Treatment of Head and Neck Paragangliomas

Kenneth Hu, MD; Mark S. Persky, MD

Disclosures

Cancer Control. 2016;23(3):228-241. 

In This Article

Multidisciplinary Treatment Algorithms

The management of paragangliomas is challenging given the complexity of possible presentations, the availability of multiple treatment options, and the nature of these tumors to indolently grow but with the potential to severely impact functioning of the head and neck.

Observation without treatment is an appropriate initial option in patients who are asymptomatic, particularly among older individuals with comorbidities who are willing to undergo watchful surveillance. Some authors indicate that paragangliomas can remain stable for long periods of time and, if growth is detected, then the rate of expansion is generally slow and more sensitive imaging techniques can detect and accurately follow the progress of these tumors.[92] In an observational study of 47 tumors followed for 5 years, the researchers found that 38% progressed at an annual growth of 2 mm while 42% were stable and 20% decreased in size.[92] Others have reported a higher rate of progression (≤ 60%) and an annual average growth rate of 1 mm, with variable doubling times between 6 months and 21 years.[93] Whether observation for all asymptomatic patients is the best initial strategy remains to be determined. Moreover, additional research is needed to identify the following:

  • Optimal surveillance strategies across a long time period

  • Thresholds for active treatment with regard to symptom onset or changes in tumor size that impact morbidity

  • Reversibility of symptoms that develop from active surveillance with subsequent treatment

If treatment is to be pursued, then we propose multidisciplinary algorithms that take into account multiple factors, such as type of paraganglioma, malignant status, whether the tumor is catecholamine-secreting, presence of synchronous tumors, and extent of pre-treatment cranial neuropathy (Figs 6 and 7).[1–3] Patient factors include age, presence of comorbidities, potential for rehabilitation, lifestyle, and wishes. The focus of the treatment algorithms is unimodality treatment to control the tumor, minimize iatrogenic morbidity, and preserve cranial nerve function as optimally as possible.

Figure 6.

Algorithm for the treatment of CBTs. CBT = carotid body tumor, RT = radiotherapy.

Figure 7.

Algorithm for the treatment of vagal and jugular paragangliomas. IMRT = intensity-modulated radiotherapy, RT = radiotherapy.

First-line surgery is preferred for patients with carotid body tumors, especially those whose tumors are less than 5 cm in size and also lack carotid artery encasement, because these tumors are usually excised with a low associated risk of carotid artery sacrifice. In cases of bilateral synchronous carotid body tumors, bilateral resection is contraindicated due to baroreflex failure syndrome. Rather, resection of the smaller tumor and radiotherapy to the larger tumor are recommended.

For patients with catecholamine-secreting or malignant tumors, first-line resection is recommended with adjuvant radiotherapy as needed based on pathological factors.

Radiotherapy is the preferred modality for the treatment of jugular and vagal paragangliomas because this therapeutic option offers the opportunity for high rates of local control combined with likely resolution of pre-existing tinnitus and possibly improvement in cranial neuropathy. First-line surgery may be considered for small tympanic tumors and in those with advanced jugular/vagal paragangliomas in which resection would not worsen any pre-existing cranial nerve palsy.

First-line radiotherapy should be considered in patients who are elderly, those with multiple comorbidities, and in those whose tumors have advanced into the skull base or extended into the intracranial space.

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