CNS tumor
Lymphoma and other hematopoietic lesions
Diffuse large B cell lymphoma

Author: Nat Pernick, M.D. (see Authors page)

Revised: 8 December 2016, last major update January 2006

Copyright: (c) 2005-2016, PathologyOutlines.com, Inc.

PubMed Search: Diffuse large B cell lymphoma [title] CNS

Cite this page: Diffuse large B cell lymphoma. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/cnstumordiffuselargeBcell.html. Accessed October 23rd, 2017.
Definition / general
  • By definition, primary CNS disease arises exclusively in central nervous system with no obvious lymphoma outside CNS at diagnosis
  • Excludes lymphomas of the dura, intravascular lymphomas and immunodeficiency-associated lymphomas (note: the WHO 2008 classification excludes immunodeficiency associated lymphomas)
  • 5-10% of CNS neoplasms in patients ages 75+ (Hum Pathol 2003;34:1137)
  • 2-12% of AIDS patients at autopsy
  • Usually EBV- in immunocompetent patients vs. EBV+ in AIDS patients
Epidemiology
  • Mean age:
    • 10 years old if inherited immunodeficiency
    • 37 years for post-transplant
    • 39 years for AIDS patients
Prognostic factors
  • Single lesion, no periventricular or meningeal involvement, no immunodeficiency, age < 60 years, Karnofsky score > 70
  • Also negative for p53, c-myc and bcl6 in one study (Arch Pathol Lab Med 2003;127:208)
  • Median survival:
    • 17-45 months if immunocompetent and chemoradiation therapy
    • 13.5 months with AIDS and multimodal therapy
  • Post-transplant:
    • Present nonspecifically, progress rapidly, stereotactic brain biopsy has significant mortality, survival is poor (Neuro Oncol 2000;2:229)
Radiology images
Images hosted on other servers:

Dura-based mass

Case reports
Treatment
  • Steroids cause tumor cell necrosis with foamy histiocytes, so-called "vanishing tumors"
Gross images
Images hosted on PathOut server, courtesy of Dr. Miren Altuna Azkargorta:

Microscopic (histologic) description
  • Dense cellular aggregates of atypical lymphoid cells with dense perivascular aggregates
  • May infiltrate parenchyma diffusely, mix with gliosis and resemble glioma
  • Often smearing of lymphoma cells with needle biopsy
Microscopic (histologic) images
Images hosted on PathOut server, courtesy of Dr. Miren Altuna Azkargorta, Case of the Week #409:

H&E

H&E


Special stains:

CD3

CD5

CD20

EBER-CISH

Ki-67

GFAP



Images hosted on other servers:

Dural mass

Diffuse infiltrates of large malignant cells

Large atypical cells

CD20

Positive stains
Molecular / cytogenetics description
  • Immunoglobulin gene rearrangement
Differential diagnosis
Additional references