Paragangliomas of the Head and Neck, a 10-Year Institutional Review

Background: Paragangliomas of the head and neck represent a rare tumor pathology. As such, controversy exists regarding optimal treatment strategies. Historically a disease managed with surgery, treatment paradigms have shifted in some institutions toward nonoperative management with radiotherapy and even clinical observation. Many patient and tumor factors influence treatment decision making, with tumor growth and the presence of cranial neuropathy representing two of the most salient. The primary objective of this study was to compare tumor growth rates and the incidence of cranial neuropathy development in patients managed with radiation versus those who undergoing observation. Additionally, this study also sought to examine traditional tumor volume estimating methods and compare them with a new technique that uses tumor contouring software.

Methods: A retrospective chart review was performed at a tertiary care referral center, identifying patients with a newly diagnosed head and neck paraganglioma who were treated during a 10-year period (2008–2018). Data analysis included patient demographics, tumor subtype and treatment modality for all identified patients. Special focus was placed on those who underwent management with observation as well with primary radiation treatment. In these patients, further data were collected and compared including tumor growth rates, presentation with or development of cranial neuropathy, requirement of tracheostomy, or gastrostomy tube placement and follow-up intervals. A traditional method for estimating paraganglioma tumor volume (which assumes ellipsoid shape and uses the equation V (volume) = 4/3 π 1/2A × 1/2B × 1/2C) was used to determine tumor size prior to treatment and at the most recent imaging event. Results were used to calculate growth rates and compared for radiated and observed patients. Subgroup growth analysis based on paraganglioma type was also performed. Finally, tumor volume estimates were then compared with those obtained using MIM tumor contouring software.

Results: Of the 176 identified, 89 patients with a total of 90 tumors met inclusion criteria. Fifty (56.2%) patients were treated surgically, 25 (28.1%) were observed, and 14 (15.7%) underwent primary radiation therapy. The averaged tumor growth rate for all paraganglioma types in observed patients was 0.047 cm³ per month. Primarily radiated tumors actually displayed negative growth (shrunk) at a rate of −0.097 cm³ per month; however, this difference did not reach significance. Development of cranial neuropathy following treatment initiation with observation or radiation was rare, occurring in one of 25 observed patients and zero of 14 radiated patients. General covariance between ellipsoid and MIM tumor volume estimation techniques was observed.

Conclusion: Surgical intervention remains the most common form of treatment for head and neck paraganglioma at our institution. Nonsurgical management strategies may be considered for selected paraganglioma patients. Radiation therapy remains an excellent treatment option for some with certain tumor or patient factors. Comparable functional deficits and frequently indolent growth patterns may allow for observational trials in select patients.

Publication History

Article published online:
12 February 2021