KEGG   DISEASE: Burkitt lymphoma
Entry
H00008                      Disease                                

Name
Burkitt lymphoma
  Supergrp
Non-Hodgkin lymphoma [DS:H02418]
Primary central nervous system lymphoma [DS:H02424]
Description
Burkitt lymphoma (BL) is a highly aggressive mature B-cell non-Hodgkin's lymphoma consisting of endemic, sporadic, and immunodeficiency-associated variants. Endemic BL (eBL) affects children and young adults in Africa and some other geographical areas and carries Epstein-Barr virus (EBV) in more than 95% of cases. In contrast, sporadic BL (sBL) among adolescents in Europe and North America are mostly EBV-negative. A third type of BL is associated with HIV-infection in adults. All of these subtypes possess chromosomal rearrangements of the c-myc oncogene, the genetic hallmark of BL that contributes to lymphomagenesis through alterations in cell cycle regulation, cellular differentiation, apoptosis, cellular adhesion, and metabolism. Many BL carry point mutation in the p53 tumor suppressor gene or other defects in the p14ARF-MDM2-p53 pathway, and inactivation of the p16INK4a gene by promoter methylation or homozygous deletion. This indicates that disruption of both the pRb and p53 tumor suppressor pathways is critical for BL development.
Category
Cancer
Brite
Human diseases [BR:br08402]
 Cancers
  Cancers of haematopoietic and lymphoid tissues
   H00008  Burkitt lymphoma
Human diseases in ICD-11 classification [BR:br08403]
 02 Neoplasms
  Neoplasms of haematopoietic or lymphoid tissues
   Mature B-cell neoplasms
    2A85  Other specified mature B-cell neoplasms or lymphoma
     H00008  Burkitt lymphoma
Tumor markers [br08442.html]
 H00008
Pathway
hsa05203  Viral carcinogenesis
hsa05169  Epstein-Barr virus infection
Network
nt06114  PI3K signaling (viruses)
nt06119  JAK-STAT signaling (viruses)
nt06120  Calcium signaling (viruses)
nt06121  TLR signaling (viruses and bacteria)
nt06122  IFN signaling (viruses)
nt06123  TNF signaling (viruses and bacteria)
nt06129  MHC presentation (viruses)
nt06130  Cell cycle (viruses)
nt06131  Apoptosis (viruses and bacteria)
nt06133  RIG-I signaling (viruses)
nt06150  Cytokine-cytokine receptor interaction (viruses)
nt06165  Epstein-Barr virus (EBV)
  Element
N00223  EBV EBNA1 to p53-mediated transcription
N00224  EBV EBNALP RBP-Jk-mediated transcription
N00225  EBV EBNA2 to RBP-Jk-mediated transcription
N00226  EBV EBNA3A/3B/3C to RBP-Jk-mediated transcription
N00262  EBV EBNA3C to intrinsic apoptotic pathway
N00263  EBV EBNA3C to p53-mediated transcription
N00264  EBV EBNA3C to p27-Cell cycle G1/S
N00265  EBV LMP1 to NFKB signaling pathway
N00266  EBV LMP2A to PI3K signaling pathway
N00466  EBV BPLF1 to TLR2/4-NFKB signaling pathway
N00467  EBV BPLF1 to TLR2/4-NFKB signaling pathway
N00468  EBV BPLF1 to TLR2/4-NFKB signaling pathway
N00470  EBV BGLF4 to RIG-I-like receptor signaling pathway
N00471  EBV LMP2A/2B to IFN signaling pathway
N00472  EBV LMP1 to IFN signaling pathway
N00473  EBV BGLF4 to IFN signaling pathway
