Extra-adrenal paraganglioma demographic data: A NCDB study.

Abstract

e16293 Background: Extra-adrenal paragangliomas are rare neuroendocrine tumors that form near blood vessels and nerves outside of adrenal glands. Despite their rare occurrence, these tumors have higher rates of malignancy and varying survival rates compared to other paragangliomas. To better understand its epidemiology, there may be clinical value in analyzing the diagnostic trends of these tumors. This study utilized information provided by the National Cancer Database (NCDB) to examine the demographic factors of patient populations diagnosed with extra-adrenal paragangliomas. Methods: A retrospective cohort analysis using the 2004–2020 National Cancer Database (NCDB) included 133 patients with a histologically-confirmed diagnosis of extra-adrenal paraganglioma (ICD-O-3 code 8693). Demographic factors (age, sex, race, Hispanic status, educational attainment, insurance status, facility type, distance from facility, and Charles/Deyo score) were analyzed by descriptive statistics. Incidence trends were interpreted via regression analysis. Results: In the NCDB, 133 patients were identified with a confirmed diagnosis of extra-adrenal paraganglioma between 2004 and 2020. This database suggested a relatively stable number of patients diagnosed per year (R² = 0.022). Men (67%) were more likely to be diagnosed than women (35%), with an average age at diagnosis of 45.7 years (SD = 17.7, range = 11-83). Patients were most commonly non-Hispanic (88%), White (68%), and had a primary site of diagnosis in the retroperitoneum (35%). Treatment was primarily surgical resection (84%) followed by radiation (17%) and chemotherapy (8%). Most patients did not receive palliative care (98%). A majority of patients (77%) had a Charlson-Deyo comorbidity score of 0, with a 2 year survival rate of 92%, a 5 year survival rate of 79%, and a 10 year survival rate of 50%. Most patients were privately insured (65%) and received treatment in an academic research facility (56%). Many individuals lived in large metropolitan counties with a population larger than 1 million (56%), and resided a mean distance of 41.6 miles (SD = 74.4, range = 0.8 - 559.3) from the treatment facility. Conclusions: To the best of our knowledge, this is the first analysis of extra-adrenal paragangliomas utilizing the NCDB, and thus addresses a significant lack of knowledge on the subject. The majority of patients are non-Hispanic White men, with a primary site of diagnosis in the retroperitoneum. This is the first time that the socioeconomic factors of extra-adrenal paraganglioma patients have been described in the literature: patients tend to be privately insured, live in large metropolitan counties, and have 10 survival rate of 50%. Continued research is necessary to gain a deeper insight on the impact of socioeconomic and demographic factors that influence the diagnosis, treatment, and survivability of extra-adrenal paraganglioma patients.