Abstract
Background:
Primary Hepatobiliary Lymphoma (PHBL) is a rare disease. In review of the literature, there are only few retrospective studies. On prior studies, the median age at presentation was 62 years and was associated with a median overall survival of 162 months. In this study, we sought to characterize standard prognostic factors and their effects on overall survival utilizing the Surveillance, Epidemiology and End Results (SEER) database.
Methods
The Surveillance, Epidemiology and End Results (SEER) database was used to identify patients diagnosed with PHBL between 1973 and 2014. B-Cell and T-Cell lymphomas of all stages were included. Patient characteristics and demographics are summarized. Overall survival (OS) was estimated using the Kaplan-Meier method for all patients and for sub-groups. Comparisons are made between the survival functions between the levels of these categorical measurements using the log-rank test, where we adjust the significance level to account for multiple comparisons using the Bonferroni correction.
Results:
A total of 1751 patients were included. The mean age was 64 years (2-97), with the majority of patients being male (n=1081, 62%) and Caucasian (n=1278, 73%). B-cell Non-Hodgkin's Lymphoma was the predominant lymphoma (n=1297, 75%), followed by Not Otherwise Specified (n=338, 19%), and T-cell NHL and Hodgkin's Lymphoma were nearly identical at n=54 and 50 respectively. Staging at presentation varied with the majority being 1 and 4 (n=703, 40% and n=698, 40%), this was followed by stage 2 (n=196, 11%). The median overall survival time was 16 months (95% CI; 12, 23). By diagnoses, the median survival time (Figure 1) for B-Cell NHL was 23 months (95% CI; 17, 33) and was significantly longer survival than patients with T-Cell NHL (1 month, 95% CI; 0, 8, p=0.0014) and NOS (9 months, 95% CI; 6, 12, p=0.0076) but was not significantly different than patients with Hodgkin's Lymphoma (12 months, 95% CI; 1, 71 p=0.15). By staging, patients with stage 1 (38 months, 95% CI; 24, 52) did not have a statistically significant difference in survival in comparison to stage 2 (15 months, 95% CI; 9, 48, p=0.85) or 3 (10 months, 95% CI; 2, 34, p=0.0173). Results were not statistically significant regarding gender nor ethnicity. On a multivariant analysis, surgery, chemotherapy, or radiation were all associated with better survival when adjusted for patient age, sex, race, disease type and cancer stage. However, there did not appear to be a statistical significance when compared to each other.
Conclusion
PHBL is a rare lymphoma affecting mostly elderly, Caucasian males. B-Cell NHL subtype and stage 1 were associated with a more favorable prognosis. However, compared to DLBCL, PHBL seem to have a poorer outcome. While treatment alone conferred a longer survival, the type of treatment option did not have an overall change to prognosis.
No relevant conflicts of interest to declare.
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