N00474  EBV BHRF1 to intrinsic apoptotic pathway
N00475  EBV BHRF1 to intrinsic apoptotic pathway
N00476  EBV BHRF1 to intrinsic apoptotic pathway
N00477  EBV BHRF1 to crosstalk between extrinsic and intrinsic apoptotic pathways
N00478  EBV BARF1 to intrinsic apoptotic pathway
N00479  EBV BNLF2a to antigen processing and presentation by MHC class I molecules
N00480  EBV BILF1 to antigen processing and presentation by MHC class I molecules
N00481  EBV BZLF1 to p53-mediated transcription
N00482  EBV EBNA3C to p27-Cell cycle G1/S
N00483  EBV EBNA3C to cell cycle G1/S
N00484  EBV EBNA3C to cell cycle G1/S
N00485  EBV LMP1 to PI3K signaling pathway
N00486  EBV LMP1 to Jak-STAT signaling pathway
N00488  EBV LMP2A to BCR signaling pathway
N01265  EBV vIL10 to IL10R signaling
N01281  EBV BARF1 to CSF1R sigaling
Gene
MYC-IgH (translocation) [HSA:4609] [KO:K04377]
p53 (mutation) [HSA:7157] [KO:K04451]
p16/INK4A (promoter methylation) [HSA:1029] [KO:K06621]
Carcinogen
Azathioprine [CPD:C06837]
Ethylene oxide [CPD:C06548]
2,3,7,8-Tetrachlorodibenzo-para-dioxin [CPD:C07557]
Pathogen
Human herpesvirus 4 (Epstein-Barr virus) [GN:T40079]
Human immunodeficiency virus 1 (HIV-1) [GN:T40001]
Drug
Cyclophosphamide [DR:D00287]
Comment
ICD-O: 9687/3, Tumor type: Burkitt lymphoma
Although the carcinogens listed and infection with HIV-1 are risk factors for non-Hodgkin lymphoma in general, the strength of association with different histological tumor types is not well understood.
Other DBs
ICD-11: 2A85.6
ICD-10: C83.7
MeSH: D002051
Reference
PMID:12191637 (gene, carcinogen, tumor type)
  Authors
Lindstrom MS, Wiman KG.
  Title
Role of genetic and epigenetic changes in Burkitt lymphoma.
  Journal
Semin Cancer Biol 12:381-7 (2002)
DOI:10.1016/S1044-579X(02)00058-5
Reference
PMID:15803153 (gene, carcinogen)
  Authors
Kuppers R.
  Title
Mechanisms of B-cell lymphoma pathogenesis.
  Journal
Nat Rev Cancer 5:251-62 (2005)
DOI:10.1038/nrc1589
Reference
PMID:16062089 (carcinogen)
  Authors
Patel P, Moazzam N, Potti A, Mehdi SA.
  Title
Azathioprine induced hodgkin lymphoma: a case report and review of literature.
  Journal
Am J Clin Oncol 28:427-8 (2005)
DOI:10.1097/01.coc.0000168670.28659.8d
Reference
PMID:15760280 (carcinogen)
  Authors
Schottenfeld D, Beebe-Dimmer JL.
  Title
Advances in cancer epidemiology: understanding causal mechanisms and the evidence for implementing interventions.
  Journal
Annu Rev Public Health 26:37-60 (2005)
DOI:10.1146/annurev.publhealth.26.021304.144402
Reference
PMID:15660110 (carcinogen)
  Authors
Luch A.
  Title
Nature and nurture - lessons from chemical carcinogenesis.
  Journal
Nat Rev Cancer 5:113-25 (2005)
DOI:10.1038/nrc1546
Reference
PMID:12464351 (carcinogen)
  Authors
Kolman A, Chovanec M, Osterman-Golkar S.
  Title
Genotoxic effects of ethylene oxide, propylene oxide and epichlorohydrin in humans: update review (1990-2001).
  Journal
Mutat Res 512:173-94 (2002)
DOI:10.1016/S1383-5742(02)00067-4
Reference
PMID:17530489 (carcinogen)
  Authors
Abnet CC.
  Title
Carcinogenic food contaminants.
