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Demographics, Tumor Characteristics, Treatment, and Survival Associated With Signet Ring Cell Carcinoma of the Gallbladder (SRCCG): A Population-Based Retrospective Cohort Study

2018; Lippincott Williams & Wilkins; Volume: 113; Issue: Supplement Linguagem: Inglês

10.14309/00000434-201810001-00096

1572-0241

Andrew Ofosu, Daryl Ramai, Emmanuel Ofori, Denzil Etienne, Krishna Gurram, Douglas G. Adler, Madhavi Reddy,

Gallbladder and Bile Duct Disorders

Introduction: Background: Gallbladder cancer is the most common malignant tumor of the biliary tract representing 80-95% of all biliary tract cancers worldwide. Signet-ring cell carcinoma of the gallbladder (SRCCG) is an extremely aggressive rare histological variant that accounts for 3% of all gallbladder cancers. Given its rarity, limited data exist for this subtype of gallbladder cancer. Aim: The objective of this study was to investigate the incidence, demographics, tumor characteristics, treatment, and survival of patients with signet ring gallbladder cancer. Methods: Data on SRCCG between 2004 and 2014 was extracted from the Surveillance, Epidemiology and End Results (SEER) Registry. The clinical epidemiology of these tumors was analyzed using SEER*Stat. American Joint Committee on Cancer (AJCC) 2004 staging was used. IBM SPSS Statistics was used for Cox proportional hazard regression modeling to determine predictors of survival. Results: A total of 196 patients with SRCCG were identified. Median age at diagnosis was 67 ± 13 years. A majority were females compared to males (77% versus 23%) respectively. Most cases were reported in Caucasians (75.5%). The overall age-adjusted incidence of SRCC between 2004 and 2014 was 0.02 per 100,000 per year. Incidence increased with age, with the highest incidence at 85 years and above. Females had a higher incidence of SRCCG compared to males (0.03 vs. 0.01 per 100,000 per year). Most tumors on presentation were poorly differentiated (71.4%). The most common stage at presentation was Stage 4 (38.2%), followed by Stage 2 (34.7%). One- and 5-year cause-specific survival for SRCCG was 35.9% and 9.1% respectively, with a median survival of 6.4 months. On Cox proportional hazard regression analysis, age (hazard ratio [HR] 1.07, 95% confidence interval [CI] 1.05-1.09, P=0.001), stage 4 cancer (HR 4.17, 95%CI 2.18-7.98, P=0.002), those not treated with surgery (HR 1.18, 95% CI 1.03-3.04, P=0.001), and those who did not receive chemotherapy (HR 2.15, 95% CI 1.36-3.41, P=0.003), had significantly worse outcomes. Radiation therapy alone did not significantly improve outcomes,p=0.590. Conclusion: Conclusions: SRCCG is extremely rare and has a very low survival rate. Its incidence increases with age, however, those treated with surgery and chemotherapy tend to have better outcomes. Further studies are needed to understand the nature of the disease and improve treatment modalities to increase survival.96_A Figure 1. Demographic and Tumor Characteristics.96_B Figure 2. Kaplan-Meier curve for survival analysis