Neurolymphomatosis: an International Primary CNS Lymphoma Collaborative Group report.

Neurolymphomatosis (NL) is a rare clinical entity. The International Primary CNS Lymphoma Collaborative Group retrospectively analyzed 50 patients assembled from 12 centers in 5 countries over a 16-year period. NL was related to non-Hodgkin lymphoma in 90% and to acute leukemia in 10%. It occurred as the initial manifestation of malignancy in 26% of cases. The affected neural structures included peripheral nerves (60%), spinal nerve roots (48%), cranial nerves (46%), and plexus (40%) with multiple site involvement in 58%. Imaging studies often suggested the diagnosis with 77% positive magnetic resonance imaging, and 84% (16 of 19) positive computed tomography-positron emission tomography studies. Cerebrospinal fluid cytology was positive in 40%, and nerve biopsy confirmed the diagnosis in 23 of 26 (88%). Treatment in 47 patients included systemic chemotherapy (70%), intra-cerebrospinal fluid chemotherapy (49%), and radiotherapy (34%). Response to treatment was observed in 46%. The median overall survival was 10 months, with 12- and 36-month survival proportions of 46% and 24%, respectively. NL is a challenging diagnosis, but contemporary imaging techniques frequently detect the relevant neural invasion. An aggressive multimodality therapy can prevent neurologic deterioration and is associated with a prolonged survival in a subset of patients.

[1]  E. Chiocca,et al.  Reversal of neurological deficit after chemotherapy in BCL-6-positive neurolymphomatosis. Case report. , 2009, Journal of neurosurgery.

[2]  L. Laroche,et al.  Neurolymphomatosis associated with Sézary syndrome. , 2009, Archives of dermatology.

[3]  C. Klein,et al.  T-cell neurolymphomatosis involving cauda equina and sciatic nerves , 2009, Neurology.

[4]  B. Owler,et al.  Isolated Neurolymphomatosis of the Cauda Equina and Filum Terminale: Case Report , 2008, Spine.

[5]  D. Schiff,et al.  Neuroleukemiosis: Case report of leukemic nerve infiltration in acute lymphoblastic leukemia , 2008, Muscle & nerve.

[6]  J. Gomori,et al.  Primary T-cell CNS lymphoma presenting with leptomeningeal spread and neurolymphomatosis , 2008, Journal of Neuro-Oncology.

[7]  J. Pulido,et al.  Histologic findings in T-cell lymphoma infiltration of the optic nerve. , 2008, Ophthalmology.

[8]  M. Lin,et al.  Neurolymphomatosis—diagnosis and assessment of treatment response by FDG PET‐CT , 2008, Hematological oncology.

[9]  K. Wong,et al.  Sacral nerve root neurolymphomatosis diagnosed on FDG-PET/CT and magnetic resonance imaging. , 2008, Clinical nuclear medicine.

[10]  K. Shima,et al.  A linear lesion along the brachial plexus on FDG-PET in neurolymphomatosis. , 2008, Internal medicine.

[11]  T. Hany,et al.  Sciatic nerve neurolymphomatosis - extent and therapy response assessment with PET/CT. , 2007, Clinical nuclear medicine.

[12]  M. Klimpfinger,et al.  Peripheral nerve involvement in lymphoma: the meninges as the crucial barrier between meningoradicular spread and neurolymphomatosis , 2007, Journal of the peripheral nervous system : JPNS.

[13]  Y. Tajima,et al.  Malignant lymphoma originating in the cauda equina mimicking the inflammatory polyradiculoneuropathy. , 2007, Internal medicine.

[14]  T. Klockgether,et al.  High-grade B-cell NHL of the brachial plexus followed by infiltration of the spinal cord , 2007, Journal of Neurology.

[15]  S. Yazawa,et al.  Brachial plexus neurolymphomatosis: a discrepancy between electrophysiological and radiological findings. , 2007, Internal medicine.

[16]  M. J. van den Bent,et al.  Diagnosis of neurolymphomatosis with FDG PET , 2006, Neurology.

