Pandora box of Lymphomas in Spleen

Abstract

Introduction: Spleen, a reticuloendothelial organ serves as a clearance site for abnormal cells and plays role in extramedullary hematopoiesis. It can serve as culture medium for the proliferation of hematopoietic cells, leading to development of hematological malignancy. Primary splenic lymphomas are rare. Most of the lymphomas in spleen tend to be infiltration arising elsewhere. Materials and methods: This was a retrospective study. The database of Department of Pathology, AIIMS, New Delhi was searched for splenectomy cases from 2014 to 2019. Cases with incomplete diagnosis were reviewed and detailed evaluation was done. All the lymphoma cases were considered for the study. The demographic data including age, sex and subtype of lymphoma were noted. The cases were divided into pediatric (50 years) groups. Results: A total of 661 splenectomies were performed for benign causes, as part of tumor resections and for the diagnosis of lymphoma. A minor proportion was formed by core biopsies. A total of 27 lymphomas were diagnosed in spleen with 11 considered as infiltration from the lymphoma arising from nodal or other sites such as stomach or colon. Most of the cases were middle age adult population (59.28%) with 60 years (25.92%). There was male predominance with male to female ratio of 1.25:1.The lymphoma subtypes are DLBCL (n=5, 18.5%), small B-cell lymphoma (n=2, 7.4%), mantle cell lymphoma (n=1, 3.7%), Gray zone lymphoma (n=1, 3.7%), Hodgkin lymphoma (n=1, 3.7%) and ALCL (n=1, 3.7%). Rest of the 15 cases had their origin in spleen with major proportion of splenic marginal zone lymphoma (n=11, 40.74%), hepatosplenic gamma-delta T-cell lymphoma (n=1, 3.7%), PTCL (n=2, 7.4%) and villous lymphoma (n=1, 3.7%). Conclusion: Lymphomas in spleen can be infiltrative or a primary, hence proper clinic radiological and pathological evaluation is required. Primary splenic lymphomas are rare and cause various complications. Detailed histomorphology, radiology and immunohistochemical evaluation is required for proper diagnosis and management of the cases.