. For example, if it was demonstrated that a large number of tumors do not grow for extended periods of observation, it could be argued that radiation therapy should be reserved until there is definitive evidence of growth.
The Otology Group of Vanderbilt has over 40 years of experience with JP. In the authors’ practice, most tumors are managed with microsurgery; however, over the last decade, the authors’ group has adopted a less aggressive approach in select patients in order to minimize cranial nerve morbidity. In this article, the authors report their experience managing JP, highlighting the paradigm shift in treatment at the authors’ center.
Disease presentation
Pulsatile tinnitus is the most common presenting symptom in patients with JP, followed by hearing decline. Hearing loss is usually conductive in nature but can be sensorineural or mixed. Lower cranial neuropathies resulting in dysphagia, hoarseness, shoulder weakness, and tongue hemiparesis are less common and are usually seen with larger tumors that extend through the medial wall of the jugular bulb. Headache and vomiting are usually late signs associated with increased intracranial pressure caused by brainstem compression and fourth ventricle effacement.
A pulsatile red middle ear mass behind an intact tympanic membrane is the most common finding on physical examination ( Fig. 1 ). By definition, a glomus jugulare extends from the jugular bulb and hypotympanum into the middle ear space. Therefore, the middle ear component only represents the tip of the iceberg. Although not universally present, increased canal and tympanic membrane vascularity surrounding the inferiorly based middle red ear mass may result in the characteristic, rising sun appearance. Less commonly, the tympanic portion of the tumor may erupt into the ear canal, resulting in bloody otorrhea.
In contrast to visceral paragangliomas, head and neck paragangliomas are rarely (<4%) secretory. Patients reporting a history of palpitations, sweats, flushing, syncope, hypertension, and headaches should be screened for serum and urine catecholamine levels. If elevated catecholamine levels are found, the patient should undergo further imaging to rule out pheochromocytoma or multicentric paraganglioma disease.
Imaging
A careful review of fine-cut temporal bone computed tomography (CT) and MRI with gadolinium is critical to differentiating jugular foramen tumors. The most common lesions to involve the jugular foramen are JP, meningiomas ( Fig. 2 ), and lower cranial nerve schwannomas ( Fig. 3 ). Metastatic disease and endolymphatic sac tumors may also secondarily involve this region ( Fig. 4 ). On CT and MRI, JPs demonstrate a diffusely infiltrative pattern of disease resulting in bony destruction and early erosion of the jugulo–carotid spine ( Fig. 5 ). Vascular flow voids within the tumor result in a characteristic salt-and-pepper appearance on T1 and T2 weighted MRI, and the tumor avidly enhances with contrast administration ( Fig. 6 ). Although not completely reliable, most JPs demonstrate middle ear extension, while meningiomas and schwannomas rarely do so. In contrast, meningiomas commonly demonstrate dural tails with en plaque growth and are often associated with underlying hyperostosis. Finally, schwannomas often “dumbbell” between the neck and posterior fossa, with a bottle neck at the jugular foramen. CT generally reveals a widened sharply demarcated jugular foramen without bony destruction.
