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Neurolymphomatosis: Diagnosis, Treatment, and Outcome

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Tumors of the Central Nervous System, Volume 9

Part of the book series: Tumors of the Central Nervous System ((TCNS,volume 9))

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Abstract

The term neurolymphomatosis (NL) encompasses nerve infiltration by neurotropic neoplastic cells in the setting of an unknown or a known hematologic malignancy. It is a rare neurologic manifestation of non-Hodgkin lymphoma (NHL) and leukemia with a poorly defined incidence. The typical manifestations of NL are of a neuropathy that may affect peripheral nerves, nerve roots, plexus, or cranial nerves. The most common presentations include painful peripheral neuropathy or radiculopathy, cranial neuropathy, painless polyneuropathy and peripheral mononeuropathy or a mononeuropathy multiplex. Successful therapy is contingent upon the recognition of this unique neurological complication, yet the diagnosis is difficult and often elusive. Of all diagnostic tools, imaging studies are of greatest clinical utility. MRI yields abnormal findings in ∼80% of affected patients and FDG-PET appears to be a highly sensitive diagnostic method facilitating identification of NL. There is no known standard treatment for NL and therefore, optimal management is ill-defined. Treatment of NL consists of either chemotherapy alone or combined with radiotherapy. An aggressive multimodality therapy can prevent neurological deterioration and is associated with a prolonged ­survival in a subset of patients.

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Authors and Affiliations

  1. Gaffin Center for Neuro-Oncology and Department of Hematology, Hadassah Hebrew University Medical Center, 91120, Jerusalem, Israel

    Tali Siegal

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  1. Tali Siegal
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Correspondence to Tali Siegal .

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  1. , Kean University, Room 213, Library building, Morris Avenue 1000, Union, 07083, New Jersey, USA

    M.A. Hayat

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Siegal, T. (2012). Neurolymphomatosis: Diagnosis, Treatment, and Outcome. In: Hayat, M. (eds) Tumors of the Central Nervous System, Volume 9. Tumors of the Central Nervous System, vol 9. Springer, Dordrecht. https://doi.org/10.1007/978-94-007-5488-1_1

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  • DOI: https://doi.org/10.1007/978-94-007-5488-1_1

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  • Publisher Name: Springer, Dordrecht

  • Print ISBN: 978-94-007-5487-4

  • Online ISBN: 978-94-007-5488-1

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