  Journal
Cancer Invest 25:189-96 (2007)
DOI:10.1080/07357900701208733
LinkDB

» Japanese version

KEGG   DISEASE: Hodgkin lymphoma
Entry
H00007                      Disease                                

Name
Hodgkin lymphoma
Description
Hodgkin's lymphoma (HL) is one of the most frequent lymphomas in the Western world and often affects young adults. HL is subdivided into classical and nodular lymphocyte-predominant forms. About 95% of cases are classical HL, and 5% are nodular lymphocyte-predominant HL (NLPHL). A characteristic feature of HL is the rareness of the tumor cells, which are called Hodgkin's and Reed/Sternberg (HRS) cells in classical HL and lymphocytic and histiocytic (L&H) cells in NLPHL. These cells represent only about 1% of the cellular infiltrate, while the vast majority of infiltrating cells are T lymphocytes, histiocytes, eosinophilic granulocytes and plasma cells. HRS cells show constitutive activity of both the classical and alternative NF-{kappa}B signalling pathways, which is probably a major pathogenetic mechanism in Hodgkin's lymphoma. The NF-{kappa}B activity in HRS cells is probably mediated by diverse mechanisms: receptor signalling through CD40, RANK, BCMA, and TACI, genomic REL amplification, destructive mutations in IKBA and IKBE. In HL pathogenesis associated with Epstein-Barr virus infection, the activation of NF-{kappa}B is induced by viral latent membrane protein 1 (LMP1).
Category
Cancer
Brite
Human diseases [BR:br08402]
 Cancers
  Cancers of haematopoietic and lymphoid tissues
   H00007  Hodgkin lymphoma
Human diseases in ICD-11 classification [BR:br08403]
 02 Neoplasms
  Neoplasms of haematopoietic or lymphoid tissues
   2B30  Hodgkin lymphoma
    H00007  Hodgkin lymphoma
Pathway
hsa05202  Transcriptional misregulation in cancer
hsa05203  Viral carcinogenesis
hsa05169  Epstein-Barr virus infection
Network
  Element
N00123  Amplified REL to transcription
N00223  EBV EBNA1 to p53-mediated transcription
N00224  EBV EBNALP RBP-Jk-mediated transcription
N00225  EBV EBNA2 to RBP-Jk-mediated transcription
N00226  EBV EBNA3A/3B/3C to RBP-Jk-mediated transcription
N00262  EBV EBNA3C to intrinsic apoptotic pathway
N00263  EBV EBNA3C to p53-mediated transcription
N00264  EBV EBNA3C to p27-Cell cycle G1/S
N00265  EBV LMP1 to NFKB signaling pathway
N00266  EBV LMP2A to PI3K signaling pathway
N00466  EBV BPLF1 to TLR2/4-NFKB signaling pathway
N00467  EBV BPLF1 to TLR2/4-NFKB signaling pathway
N00468  EBV BPLF1 to TLR2/4-NFKB signaling pathway
N00470  EBV BGLF4 to RIG-I-like receptor signaling pathway
N00471  EBV LMP2A/2B to IFN signaling pathway
N00472  EBV LMP1 to IFN signaling pathway
N00473  EBV BGLF4 to IFN signaling pathway
N00474  EBV BHRF1 to intrinsic apoptotic pathway
N00475  EBV BHRF1 to intrinsic apoptotic pathway
N00476  EBV BHRF1 to intrinsic apoptotic pathway
N00477  EBV BHRF1 to crosstalk between extrinsic and intrinsic apoptotic pathways
N00478  EBV BARF1 to intrinsic apoptotic pathway
