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Primary leptomeningeal lymphoma

Report of 9 cases, diagnosis with immunocytochemical analysis, and review of the literature

Department of Neurology (Dr. Lachance and O'Neill), the Division of Medical Oncology (Dr. O'Neill), and the Division of Hematology and Internal Medicine (Drs. Witzig and Li), Mayo Clinic and Mayo Foundation, Rochester, MN; the Department of Clinical Neurological Sciences and the Department of Oncology (Dr. Macdonald), London Regional Cancer Centre and the University of Western Ontario, London. ON, Canada, the Department of Neurology (Dr. Jaeckle), University of Utah Medical Center, Salt Lake City, UT and the Department of Neurology (Dr. Posner), Memorial Sloan-Kettering Cancer Center, New York, NY.

We describe 9 patients who presented with a neoplastic meningitis of lymphomatous origin. No evidence of parenchymal central nervous system or systemic tumor was identified either at the time of presentation or throughout the course of their disease. We have chosen to call this entity "primary leptomeningeal lymphoma" (PLML). This unusual form of neurologic lymphoma must be differentiated from the more common clinical situations of primary parenchymal lymphoma with meningeal involvement and systemic lymphoma complicated by lymphomatous meningitis.

Address correspondence and reprint requests to Dr. B.P. O'Neill, Mayo Clinic, 200 First Street SW, Rochester, MN 55905.

Presented in part at the 39th annual meeting of the American Academy of Neurology, New York, NY, April 1987 and published as an abstract in Neurology 1987;37 (suppl 1):304.

Received January 8, 1990. Accepted for publication in final form June 1, 1990.

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