KEGG   DISEASE: Kaposi sarcoma
Entry
H00041                      Disease                                

Name
Kaposi sarcoma
  Supergrp
Solid tumor [DS:H02421]
Soft tissue sarcomas [DS:H02427]
Description
Kaposi sarcoma (KS) is an angioproliferative disease classified into classic KS, endemic KS, iatrogenic KS, and HIV-associated KS (HIV-KS), however, share the same histological traits and are all associated with infection by the human herpesvirus 8 (HHV8; also known as KSHV). Evidence indicates that KS progression occurs upon the deregulated expression of anti-apoptotic genes (Bcl-2), oncogenes (c-myc, c-int, ras) and oncosuppressor genes (TP53), and is associated with the long-lasting expression of HHV8 latency genes (LANA, v-cyc D, v-FLIP, Kaposin). All these genes are, in fact, expressed or altered in most KS spindle cells in the nodular-late stage of KS. Bcl-2 acts as a major KS progression factor, and TP53 and c-myc may also have a role in disease progression. HHV8 latency gene products may be involved in KS progression due to their capability of promoting cell growth by direct effects or antiapoptotic effects.
Category
Cancer
Brite
Human diseases [BR:br08402]
 Cancers
  Cancers of soft tissues and bone
   H00041  Kaposi sarcoma
Infectious diseases [BR:br08401]
 Viral infections
  Infections caused by dsDNA viruses
   H00041  Kaposi sarcoma
Human diseases in ICD-11 classification [BR:br08403]
 02 Neoplasms
  Malignant neoplasms, except primary neoplasms 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 mesenchymal neoplasms
     2B57  Kaposi sarcoma, primary site
      H00041  Kaposi sarcoma
Pathway
hsa05167  Kaposi sarcoma-associated herpesvirus infection
Related
pathway
hsa05200  Pathways in cancer
hsa05203  Viral carcinogenesis
Network
nt06110  MAPK signaling (viruses and bacteria)
nt06114  PI3K signaling (viruses)
nt06115  WNT signaling (viruses and bacteria)
nt06119  JAK-STAT signaling (viruses)
nt06120  Calcium signaling (viruses and bacteria)
nt06121  TLR signaling (viruses and bacteria)
nt06122  IFN signaling (viruses)
nt06123  TNF signaling (viruses and bacteria)
nt06124  Chemokine signaling (viruses)
nt06128  Autophagy (viruses and bacteria)
nt06129  MHC presentation (viruses)
nt06130  Cell cycle (viruses)
nt06131  Apoptosis (viruses and bacteria)
nt06136  Complement activation (viruses)
nt06150  Cytokine-cytokine receptor interaction (viruses)
nt06164  Kaposi sarcoma-associated herpesvirus (KSHV)
  Element
N00157  KSHV vGPCR to GNB/G-ERK signaling pathway
N00158  KSHV vGPCR to GNB/G-PI3K-AKT signaling pathway
N00159  KSHV K1 to PI3K signaling pathway
N00160  KSHV K1 to RAS-ERK signaling pathway
N00161  KSHV vIRF1/2 to TLR3-IRF3 signaling pathway
N00162  KSHV vIRF3 to TLR3-IRF7 signaling pathway
N00163  KSHV KIE1/2 to TLR3-IRF7 signaling pathway
N00164  KSHV vBCL2 to crosstalk between extrinsic and intrinsic apoptotic pathways
N00165  KSHV vIAP to crosstalk between extrinsic and intrinsic apoptotic pathways
N00166  KSHV vFLIP to crosstalk between extrinsic and intrinsic apoptotic pathways
N00167  KSHV vIRF1/3 to p21-cell cycle G1/S
N00168  KSHV vCyclin to cell cycle G1/S
N00169  KSHV LANA to p21-cell cycle G1/S
N00170  KSHV LANA to cell cycle G1/S
N00171  KSHV vFLIP to NFKB signaling pathway
N00172  KSHV K15 to PLCG-calcineurin signaling pathway
N00173  KSHV K15 to TNF-NFKB signaling pathway
N00174  KSHV vFLIP to TNF-NFKB signaling pathway
N00175  KSHV LANA to Wnt signaling pathway
