Pagetic osteosarcoma: An analysis of the National Cancer Database.
Authors: Quinton Anderson, Yanick Tade, Suraj Puvvadi, Sharon Choe, Anton Sachs, Mahadevan Iyer, Amy Keane, Frances Nowlen, Peter T. Silberstein, Daniel Lee, Michael Demangone, Anthony Rossi, Craig Heise, Beau Hsia
Journal: Journal of Clinical Oncology (2024)

e23503 Background: Pagetic osteosarcoma (Pagetic OS) is a rare cancer typically originating at bones affected by Paget disease. Pagetic OS may present as metachronous multifocal lesions primarily affecting the pelvis, proximal femur, and proximal humerus. There are currently no available sociodemographic descriptions of patients with Pagetic OS. Analyzing sociodemographic factors through the National Cancer Database may unveil important information about its epidemiology and guide future treatment. Methods: A retrospective cohort analysis utilizing the 2004–2020 National Cancer Database (NCDB) included patients with a histologically-confirmed diagnosis of Pagetic osteosarcoma (N = 128) using ICD-O-3 code 9184. 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 in regression analysis. Results: In the NCDB, 128 patients were identified with a confirmed diagnosis of Pagetic osteosarcoma (Pagetic OS) between 2004 and 2020, with a steady incidence of diagnoses per year (R 2 = 0.0). The average age of diagnosis was 68.9 years (SD = 13.4, range = 11-90 years). Men were more likely to be diagnosed than women (57% and 43%, respectively). Most patients were non-Hispanic White (71%), insured by Medicare (56%), and lived in a metropolitan area (85%). When compared individually, more patients were in the top median household income quartile (31%). The top two primary sites were the long bones of the lower limb including their associated joints (27%) and the pelvic bones excluding the sacrum, coccyx, and symphysis pubis (27%). Within the cohort, 31 patients received chemotherapy as primary treatment (24%) while 28 patients received primary radiation therapy (22%). Most patients (63%) did not undergo primary site surgery while 39 patients (31%) did receive surgery as primary treatment. The 90-day mortality included 15% of patients, who died 90 or fewer days after surgery was performed. The 2-year survival was 82% while the 5-year survival was 53% and the 10-year survival was significantly lower at approximately 11%. Most individuals (76%) had Charlson-Deyo comorbidity scores of 0. Conclusions: To the best of our knowledge, this NCDB analysis on Pagetic osteosarcoma (Pagetic OS) is the first of its kind on this specific subset of osteosarcoma. Previous research suggests the broader osteosarcoma occurs most frequently in adolescents. This data reveals that Pagetic OS is primarily diagnosed later in life (68.9 years), while still affecting a significantly wide age range (range = 11-90 years). Most patients were non-Hispanic White (71.1%), lived in a metropolitan area (85.2%), and belonged to the top median household income quartile (31.3%). Further research could unveil additional guidance on best practice in diagnosing and treating this cancer.