Primary anaplastic large-cell lymphoma, ALK1 negative, of the liver: A case report

Abstract Introduction Primary extranodal lymphomas of the liver are notably rare. A proportion of cases are associated with infection with hepatitis C or B, HIV, EBV or primary biliary cirrhosis. We report the case of a 45-year-old man who presented with abdominal pain and weight loss. Case report Physical examination revealed an enlarged liver, but ascites, jaundice, splenomegaly and peripheral lymphadenopathy were absent. Laboratory studies showed elevated hepatic enzymes. Tumour markers CEA and AFP were normal. Abdominal computed tomography revealed multiple hypodense lesions in both lobes of the liver. Liver biopsy examination confirmed a diagnosis of non-Hodgkin’s anaplastic large-cell lymphoma of T phenotype. Immunohistochemically, the neoplastic cells were positive for CD43, CD4, CD2, CD3, CD30, CD5 and granzyme B and negative for CD20, CD79a, CD10, EBV, CD15, CEA, EMA, CD56, CD57 and ALK1. Bone marrow biopsy did not reveal lymphomatous involvement. The patient received combination chemotherapy, and he was alive 2 years after diagnosis. Conclusion Although primary lymphoma of liver is rare compared with secondary hepatic involvement by lymphoma, primary epithelial neoplasms and metastatic carcinoma, the diagnosis should be considered in certain clinical circumstances. The prognosis relates to the specific disease entity.