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@ARTICLE{Kaulen:292086,
      author       = {L. Kaulen$^*$ and T. Hielscher$^*$ and S. Doubrovinskaia
                      and D. C. F. Hoffmann$^*$ and T. Kessler$^*$ and B.-L. Traub
                      and J. M. Baehring and W. Wick$^*$},
      title        = {{C}linical {P}resentation, {M}anagement, and {O}utcome in
                      {N}eurolymphomatosis: {A} {S}ystematic {R}eview.},
      journal      = {Neurology},
      volume       = {103},
      number       = {4},
      issn         = {0028-3878},
      address      = {[Erscheinungsort nicht ermittelbar]},
      publisher    = {Ovid},
      reportid     = {DKFZ-2024-01590},
      pages        = {e209698},
      year         = {2024},
      note         = {#EA:B320#LA:B320#},
      abstract     = {Neurolymphomatosis (NL) refers to lymphomatous infiltration
                      of the peripheral nervous system (PNS). NL diagnosis and
                      treatment are challenging given the broad differential
                      diagnosis of peripheral neuropathy, the lack of larger
                      cohorts, and the subsequent unavailability of prognostic
                      factors or consensus therapy. This study aimed to define
                      characteristics and prognostic factors of NL.A systematic
                      review of the literature (2004-2023) was performed using
                      PubMed and Scopus databases and reported following PRISMA
                      guidelines. Studies reporting individual patient data on
                      cases with definitive NL diagnosis were included. Clinical,
                      radiologic, pathologic, and outcome information were
                      extracted. Univariable and multivariable survival analyses
                      were performed using log-rank tests and Cox proportional
                      hazard models.A total of 459 NL cases from 264 studies were
                      accumulated. NL was the first manifestation of malignancy
                      (primary NL) in 197 patients. PNS relapse of known
                      non-Hodgkin lymphoma (secondary NL) occurred in 262 cases
                      after a median 12 months. NL predominantly presented with
                      rapidly deteriorating, asymmetric painful polyneuropathy.
                      Infiltrated structures included peripheral nerves $(56\%),$
                      nerve roots $(52\%),$ plexus $(33\%),$ and cranial nerves
                      $(32\%).$ Diagnosis was established at a median of 3 months
                      after symptom onset with substantial delays in primary NL.
                      It mainly relied on PNS biopsy or FDG-PET, which carried
                      high diagnostic yields $(>90\%).$ Postmortem diagnoses were
                      rare $(3\%).$ Most cases were classified as B-cell $(90\%)$
                      lymphomas. Tumor-directed therapy was administered in $96\%$
                      of patients and typically consisted of methotrexate or
                      rituximab-based polychemotherapy. The median overall
                      survival was 18 months. Primary NL without concurrent
                      systemic disease outside the nervous system (hazard ratio
                      [HR]: 0.44; $95\%$ CI 0.25-0.78; p = 0.005), performance
                      status (ECOG <2, HR: 0.30; $95\%$ CI 0.18-0.52; p < 0.0001),
                      and rituximab-based treatment (HR: 0.46; $95\%$ CI
                      0.28-0.73; p = 0.001) were identified as favorable
                      prognostic markers on multivariable analysis when adjusting
                      for clinical and sociodemographic parameters.Advances in
                      neuroimaging modalities, particularly FDG-PET, facilitate NL
                      diagnosis and offer a high diagnostic yield. Yet, diagnostic
                      delays in primary NL remain common. Rituximab-based therapy
                      improves NL outcome. Findings may assist clinicians in early
                      recognition, prognostic stratification, and treatment of
                      NL.},
      keywords     = {Humans / Neurolymphomatosis: therapy / Neurolymphomatosis:
                      diagnostic imaging / Disease Management / Prognosis},
      cin          = {B320 / C060 / HD01},
      ddc          = {610},
      cid          = {I:(DE-He78)B320-20160331 / I:(DE-He78)C060-20160331 /
                      I:(DE-He78)HD01-20160331},
      pnm          = {312 - Funktionelle und strukturelle Genomforschung
                      (POF4-312)},
      pid          = {G:(DE-HGF)POF4-312},
      typ          = {PUB:(DE-HGF)16},
      pubmed       = {pmid:39102613},
      doi          = {10.1212/WNL.0000000000209698},
      url          = {https://inrepo02.dkfz.de/record/292086},
}