N00479  EBV BNLF2a to antigen processing and presentation by MHC class I molecules
N00480  EBV BILF1 to antigen processing and presentation by MHC class I molecules
N00481  EBV BZLF1 to p53-mediated transcription
N00482  EBV EBNA3C to p27-Cell cycle G1/S
N00483  EBV EBNA3C to cell cycle G1/S
N00484  EBV EBNA3C to cell cycle G1/S
N00485  EBV LMP1 to PI3K signaling pathway
N00486  EBV LMP1 to Jak-STAT signaling pathway
N00488  EBV LMP2A to BCR signaling pathway
N01265  EBV vIL10 to IL10R signaling
N01281  EBV BARF1 to CSF1R sigaling
Gene
IKBA (mutation) [HSA:4792] [KO:K04734]
IKBE (mutation) [HSA:4794] [KO:K05872]
c-rel (amplification) [HSA:5966] [KO:K09254]
CD95 (mutation) [HSA:355] [KO:K04390]
Pathogen
Human herpesvirus 4 (Epstein-Barr virus) [GN:T40079]
Drug
Cyclophosphamide [DR:D00287]
Chlorambucil [DR:D00266]
Carmustine [DR:D00254]
Lomustine [DR:D00363]
Dacarbazine [DR:D00288]
Vinblastine sulfate [DR:D01068]
Vincristine sulfate [DR:D02197]
Doxorubicin hydrochloride [DR:D01275]
Bleomycin sulfate [DR:D02177]
Procarbazine hydrochloride [DR:D00478]
Brentuximab vedotin [DR:D09587]
Nivolumab [DR:D10316]
Pembrolizumab [DR:D10574]
Comment
ICD-O: 9650/3, Tumor type: Classical Hodgkin lymphoma
ICD-O: 9663/3, Tumor type: Nodular sclerosis classical Hodgkin lymphoma
ICD-O: 9651/3, Tumor type: Lymphocyte-rich classical Hodgkin lymphoma
ICD-O: 9652/3, Tumor type: Mixed cellularity classical Hodgkin lymphoma
ICD-O: 9653/3, Tumor type: Lymphocyte-depleted classical Hodgkin lymphoma
Other DBs
ICD-11: 2B30
ICD-10: C81
MeSH: D006689
Reference
PMID:15618006 (tumor type)
  Authors
Kuppers R, Hansmann ML.
  Title
The Hodgkin and Reed/Sternberg cell.
  Journal
Int J Biochem Cell Biol 37:511-7 (2005)
DOI:10.1016/j.biocel.2003.10.025
Reference
PMID:15803153 (gene, carcinogen, tumor type)
  Authors
Kuppers R.
  Title
Mechanisms of B-cell lymphoma pathogenesis.
  Journal
Nat Rev Cancer 5:251-62 (2005)
DOI:10.1038/nrc1589
Reference
PMID:16385563 (gene, carcinogen)
  Authors
Brauninger A, Schmitz R, Bechtel D, Renne C, Hansmann ML, Kuppers R.
  Title
Molecular biology of Hodgkin's and Reed/Sternberg cells in Hodgkin's lymphoma.
  Journal
Int J Cancer 118:1853-61 (2006)
DOI:10.1002/ijc.21716
Reference
PMID:15760280 (carcinogen)
  Authors
Schottenfeld D, Beebe-Dimmer JL.
  Title
Advances in cancer epidemiology: understanding causal mechanisms and the evidence for implementing interventions.
  Journal
Annu Rev Public Health 26:37-60 (2005)
DOI:10.1146/annurev.publhealth.26.021304.144402
Reference
PMID:12078906 (marker)
  Authors
Zander T, Wiedenmann S, Wolf J.
  Title
Prognostic factors in Hodgkin's lymphoma.
  Journal
Ann Oncol 13 Suppl 1:67-74 (2002)
DOI:10.1093/annonc/mdf613
Reference
PMID:11142480 (marker)
  Authors
Axdorph U, Sjoberg J, Grimfors G, Landgren O, Porwit-MacDonald A, Bjorkholm M.
  Title
Biological markers may add to prediction of outcome achieved by the International Prognostic Score in Hodgkin's disease.
