%0 Journal Article
%A Kaulen, Leon
%A Hielscher, Thomas
%A Doubrovinskaia, Sofia
%A Hoffmann, Dirk Carsten Frieder
%A Kessler, Tobias
%A Traub, Benjamin-Leon
%A Baehring, Joachim M
%A Wick, Wolfgang
%T Clinical Presentation, Management, and Outcome in Neurolymphomatosis: A Systematic Review.
%J Neurology
%V 103
%N 4
%@ 0028-3878
%C [Erscheinungsort nicht ermittelbar]
%I Ovid
%M DKFZ-2024-01590
%P e209698
%D 2024
%Z #EA:B320#LA:B320#
%X 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
%K Humans
%K Neurolymphomatosis: therapy
%K Neurolymphomatosis: diagnostic imaging
%K Disease Management
%K Prognosis
%F PUB:(DE-HGF)16
%9 Journal Article
%$ pmid:39102613
%R 10.1212/WNL.0000000000209698
%U https://inrepo02.dkfz.de/record/292086