Treatment of Head and Neck Paragangliomas

Kenneth Hu, MD; Mark S. Persky, MD

Disclosures

Cancer Control. 2016;23(3):228-241. 

In This Article

Abstract and Introduction

Abstract

Background: Commonly occurring in the head and neck, paragangliomas are typically benign, highly vascular neoplasms embryologically originating from the extra-adrenal paraganglia of the neural crest. Frequently, these tumors are associated with the vagus, tympanic plexus nerve, the carotid artery, or jugular bulb. Their clinical presentation can vary across a wide spectrum of signs and symptoms.

Methods: We reviewed and compared standard treatment approaches for paragangliomas of the head and neck.

Results: In general, surgery is the first-line choice of therapy for carotid body tumors, whereas radiotherapy is the first-line option for jugular and vagal paragangliomas.

Conclusions: Because of the complexity of clinical scenarios and treatment options for paragangliomas, a multidisciplinary algorithmic approach should be used for treating paragangliomas. The approach should emphasize single-modality treatment that yields excellent rates of tumor control, low rates of severe, iatrogenic morbidity, and the preservation of long-term function in this patient population.

Introduction

Commonly occurring in the head and neck, paragangliomas are typically benign, highly vascular neoplasms embryologically originating from the extra-adrenal paraganglia of the neural crest, and they are typically associated with the vagus, tympanic plexus nerve, the carotid artery, or jugular bulb.[1–3] Clinically, patients with paragangliomas present with diverse signs and symptoms. They may have a family history of paragangliomas and can present with multicentric tumors regardless of sporadic or familial origin.[1–3]

In the era of safe embolization protocols and sophisticated surgical approaches to the skull base, surgery has become the preferred treatment method for paragangliomas.[1–5] Historically, health care professionals relied on radiotherapy to treat debilitated or elderly patients with unresectable, extensive tumors or paragangliomas, but long-term experience and advances in the field of radiation oncology have demonstrated that first-line radiotherapy for the treatment of paragangliomas has low rates of long-term complications and high rates of tumor control.[1–3] Thus, radiotherapy now plays an important role in treatment algorithms.[1–3] In addition, individualized treatment strategies may also include observation.

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