Extensive Central Nervous System Lymphoma Detected at the Time of Diagnosis of Chronic Lymphocytic Leukemia - An Isolated Central Nervous System Richter’s Syndrome: A Case Report

Abstract

When a central nervous system (CNS) lesion is found in patients with chronic lymphocytic leukemia (CLL), we can consider CNS involvement of CLL or transformation to large B-cell lymphoma (Richter's syndrome; RS), or coincidence of other tumor/non-tumorous diseases.CNS RS commonly occurs in preexisting CLL with other nodal/extranodal involvement. It is extremely rare that isolated CNS RS is found concurrently with the initial diagnosis of CLL.A 73-year-old woman presented headache and visited with an elevated white blood cell count and intraventricular masses. Peripheral blood smear and BM biopsy revealed CLL.Brain MRI with gadolinium enhancement showed a suprasellar mass and a pineal mass within the 3rd ventricle, and extensive leptomeningeal enhancements. Whole body FDG PET/CT showed no hot uptake except brain lesions. CSF cytology showed small atypical 4 lymphocytes, suggestive of CLL involvement. But endoscopic biopsy for the 3rd ventricle mass resulted diffuse large B-cell lymphoma. After 3 months of systemic high dose methotrexate, all preexisting lesions disappeared, but new hemorrhagic masses were found in the right lateral ventricle and in the 4th ventricle. She got palliative cranial radiotherapy, but died at 6 months after the initial diagnosis. Exact CNS tissue diagnosis and appropriate treatment should be critical.