© 2003 by Society
© 2003 by the Society forNeuro-Oncology
Neurolymphomatosis
Brain Tumor Center, Department of Neurology,Massachusetts General Hospital, Boston, MA 02114, USA (J.M.B., D.D.,F.H.H.); Department of Biostatistics, Harvard Schoolof Public Health, Boston, MA 02115, USA (E.C.M., R.A.B.)
2 Address correspondence and reprint requests to Fred H. Hochberg, 100 BlossomStreet, Cox 315, Massachusetts General Hospital, Boston, MA 02114(fhochberg{at}partners.org).
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Abstract |
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The term "neurolymphomatosis" (NL) has included infiltration ofthe peripheral nervous system by lymphoma and nontumor lymphocytes. Wedescribe NL as a lymphoma entity that affects cranial and peripheral nervesand roots. We reviewed the medical records of patients at the MassachusettsGeneral Hospital (MGH) who registered between 1972 and 2000, as well as casespublished in the English-language literature. Inclusion criteria were (A)histopathologic demonstration of lymphoma within peripheral nerve, nerveroot/plexus, or cranial nerve or (B) CT/MRI or intraoperative evidence ofnerve enlargement and/or enhancement beyond the dural sleeve in the setting ofprior or concurrent lymphoma in systemic or CNS sites. We identified 25patients with NL in addition to 47 reported by others. Four clinicalpresentations were (1) painful involvement of nerves or roots, (2) cranialneuropathy with or without pain, (3) painless involvement of peripheralnerves, (4) painful or painless involvement of a single peripheral nerve.Twenty of our patients and 44 of those reported had histopathologicconfirmation of lymphoma infiltrating root or nerve. In the remainder,diagnosis was based upon clinical presentation, nodular nerve enlargement orenhancement, and lymphoma cells in spinal fluid or extraneural sites. Forantemortem diagnosis, imaging studies were of greatest utility, followed bybiopsy. Thirty-three patients of the combined series were not correctlydiagnosed until postmortem examination. Systemic chemotherapy was used toaddress the multiple potential sites of involvement. When properly treated, NLcarries a prognosis similar to primary CNS lymphoma in the modern era.
Received April 29, 2002; Accepted November 12, 2002
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