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Mohamedraed Elshami*, Alexander W. Loftus, Richard S. Hoehn, John Ammori, Jeffrey Hardacre, J. Eva Selfridge, David Bajor, Amr Mohamed, Sakti Chakrabarti, Amit Mahipal, Jordan M. Winter, Lee M. Ocuin
Department of Surgery, UH Cleveland Medical Center, Cleveland, OH


We examined disparities in providing guideline-compliant care for patients with early-onset versus average-onset biliary tract cancers (BTC) in both localized and metastatic settings. In addition, we examined the association between guideline compliance and overall survival (OS), stratified by age of onset and clinical stage.


Patients with BTC [intrahepatic cholangiocarcinoma, gallbladder adenocarcinoma, extrahepatic cholangiocarcinoma] were identified within the National Cancer Database (2004-2018). Patients <50 years were considered ‘early-onset'; those ≥70 years were considered ‘average-onset.' Guideline-compliant care was defined as surgical resection and chemotherapy ±radiation therapy for localized disease and receiving systemic chemotherapy for metastatic disease.


A total of 49,715 patients diagnosed with BTC were identified, of whom 4,336 patients (8.7%) had early-onset disease; 1,421 (32.8%) had intrahepatic cholangiocarcinoma, 1,079 (24.9%) had gallbladder adenocarcinoma, and 1,836 (42.3%) had extrahepatic cholangiocarcinoma. Early-onset patients were more likely than average-onset patients to have metastatic disease at presentation (34.1% vs. 25.1%).
Throughout the study period, the trend to diagnose early-onset BTC (overall), intrahepatic cholangiocarcinoma, gallbladder adenocarcinoma, and extrahepatic cholangiocarcinoma was unchanged. There was a growing trend to provide guideline-compliant care for early-onset and average-onset patients in both localized and metastatic settings. However, patients with early-onset BTC were more likely than those with average-onset BTC to receive guideline-compliant care in both localized (37.2% vs. 13.1%, OR=4.03, 95% CI: 3.55-4.58) and metastatic (74.1% vs. 41.8%, OR=4.36, 95% CI: 3.75-5.07) settings.
Among patients with localized BTC, guideline compliance was associated with an improvement in OS for average-onset (median OS: 24.8 vs. 8.1 months; HR=0.53, 95% CI: 0.51-0.55; Figure) and early-onset (median OS: 38.1 vs. 18.2 months; HR=0.65, 95% CI: 0.60-0.72) disease. Similarly, among patients with metastatic BTC, guideline compliance was associated with improved OS for average-onset (median OS: 7.4 vs. 1.7 months; HR=0.41, 95% CI: 0.39-0.43) and early-onset (median OS: 9.3 vs. 2.7 months; HR=0.49, 95% CI: 0.40-0.58) disease.


Approximately 9% of patients diagnosed with BTC are <50 years old and are more likely to present with metastatic disease. Patients with early-onset BTC had a higher likelihood of receiving guideline-compliant care in both the localized and metastatic settings, but guideline-compliant care was infrequently administered in the localized setting. Regardless of age of onset and clinical stage, guideline compliance was associated with an improvement in OS.

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