Primary hepatic lymphoma: a retrospective, multicenter Rare Cancer Network study

Abstract

Primary hepatic lymphoma (PHL) is a rare malignancy. We aimed to assess the clinical profile. outcome and prognostic factors in PHL through the Rare Cancer Network (RCN). A retrospective analysis of 41 patients was performed. Median age was 62 years (range. 2386 years) with a male-to-female ratio of 1.9:1.0. Abdominal pain or discomfort was the most common presenting symptom. Regarding B-symptoms, 19.5\% of patients had fever, 17.1\% weight loss, and 9.8\% night sweats. The most common radiological presentation was multiple lesions. Liver function tests were elevated in 56.1\% of patients. The most common histopathological diagnosis was diffuse large B-cell lymphoma (65.9\%). Most of the patients received Chop-like (cyclophosphamide. doxorubicin, vincristine, and prednisone) regimens, 4 patients received radiotherapy (dose range, 30.6-40.0 Gy). Median survival was 163 months, and 5- and 10-year overall survival rates were 77 and 59\%, respectively. The 5- and 10-year disease-free and lymphoma-specific survival rates were 69, 56, 87 and 70\%, respectively. Multivariate analysis revealed that fever. weight loss, and normal hemoglobin level were the independent factors influencing the outcome. In this retrospective multicenter RCN study, patients with PHL had a relatively better prognosis than that reported elsewhere. Multicenter prospective studies are still warranted to establish treatment guidelines, outcome and prognostic factors.