Contents

Search

neurolymphomatosis

Etiology: - B-cell non-Hodgkin's lymphoma - diffuse large B-cell lymphoma [3] Epidemiology: - rare Pathology: - lymphomatous infiltration of peripheral nerves - peripheral nerves (60%) - spinal nerve roots (48%) - cranial nerves (46%) - plexus (40%) - multiple site involvement (58%) Clinical manifestations: - progressive peripheral neuropathy (sensory & motor) - numbness, weakness Special laboratory: - nerve biopsy confirms diagnosis (88%) Radiology: - magnetic resonance imaging (77% positive) - PET scan (84% positive) Management: - response to treatment in 46% - systemic chemotherapy (70%) - intrathecal chemotherapy (49%) - radiotherapy (34%) - prognosis - median overall survival 10 months - 12-month survival 46% - 36-month survival 24%

General

lymphoproliferative disorder peripheral nervous system (CNS) disease syndrome

References

  1. Gan HK, Azad A, Cher L, Mitchell PL. Neurolymphomatosis: diagnosis, management, and outcomes in patients treated with rituximab. Neuro Oncol. 2010 Feb;12(2):212-5. Epub 2009 Dec 24. PMID: 20150388 Free PMC Article
  2. Grisariu S, Avni B, Batchelor TT et al Neurolymphomatosis: an International Primary CNS Lymphoma Collaborative Group report. Blood. 2010 Jun 17;115(24):5005-11. Epub 2010 Apr 5. PMID: 20368468 Free PMC Article
  3. David WS, Bowley MP, Mehan WA et al Case 19-2017 - A 53-Year-Old Woman with Leg Numbness and Weakness. N Engl J Med 2017; 376:2471-2481. June 22, 2017 PMID: 28636859 http://www.nejm.org/doi/full/10.1056/NEJMcpc1701762
  4. Rush RP, Saltman AP, Prica AA, Breiner A, Detsky AS. Connecting the Dots. N Engl J Med. 2017 Sep 7;377(10):978-984. PMID: 28877025 http://www.nejm.org/doi/full/10.1056/NEJMcps1613804