Incidence, Prognostic Factors and Survival Outcome in Patients With Primary Hepatic Lymphoma.

Shi-Long Zhang,Zhe Guo,Qian-Wen Rao,Li-Shun Wang,Chen Chen,Zhi-Ming Wang,Xin Wang

doi:10.3389/fonc.2020.00750

Abstract

Aim: The objective of our study was to investigate the epidemiologic characteristics, prognostic factors and survival in patients with primary hepatic lymphoma (PHL).Methods: PHL patients diagnosed between 1983 and 2015 were identified from the SEER database. The temporal trend in PHL incidence was assessed using joinpoint regression software. Overall survival(OS) and disease-specific survival (DSS) was evaluated using the Kaplan-Meier method and log-rank test. Univariate and multivariate Cox regression analysis was performed to identify the independent prognostic factors for OS and DSS. Nomograms to predict survival possibilities were constructed based on the identified independent prognostic factors.Results: A total of 1,182 patients were identified with PHL. The mean age was 61.7 ± 17.1 years with a male to female of 1.6:1. Diffuse large B-cell lymphoma (59.8%) was the most common histological subtype. The incidence of PHL steadily increasing by an annual percentage change (APC) of 2.6% (95% CI 2.0–3.2, P < 0.05). The 1-, 5-, and 10-year OS rates were 50.85, 39.6, and 30.4%, respectively, and the corresponding DSS rates were 55.3, 47.9, and 43.3%, respectively. Multivariate Cox regression analysis revealed that age, sex, race, marital status, histological subtype, surgery, and chemotherapy were independent prognostic factors for survival. Nomograms specifically for DLBCL were constructed to predict 1-, 5-, and 10-year OS and DSS possibility, respectively. The concordance index (C-index) and calibration plots showed the established nomograms had robust and accurate performance.Conclusion: PHL were rare but the incidence has been steadily increasing over the past four decades. Survival has improved in recent years. Surgery or chemotherapy could provide better OS and DSS. The established nomograms specifically for DLBCL were robust and accurate in predicting 1-, 5-, and 10-year OS and DSS.

Highlights

Primary hepatic lymphoma (PHL) is a rare malignancy whose pathogenesis is still unclear
To reduce the risk of including secondary liver involvement of terminal systemic or adjacent lymphoma, our study focused on a diagnosis of lymphoma and a primary location confined to the liver with no history of prior tumor diagnosis
The trend in incidence was relatively steady increasing from 1973 to 2015, with an Annual percentage changes (APCs) of 2.6% (Figure 1A)

Summary

Introduction

Primary hepatic lymphoma (PHL) is a rare malignancy whose pathogenesis is still unclear. It accounts for ∼0.1% of hepatic malignant tumors and 0.4% of extranodal lymphoma [1]. It was first described in 1998, and Lei et al [2] defined it as a lymphoproliferative disorder confined to the liver without any involvement of the lymph nodes, spleen, or bone marrow. Most PHL patients present with upper abdominal pain, upper abdominal distention or discomfort. Laboratory tests may reveal either a cholestatic or a cytolytic process, with elevated lactate dehydrogenase and alkaline phosphatase in most patients. PHL is often misdiagnosed as many other liver diseases, and biopsy is usually performed to make a definite diagnosis [2]

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