Malignant Head and Neck Paraganglioma: A Comprehensive and Contemporary Database Review of a Rare Entity

There remains an overall paucity of literature regarding malignant head and neck paragangliomas (MHNPs) and optimal treatment strategies. Our study aims to elucidate contemporary trends regarding the incidence, management modalities, and predictors of survival in a rare entity of a malignancy for which there is no well-established treatment guideline.

The Surveillance, Epidemiology, and End Results (SEER) database was queried for all cases of MHNPs from 2000 to 2019.

The age-adjusted incidence of MHNPs has increased 56% from 2000 to 2019 (0.096-0.150 per 1 000 000). One hundred forty-two patients were included for case-listed statistical analysis, with the mean age at diagnosis being 47 years ± 18.4, presence of regional disease in 47% of cases, and distant disease in 53%. Surgery was the most common form of treatment for MHNP (90, 63.4%) followed by radiation (57, 40.1%). Two-year and 5-year disease-specific survival were noted to be 92% and 79%, respectively. Patients treated with surgery had a decreased risk of mortality (0.227 [95% CI: 0.122-0.424], p < 0.001), whereas those with distant disease at diagnosis had an increased risk of mortality (3.47 [95% CI: 1.780-6.800], p < 0.001).

This study represents the largest and most contemporary known cohort of patients with MHNP and provides details regarding the incidence, survival, and treatment strategies for this rare malignancy with little known treatment data. There was a statistically significant benefit for surgical management of MHNP. Distant metastasis at diagnosis was an independent predictor of increased mortality risk.