  Journal
Ann Oncol 11:1405-11 (2000)
DOI:10.1023/A:1026551727795
LinkDB

» Japanese version

KEGG   DISEASE: Nasopharyngeal cancer
Entry
H00054                      Disease                                

Name
Nasopharyngeal cancer
Description
Nasopharyngeal carcinoma (NPC) is a rare disease in most parts of the world, with an age-standardised annual incidence of less than 1 per 100000. However, in Southern China, parts of Southeast Asia and the Mediterranean basin, NPC is an endemic disease with an incidence of 10-30 per 100000. Genome-wide studies have unravelled multiple chromosomal abnormalities with involvement of specific oncogenes and tumor suppressor genes. Alterations of genes such as Ras association domain family 1A (RASSF1A), p16/INK4A, p14/ARF suggest that multiple cellular pathways were dysregulated in the NPC cells. Studies on the precancerous lesions revealed early genetic changes and a critical role of Epstein- Barr virus (EBV) latent infection in the development of this cancer.
Category
Cancer
Brite
Human diseases [BR:br08402]
 Cancers
  Head and neck cancers
   H00054  Nasopharyngeal cancer
Human diseases in ICD-11 classification [BR:br08403]
 02 Neoplasms
  Malignant neoplasms, except of lymphoid, haematopoietic, central nervous system or related tissues
   Malignant neoplasms, stated or presumed to be primary, of specified sites, except of lymphoid, haematopoietic, central nervous system or related tissues
    Malignant neoplasms of lip, oral cavity or pharynx
     2B6B  Malignant neoplasms of nasopharynx
      H00054  Nasopharyngeal cancer
Pathway
hsa05203  Viral carcinogenesis
hsa05169  Epstein-Barr virus infection
Network
  Element
N00223  EBV EBNA1 to p53-mediated transcription
N00224  EBV EBNALP RBP-Jk-mediated transcription
N00225  EBV EBNA2 to RBP-Jk-mediated transcription
N00226  EBV EBNA3A/3B/3C to RBP-Jk-mediated transcription
N00262  EBV EBNA3C to intrinsic apoptotic pathway
N00263  EBV EBNA3C to p53-mediated transcription
N00264  EBV EBNA3C to p27-Cell cycle G1/S
N00265  EBV LMP1 to NFKB signaling pathway
N00266  EBV LMP2A to PI3K signaling pathway
N00466  EBV BPLF1 to TLR2/4-NFKB signaling pathway
N00467  EBV BPLF1 to TLR2/4-NFKB signaling pathway
N00468  EBV BPLF1 to TLR2/4-NFKB signaling pathway
N00470  EBV BGLF4 to RIG-I-like receptor signaling pathway
N00471  EBV LMP2A/2B to IFN signaling pathway
N00472  EBV LMP1 to IFN signaling pathway
N00473  EBV BGLF4 to IFN signaling pathway
N00474  EBV BHRF1 to intrinsic apoptotic pathway
N00475  EBV BHRF1 to intrinsic apoptotic pathway
N00476  EBV BHRF1 to intrinsic apoptotic pathway
N00477  EBV BHRF1 to crosstalk between extrinsic and intrinsic apoptotic pathways
N00478  EBV BARF1 to intrinsic apoptotic pathway
N00479  EBV BNLF2a to antigen processing and presentation by MHC class I molecules
N00480  EBV BILF1 to antigen processing and presentation by MHC class I molecules
N00481  EBV BZLF1 to p53-mediated transcription
N00482  EBV EBNA3C to p27-Cell cycle G1/S
N00483  EBV EBNA3C to cell cycle G1/S
N00484  EBV EBNA3C to cell cycle G1/S
N00485  EBV LMP1 to PI3K signaling pathway
N00486  EBV LMP1 to Jak-STAT signaling pathway
N00488  EBV LMP2A to BCR signaling pathway
N01265  EBV vIL10 to IL10R signaling
N01281  EBV BARF1 to CSF1R sigaling
Gene
RASSF1 (promoter hypermethylation) [HSA:11186] [KO:K09850]
p16/INK4A (promoter hypermethylation) [HSA:1029] [KO:K06621]
Bcl-2 (overexpression) [HSA:596] [KO:K02161]
EDNRB (promoter hypermethylation) [HSA:1910] [KO:K04198]
TSLC1 (promoter hypermethylation) [HSA:23705] [KO:K06781]
E-cadherin (decreased expression) [HSA:999] [KO:K05689]
Carcinogen
Formaldehyde [CPD:C00067]
Salted fish (Chinese-style)
Tobacco smoking and tobacco smoke
Wood dust
Pathogen
Human herpesvirus 4 (Epstein-Barr virus) [GN:T40079]
Comment
ICD-O: 8072/3, Tumor type: Non-keratinizing carcinoma
ICD-O: 8071/3, Tumor type: Keratinizing squamous cell carcinoma
ICD-O: 8083/3, Tumor type: Basaloid squamous cell carcinoma
Other DBs
ICD-11: 2B6B
ICD-10: C11
MeSH: D009303
Reference
PMID:15958470 (description, carcinogen)
  Authors
Chan AT
  Title
Head and neck cancer: treatment of nasopharyngeal cancer.