[17]  N. Bulakbaşı,et al.  Detection of widespread neurolymphomatosis with 18F-FDG PET , 2006, European Journal of Nuclear Medicine and Molecular Imaging.

[18]  S. Koh,et al.  A case of neurolymphomatosis involving cranial nerves: MRI and fusion PET-CT findings , 2006, Journal of Neuro-Oncology.

[19]  S. Terahata,et al.  Thickening of Multiple Cranial Nerves in a Patient with Extranodal Peripheral T‐cell Lymphoma , 2006, Journal of neuroimaging : official journal of the American Society of Neuroimaging.

[20]  A. C. Iplikcioglu,et al.  Primary lymphoma of the trigeminal nerve , 2006, British journal of neurosurgery.

[21]  T. Siegal,et al.  Temozolomide treatment for newly diagnosed anaplastic oligodendrogliomas: a clinical efficacy trial , 2006, Journal of Neuro-Oncology.

[22]  M. Tatagiba,et al.  Primary non-Hodgkin lymphoma of the cranial nerves mimicking neurofibromatosis Type 2. Case illustration. , 2005, Journal of neurosurgery.

[23]  U. Metser,et al.  PET‐CT imaging of combined brachial and lumbosacral neurolymphomatosis , 2005, European journal of haematology.

[24]  K. Bulsara,et al.  Malignant lymphoma of the trigeminal region. Case illustration , 2005, Journal of Neuro-Oncology.

[25]  U. Metser,et al.  Assessment of neurolymphomatosis by brachial plexus biopsy and PET/CT. Report of a case , 2005, Journal of Neuro-Oncology.

[26]  E. Dakwar,et al.  Sciatic neurolymphomatosis , 2004, Neurology.

[27]  C. Marty‐Double,et al.  Sensory Neuropathy as Revealing Symptom of Neurolymphomatosis: Report of a Case with a 15-Year Duration , 2004, European Neurology.

[28]  B. O'neill,et al.  High‐dose intravenous methotrexate followed by autologous stem cell transplantation as a potentially effective therapy for neurolymphomatosis , 2004, Cancer.

[29]  T. Yoshimine,et al.  Primary malignant lymphoma of the trigeminal region treated with rapid infusion of high-dose MTX and radiation: case report and review of the literature. , 2003, Surgical neurology.

[30]  A. Erden,et al.  Trigeminal nerve involvement in T-cell acute lymphoblastic leukemia: value of MR imaging. , 2002, European journal of radiology.

[31]  H. Ohta A case of non-Hodgkin’s lymphoma infiltrating the brachial plexus detected by Ga-67 scintigraphy , 2002, Annals of nuclear medicine.

[32]  T. Hany,et al.  Fusion PET-CT imaging of neurolymphomatosis. , 2002, Annals of oncology : official journal of the European Society for Medical Oncology.

[33]  Z. Odabaşı,et al.  Neurolymphomatosis associated with muscle and cerebral involvement caused by natural killer cell lymphoma: a case report and review of literature , 2001, Journal of the peripheral nervous system : JPNS.

[34]  A. Smith,et al.  Neurolymphomatosis of the lumbar plexus: High-resolution MR neurography findings , 2001, Neurology.

[35]  P. Seror Brachial plexus neoplastic lesions assessed by conduction study of medial antebrachial cutaneous nerve , 2001, Muscle & nerve.

[36]  M. Mottolese,et al.  Brachial plexus neuropathy as unusual onset of diffuse neurolymphomatosis , 2000, Neurological Sciences.

[37]  K. Jellinger,et al.  Human neurolymphomatosis in a patient with chronic lymphatic leukemia. , 1990, Clinical neuropathology.

[38]  R. Kakigi,et al.  Human neurolymphomatosis by adult T‐cell leukemia , 1989, Neurology.

[39]  E. Kaplan,et al.  Nonparametric Estimation from Incomplete Observations , 1958 .