Primary central nervous system lymphoma is a rare presentation of lymphoma and is usually seen in adults, often in association with immunodeficiency. Evaluation of these patients classically shows discrete intracranial lesions, and long-term prognosis despite treatment is poor. The case of a child is presented who had no identifiable predisposing factors, no radiologic evidence of intracranial mass, no evidence of systemic disease, and in whom the diagnosis of primary CNS lymphoma was made based on documentation of a monoclonal population of malignant B-cells in the cerebrospinal fluid. The patient, in whom irreversible blindness developed, was treated with a combination of cranial radiation, and intrathecal and systemic chemotherapy, and is currently alive and disease-free 15 months after diagnosis.
Cuncer 569804-2808, 1985.
LTHOUGH IT IS ESSENTIALLY a disease of the lymph
A nodes and bone marrow, non-Hodgkin's lymphoma may spread to the central nervous system (CNS), and secondary meningeal involvement is seen at diagnosis in approximately 5% of pediatric patients.' In contrast, primary lymphoma of the CNS without any evidence of nodal or marrow involvement is extremely rare, and has mainly been reported in adults. where it represents approximately 2% of all cases of malignant lymphoma.2 This presentation is generally accompanied by focal neurologic deficits associated with an intracranial mass detectable by computerized tomography (CT) scan. Recent reports have associated this presentation with immunodeficiency states, both inherited and acquired. A linkage between Epstein-Barr virus infection and primary CNS lymphoma has also been suggested in some cases3
From the Childrens Hospital of Los Angeles,