  Journal
Ann Oncol 16 Suppl 2:ii265-8 (2005)
DOI:10.1093/annonc/mdi732
Reference
PMID:12450731 (description, gene, carcinogen)
  Authors
Lo KW, Huang DP.
  Title
Genetic and epigenetic changes in nasopharyngeal carcinoma.
  Journal
Semin Cancer Biol 12:451-62 (2002)
DOI:10.1016/S1044579X02000883
Reference
PMID:15510157 (gene, tumor type)
  Authors
Young LS, Rickinson AB.
  Title
Epstein-Barr virus: 40 years on.
  Journal
Nat Rev Cancer 4:757-68 (2004)
DOI:10.1038/nrc1452
Reference
PMID:15760280 (carcinogen)
  Authors
Schottenfeld D, Beebe-Dimmer JL.
  Title
Advances in cancer epidemiology: understanding causal mechanisms and the evidence for implementing interventions.
  Journal
Annu Rev Public Health 26:37-60 (2005)
DOI:10.1146/annurev.publhealth.26.021304.144402
Reference
PMID:17033133 (carcinogen)
  Authors
Binetti R, Costamagna FM, Marcello I.
  Title
Development of carcinogenicity classifications and evaluations: the case of formaldehyde.
  Journal
Ann Ist Super Sanita 42:132-43 (2006)
Reference
PMID:16140628 (carcinogen)
  Authors
Cogliano VJ, Grosse Y, Baan RA, Straif K, Secretan MB, El Ghissassi F.
  Title
Meeting report: summary of IARC monographs on formaldehyde, 2-butoxyethanol, and 1-tert-butoxy-2-propanol.
  Journal
Environ Health Perspect 113:1205-8 (2005)
DOI:10.1289/ehp.7542
Reference
PMID:17530489 (carcinogen)
  Authors
Abnet CC.
  Title
Carcinogenic food contaminants.
  Journal
Cancer Invest 25:189-96 (2007)
DOI:10.1080/07357900701208733
Reference
PMID:15552776 (carcinogen)
  Authors
Sasco AJ, Secretan MB, Straif K.
  Title
Tobacco smoking and cancer: a brief review of recent epidemiological evidence.
  Journal
Lung Cancer 45 Suppl 2:S3-9 (2004)
DOI:10.1016/j.lungcan.2004.07.998
Reference
PMID:12176778 (marker)
  Authors
Chan AT, Teo PM, Johnson PJ.
  Title
Nasopharyngeal carcinoma.
  Journal
Ann Oncol 13:1007-15 (2002)
DOI:10.1093/annonc/mdf179
Reference
PMID:12450734 (marker)
  Authors
Chan KC, Lo YM.
  Title
Circulating EBV DNA as a tumor marker for nasopharyngeal carcinoma.
  Journal
Semin Cancer Biol 12:489-96 (2002)
DOI:10.1016/S1044579X02000913
LinkDB

» Japanese version

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