N00176  KSHV vFLIP to autophagy-vesicle elongation
N00177  KSHV vBCL2 to autophagy-vesicle nucleation
N00178  KSHV vGPCR to GNB/G-PI3K-JNK signaling pathway
N00179  KSHV K1 to PI3K-NFKB signaling pathway
N00180  KSHV K1 to PLCG-calcineurin signaling pathway
N00181  KSHV vIL-6 to Jak-STAT signaling pathway
N00184  KSHV MIR1/2 to antigen processing and presentation by MHC class I molecules
N00185  KSHV MIR2 to cell surface molecule-endocytosis
N00187  KSHV Kaposin B to p38 signaling pathway
N00189  KSHV K15 to JNK signaling pathway
N00212  KSHV vCCL2 to CCR signaling pathway
N00213  KSHV Kaposin to alternative pathway of complement activation
N00261  KSHV vIRF2 to IFN signaling pathway
N00462  KSHV vCCL1/2/3 to CCR signaling pathway
N01170  KSHV vCCL2 to CCR1 signaling
N01178  KSHV vCCL2 to CCR2 signaling
N01189  KSHV vCCL2 to CCR3 signaling
N01195  KSHV vCCL3 to CCR4 signaling
N01206  KSHV vCCL2 to CCR5 signaling
N01210  KSHV vCCL1 to CCR5 signaling
N01218  CCL1 to KSHV vGPCR signaling
N01220  KSHV vCCL1/2 to CCR8 signaling
N01226  KSHV vCCL2 to CCR10 signaling
N01234  CXCL1/5/8 to KSHV vGPCR signaling
N01235  CXCL6 to KSHV vGPCR signaling
N01239  CXCL2/3/7 to KSHV vGPCR signaling
N01242  CXCL4 to KSHV vGPCR signaling
N01243  CXCL10 to KSHV vGPCR signaling
N01251  CXCL12 to KSHV vGPCR signaling
N01252  KSHV vCCL2 to CXCR4 signaling
N01260  KSHV vCCL2 to XCR1 signaling
N01261  KSHV vCCL3 to XCR1 signaling
N01263  KSHV vCCL2 to CX3CR1 signaling
N01267  KSHV vIL6 to IL6R signaling
Gene
Bcl-2 (overexpression) [HSA:596] [KO:K02161]
c-MYC (overexpression) [HSA:4609] [KO:K04377]
c-INT (overexpression, mutation) [HSA:2248] [KO:K04358]
K-ras (overexpression, mutation) [HSA:3845] [KO:K07827]
p53 (mutation) [HSA:7157] [KO:K04451]
Carcinogen
Azathioprine [CPD:C06837]
Pathogen
Human herpesvirus 8 (KSHV) [GN:T40081]
Human immunodeficiency virus 1 [GN:T40001]
Drug
Alitretinoin [DR:D02815]
Vinblastine sulfate [DR:D01068]
Paclitaxel [DR:D00491]
Doxorubicin hydrochloride [DR:D01275]
Interferon alfa-2b [DR:D02745]
Comment
ICD-O: 9140/3, Tumor type: Kaposi sarcoma
Other DBs
ICD-11: 2B57
ICD-10: C46
MeSH: D012514
Reference
PMID:11423257 (description, gene, carcinogen)
  Authors
Ensoli B, Sgadari C, Barillari G, Sirianni MC, Sturzl M, Monini P
  Title
Biology of Kaposi's sarcoma.
  Journal
Eur J Cancer 37:1251-69 (2001)
DOI:10.1016/S0959-8049(01)00121-6
Reference
PMID:15334644 (carcinogen, tumor type)
  Authors
Schwartz RA.
  Title
Kaposi's sarcoma: an update.
  Journal
J Surg Oncol 87:146-51 (2004)
DOI:10.1002/jso.20090
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: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:17142074 (carcinogen)
  Authors
Feller L, Wood NH, Lemmer J
  Title
HIV-associated Kaposi sarcoma: pathogenic mechanisms.
  Journal
Oral Surg Oral Med Oral Pathol Oral Radiol Endod 104:521-9 (2007)
DOI:10.1016/j.tripleo.2006.08.015
Reference
PMID:17630560 (marker)
  Authors
Anderson LA, Goedert JJ.
  Title
Tumor markers and treatments for Kaposi sarcoma.
  Journal
AIDS 21:1637-9 (2007)
DOI:10.1097/QAD.0b013e32826fb721
Reference
PMID:15578080 (marker)
  Authors
Hammock L, Reisenauer A, Wang W, Cohen C, Birdsong G, Folpe AL.
  Title
Latency-associated nuclear antigen expression and human herpesvirus-8 polymerase chain reaction in the evaluation of Kaposi sarcoma and other vascular tumors in HIV-positive patients.
  Journal
Mod Pathol 18:463-8 (2005)
DOI:10.1038/modpathol.3800221

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