SUN-508 Survival Trends and Prevalence Disparities in Malignant Paraganglioma

Abstract Disclosure: H. Ali: None. S. Bajwa: None. B. Agrawal: None. U. Bajwa: None. A. Imran: None. F. Cavdar: None. K. Pereira: None. K. Desai: None. S. Durugu: None. R. Thirumaran: None. Background: Paragangliomas (PGL) are rare neuroendocrine tumors of autonomic ganglia. Sporadic cases are common in women while hereditary cases are commonly seen with MEN2 A&B, neurofibromatosis type 1 and VHL disease. Metastasis, rather than histology, affirms the diagnosis of malignant paraganglioma. Surgery is the first line of treatment, while metastatic or extensive disease may require radiation or chemotherapy. The purpose of this study is to determine epidemiological trends and factors affecting survival in PGL. Methods: Data was collected from Surveillance, Epidemiology and End Result database Research Plus Data, 17 Registries, Nov 2023 Sub (2000-2021), using the ICD Code 8680/3 for malignant paraganglioma. The analysis was stratified based on age, sex, race, stage, laterality, primary site labelled, median household income inflation adjusted to 2022, various treatment modalities, and analyzed using the Log-Rank test (GraphPad Prism). Results: Total 550 cases of PGL were identified, of which 47.27% were males and 52.73% were females. The median age of diagnosis was 50 years. Racial distribution was observed as: 60.73% Caucasians, 17.09% Blacks, 13.09% Hispanics, 8.18% Asian or Pacific Islanders, and <1% were American Indians/Alaskan natives and unknown race. Overall median of survival (MoS) was 180 months, with 1-year OS of 0.93 (CI 95%, 0.90-0.95), 3-year OS of 0.84 (CI 95%, 0.80-0.87), and 5-year OS of 0.74 (CI 95%, 0.69-0.78). MoS were significant for Age: 0-30 years (undefined), 31-60 years (196), and 60+ years (81) (p <0.0001); Gender: males (129) vs females (188) (p 0.0224); Laterality: right (220), left (174), unknown origin (129) (p 0.0018). Stage based survival analysis showed higher survival for locoregional disease, while lower for distant disease with unknown stage in between (p <0.0001). Comparing survival based on treatment types showed: surgery 215 vs no-surgery 88 (p value <0.0001), chemotherapy 49 vs no chemo 200 (p value <0.0001), and radiotherapy (XRT) 113 vs no XRT 200 (p value 0.0025). Survival analysis was insignificant on the basis of race, primary site, and income. Undefined MoS was likely observed due to insignificant numbers of death in that age category (0-30 yrs) to calculate 50% survival probability. Conclusion: Only 550 cases were noted in a period of 22 years, rendering it one of the rarest encountered malignancies. Malignant paraganglioma had a predilection for female gender, and Caucasians. Better survival outcomes were associated with younger age, female sex, right-sided origin, locoregional disease, and surgical management. Survival was independent of race, primary site, and median household income. Arguably, lower survival observed with chemotherapy/radiotherapy could be secondary to their use in higher tumor burden states w/wo systemic metastasis, leading to lower overall survival in that cohort. Presentation: Sunday, July 13, 2025