Precursor B Lymphoblastic Lymphoma Restricted to the Central Nervous System: A Case Report

Precursor B lymphoblastic leukemia/lymphoma, an uncommon hematologic malignancy that typically occurs in children and young adults, usually has a good prognosis. Precursor B lymphoblastic lymphoma typically involves the central nervous system, spleen, liver, and/or gonads, but not the blood or bone marrow. A 26 year‐old woman developed progressive monocular blindness and headache in late 2001. By 2003, she had developed malaise, difficulty sleeping and had gained 60–80 pounds. In 2004, an MRI showed a small area of enhancement in the head of the right caudate. A biopsy revealed diffuse infiltrates of lymphoid cells with irregular nuclear contours, finely dispersed chromatin, and small nucleoli. By immunohistochemistry, the tumor cells were positive for CD20, CD10, and TdT, consistent with a precursor B cell lymphoma. No cytogenetic aberrations or evidence of tumor outside the central nervous system were found. Despite treatment with whole brain radiotherapy and multiple different chemotherapy regimens, the tumor progressed, and she died in 2006. An autopsy revealed precursor B lymphoblastic lymphoma limited to the brain, brain stem, and cerebellum, with no evidence of disease in any other organ. The restriction of this type of lymphoma to the central nervous system and lack of response to therapy are unusual.