Anatomic Location is the Primary Determinant of Survival for Paragangliomas

Abstract

Paragangliomas (PGL) are rare neuroendocrine tumors. This study describes the largest collection of PGLs and evaluates factors that impact survival. Patients with PGL from 1998 to 2013 in the NCDB were reviewed. Independent predictors of overall survival (OS) were identified for patients with non-central nervous system (CNS) tumors. Of 867 PGLs, the primary site was CNS (39.9%), abdomen/pelvis (A&P) (21.0%), head and neck (H&N) (17.5%), thoracic (15.1%), bladder (3%), or unspecified (3.5%). Of 521 non-central nervous system (CNS) PGLs, there were differences in sex, comorbidities, treatment facility, tumor size, treatment modality (P < 0.001). Five-year OS for this cohort was 66 per cent and 10-year OS was 51 per cent. Median OS differed significantly between primary sites (H&N 106.0 months, thoracic 89.0 months, A&P 81.7 months, bladder 69.7 months, and unspecified 27.2, P < 0.001). After controlling for multiple factors, age greater than 50 (HR 1.97; CI 1.38–2.81), primary site A&P (HR 2.01; CI) 1.17–3.48) or bladder (HR 4.03; CI 1.64–9.89) as compared with H&N, distant metastasis (HR 2.25; CI 1.44–3.53) and those who did not receive surgery (HR 2.85; CI 1.89–4.31) all exhibited decreased OS. This is the largest series of PGLs and the first to demonstrate significant survival differences based on PGL site. Abdominal/pelvic and bladder PGLs had the lowest survival in addition to patients that did not have a surgical resection, those with distant metastases, and >50 years of age.