Primary liver lymphoma (PLL) with isolated CNS relapse

Background : Primary liver lymphoma (PLL) is a rare disease. Patients usually present with abdominal problems and liver mass without any extrahepatic tumor. PLL usually responds well to chemotherapy, while chemotherapy±radiation is the standard treatment of choice. Case report : A 42 year-old man was presented because of abdominal pain, 8.5kg weight loss and fever for 3 months. There was not lymphadenopathy, splenomegaly, abdominal mass or hepatomegaly in physical examination. A computed tomography (CT) scan of the abdomen disclosed multiple large liver masses without adenopathy. Patient responded well to CHOP chemotherapy regimen. Three months later, he was visited because of headache, dizziness and convulsion. A brain CT scan showed a mass in brain. He was treated by brain radiation plus intrathecal injection of methotrexate (MTX) (15mg) followed by 6 cycles of ICE protocol. Conclusion : PLL is a rare disease that responds well to multi-agent chemotherapy. CNS metastasis should be considered in the affected patients.