Primary Extranodal Non‐hodgkin's Lymphoma of the Liver (Pll)

Abstract

EPOCH (infusional dose-adjusted etoposide, doxorubicin and cyclophosphamide with vincristine, prednisone and rituximab).Patients were classified into 3 risk groups (according to presence of pleural/pericardial effusion and IPI high/intermediate-risk or high-risk).Residual disease (RD) was evaluated by FDG-PET and defined as score 2 according to Deauville Criteria and partial response (PR) defined as score 4-5 with a reduction >50% in size of mass.Results: Median age at diagnosis was 29 (21-43) years, age <40 years in 86.7% (n = 13).Eleven patients (78.6%, n = 14) had a bulky disease (tumor mass-10 cm), and 6 (42.9%, n = 14) had superior vena cava syndrome at presentation. Presence of pleural/pericardial effusion in 7 patients (53.8%, n = 13) and pulmonary involvement in 5 (38.5%, n = 13).According to the prognostic score, two patients (15.4%) were classified as high risk (2 adverse factors), five (38.5%) in intermediate risk (1 factor) and six (42.2%) in low risk (0 factors).