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Lymphoma and the Nervous System Fred H. Hochberg MD

This article provides information on the epidemiology, etiology, pathogenesis, clinical presentation, diagnosis, and imaging of lymphoma in the nervous system. It also discusses the risks and factors associated with CNS relapse, as well as the differential diagnosis and pitfalls in imaging.

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Lymphoma and the Nervous System Fred H. Hochberg MD

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  1. Lymphoma and the Nervous SystemFred H. Hochberg MD

  2. Epidemiology • 1-2% of brain tumors. • 3000 in U.S. per year. • Incidence in AIDS patients is 1.9% to 6%. • Peak in immunocompetent patients is 6th decade; immunosuppressed 3 rd decade • Slight male preponderance: 1.7:1 • THE ONE CURABLE BRAIN TUMOR • ALWAYS A DETECTIVE STORY

  3. Epidemiology-Incidence • SEER: 1973-1997 • CT (1973-1984) vs MRI (1985-1997) • incidence PCNSL increased all registries • Incidence (0.15 to 0.48) outpaced systemic lymphoma (14.1 to 18.5) • Rate of increase slowed p.1985 Olson et al. (Mayo Clinic). Cancer 95: 1504 (2002)

  4. Reduction of NHL (events/100 PYF) among HIV-infected patients from 1995 to 2000.  The EuroSIDA study.

  5. Etiology • Polyclonal B cells clone w. CNS ‘addressins’ • B-cell clone targets a CNS superantigen Lyme, MS. • Virus (neurotropic virus (HTLV1), Herpes virus in Marek’s disease) • EBV: transplant, AIDS

  6. Autoimmune Disease and Pharmacologic Immunosuppression • lymphoma link to Sjogren’s, RA, SLE, sprue • lymphoma risk reflects underlying disease and immunosuppressive (anti-TNF therapy (etanercept, infliximab); methotrexate, azathioprine, cyclophosphamide, chlorambucil) Pathogenesis PCNSL and Infection Chronic antigenic Stimulation

  7. PCNSL in Acquired Immunosuppression Clinical Presentation, Diagnosis

  8. The 3 B cell rearrangements Kuppers. Nat Rev Can, 2005

  9. PCNSL Angiotropic GenesRubenstein 2006

  10. IgH studies- JCO 2006 Thiel

  11. Histopathology Diagnosis HE Ki-67 CD20 k light chain

  12. Histopathology Diagnosis CD79a HE HE CD20

  13. SYSTEMIC NHL • Epidural compression • Peripheral neuropathy • Motor neuronopathy • Paraneoplastic syndromes (PCD) • Opportunistic infections • Chemotherapy- Leukoencephalopathy • Waldenstrom’s neuropathy and BN

  14. Epidural Lymphoma • 60 year old woman with “disc” at C5-6. Progressive arm weakness then quadriparesis. Biopsy revealed lymphoma.

  15. A Baseball Player with A Lump on the Head • 27 with 4 weeks of unsteady gait, leg weakness, bladder change, OU changes • Right occipitoparietal mass effacing sulci • Marrow with features of Burkitt

  16. Systemic to Brain Plasma cell Myeloma Testis to Brain

  17. Risk Factor Summary • Overall risk of CNS relapse is ~ 5% • Multivariate risk factors: LDH, > 1 extranodal site, Age, Low albumin, IPI Retroperitoneal lymphadenopathy • 10-15% of patients with DLBCL have a > 15% risk of CNS relapse and death

  18. Hollender Model of CNS Risk Hollender Ann Onc ‘02

  19. Risks by factor over time Hollender Ann Onc ‘02

  20. 18FDG-PET staging of PCNSL (Mohile et al., 2008) • CT + BM identifies 4% are actually systemic NHL • PET is sensitive for systemic lymphoma • No correlation between SUV & malignancy

  21. Testicular lymphoma mandates CNS prophylaxis N = 373 56 CNS relapses Zucca JCO ‘03

  22. CNS relapse is primarily meningeal

  23. CNS relapse is rapidly fatal

  24. PCNSL Presentation • Parenchymal: Personality, ICP, Focal signs • with rare seizures • Neurolymphoma • Angiocentric • Meningeal NHL- CN • Vitreal lymphoma

  25. Parenchymal Disease Clinical Presentation

  26. Other Imaging Characteristics Diagnosis Parenchymal Disease

  27. Parenchymal Disease Clinical Presentation • Parenchymal (CNS, spinal cord or retina) • Leptomeningeal • Peripheral nervous systeminfiltrative/intravascular

  28. Scan Presentation

  29. Diagnosis MRI DWI, ADC, EXP

  30. PET Schlegel 2005

  31. 18FDG-PET in staging evaluation of PCNSL (Mohile et al., 2008) • conventional staging (CT CAT + BM biopsy): identifies 4% of secondary systemic NHL • injection w/ 12-16.5 mCi of FDG • PET may be more sensitive in detecting systemic lymphoma, especially if outside of torso • NB: wide range of SUV (3.8 – 30.5) but no definite correlation between SUV & degree of malignancy • consider additional use of other radiotracers (e.g. 18F-fluorothymidine: identifier of disease foci, better predictor of higher tumor grade)

  32. Imaging Pitfalls AIDS PCNSL after steroids Diagnosis PTLD Non-enhancing PCNSL

  33. Differential Diagnosis Lyme disease and lymphoma

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