KEGG   DISEASE: Primary central nervous system lymphoma
Entry
H02424                      Disease                                

Name
Primary central nervous system lymphoma
  Subgroup
Diffuse large B-cell lymphoma
T-cell lymphoma
Burkitt lymphoma [DS:H00008]
Description
Primary central nervous system lymphoma (PCNSL) is an extranodal non-Hodgkin lymphoma (NHL) confined to the brain, leptomeninges, eyes, or spinal cord. Most PCNSLs are diffuse large B-cell lymphoma (DLBCL; 90%), with the remainder consisting of T-cell lymphomas, poorly characterized low-grade lymphomas, or Burkitt lymphomas. The molecular pathogenesis of PCNSL is now better understood. The B-cell receptor (BCR) signaling axis with its downstream target, NF-kappa B, is affected by frequent recurrent mutations, mainly in MYD88, CD79B and, less frequently, CARD11, and TNFAIP3. Recurring chromosomal losses affect the 6q, 6p21.32 (HLA locus) and 9p21 (CDKN2A locus) regions.
Category
Cancer
Brite
Human diseases [BR:br08402]
 Cancers
  Cancers of haematopoietic and lymphoid tissues
   H02424  Primary central nervous system lymphoma
Human diseases in ICD-11 classification [BR:br08403]
 02 Neoplasms
  Neoplasms of haematopoietic or lymphoid tissues
   Mature B-cell neoplasms
    2A81  Diffuse large B-cell lymphomas
     H02424  Primary central nervous system lymphoma
Pathway
hsa04662  B cell receptor signaling pathway
Gene
MYD88 (mutation) [HSA:4615] [KO:K04729]
CD79B (mutation) [HSA:974] [KO:K06507]
CARD11 (mutation) [HSA:84433] [KO:K07367]
TNFAIP3 (mutation) [HSA:7128] [KO:K11859]
HLA-DRB5 (deletion) [HSA:3127] [KO:K06752]
CDKN2A (deletion) [HSA:1029] [KO:K06621]
Other DBs
ICD-11: 2A81.5
Reference
  Authors
Citterio G, Reni M, Gatta G, Ferreri AJM
  Title
Primary central nervous system lymphoma.
  Journal
Crit Rev Oncol Hematol 113:97-110 (2017)
DOI:10.1016/j.critrevonc.2017.03.019
Reference
  Authors
Batchelor TT
  Title
Primary central nervous system lymphoma.
  Journal
Hematology Am Soc Hematol Educ Program 2016:379-385 (2016)
DOI:10.1182/asheducation-2016.1.379
Reference
  Authors
Grommes C, DeAngelis LM
  Title
Primary CNS Lymphoma.
  Journal
J Clin Oncol 35:2410-2418 (2017)
DOI:10.1200/JCO.2017.72.7602
Reference
  Authors
Batchelor TT
  Title
Primary central nervous system lymphoma: A curable disease.
  Journal
Hematol Oncol 37 Suppl 1:15-18 (2019)
DOI:10.1002/hon.2598
Reference
  Authors
Phillips EH, Fox CP, Cwynarski K
  Title
Primary CNS lymphoma.
  Journal
Curr Hematol Malig Rep 9:243-53 (2014)
DOI:10.1007/s11899-014-0217-2

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