Primary hepatic lymphoma

Primary hepatic lymphoma (PHL) is a lymphoproliferative disorder confined to the liver without evidence of involvement of spleen, lymph nodes, bone marrow or other lymphoid structures. This is in contrast to Non Hodgkin's Lymphoma (NHL) that often involves the liver as a secondary manifestation. PHL is a rare disease and constitutes 0.016% of all cases of NHL. PHL typically occurs in middle aged men, and usually the chief presenting symptoms are non specific which includes right upper quadrant pain, B symptoms like fever and weight loss and constitutional symptoms. Most frequent physical finding is hepatomegaly which occurs in 75% of patients. Jaundice is rare and present only in less than 5% of patients. Majority of PHL originates from B cells. The blood investigations and imaging findings are nonspecific. Histopathology is essential and confirms the diagnosis. Treatment modalities include combination of surgical resection, chemotherapy and radiotherapy. The prognosis without therapy is grim. The prognosis and management of PHL is different from hepatocellular carcinoma or metastatic disease, hence it is essential to differentiate it from these diseases. The purpose of this review is to emphasize the importance of accurate diagnosis before implementing therapeutic plan for any hepatic space occupying lesion in liver.