Primary extranodal lymphoma: a report of six cases in a private cancer centre in Nigeria

Abstract

Primary extra-nodal lymphoma (PENL) refers to lymphomas which present with disease in any organ or tissue other than lymph nodes or spleen. Symptoms at initial presentation are mainly from extra nodal involvement and after routine staging procedures, the extra nodal involvement remains the clinically dominant site of disease with little or no nodal disease [1, 2]. About 25–40% of lymphomas arise at extra nodal sites and with clinical presentation similar to solid tumors [3, 4] creating ambiguity in diagnosis and delays in treatment especially in resource poor settings. We report six cases of PENL seen at a private cancer centre in Lagos, Nigeria between 2021 and 2022. The age range was 32–95 years with five females and one male. Two patients were HIV positive. Five patients had initial symptoms of vague abdominal pain with one patient investigated multiple times for peptic ulcer disease. One patient presented with pathological fracture of left femur. Another patient had previously been diagnosed with an inoperable cholangiocarcinoma. All patients had non-Hodgkin’s lymphoma, DLBCL being most common (83.37%). Four patients started chemotherapy. All treated patients showed good outcomes with complete or good partial response highlighting the importance of accurate diagnosis. Initial histological diagnosis in most of the cases did not suggest lymphoma until immunohistochemistry (IHC) results were received. Cancer care in Nigeria is fraught with difficulties obtaining accurate diagnosis due in part to the limited availability of IHC and molecular testing facilities required to characterize the malignant cells leading to delays in treatment and poorer prognosis [6–8].