KEGG   DISEASE: Hepatic angiosarcomaHelp
Entry
H01557                      Disease                                

Name
Hepatic angiosarcoma
Description
Angiosarcoma represents 1 to 2% of soft tissue tumors. It originates from endothelial cells of small blood vessels and may affect a variety of organs, including the retroperitoneum, skeletal muscle, subcutis, liver, heart and breast. Primary hepatic angiosarcomas (HAS) are much rare tumors, with worse prognosis compared with other angiosarcomas. HAS has been found to be caused by occupational exposure to vinyl chloride (VC) monomer since 1970, and the number of VC-associated HAS cases reported up to the end of 1998 was 197 worldwide. Exposure of VC connects with a series of mechanistic events leading to carcinogenic outcome; mutations of K-ras and p53 are also detected.
Category
Cancer
Brite
Human diseases [BR:br08402]
 Cancers
  Cancers of the digestive system
   H01557  Hepatic angiosarcoma
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 mesenchymal neoplasms
     2B56  Angiosarcoma, primary site
      H01557  Hepatic angiosarcoma
BRITE hierarchy
Gene
TP53 (mutation) [HSA:7157] [KO:K04451]
KRAS-2 (mutation) [HSA:3845] [KO:K07827]
Carcinogen
Vinyl chloride [CPD:C06793]
Comment
ICD-O-3: 9120/3 Angiosarcoma
Other DBs
ICD-11: 2B56.3
ICD-10: C22.3
MeSH: D006394
Reference
PMID:26008857 (description, gene, carcinogen, marker, drug)
  Authors
Chaudhary P, Bhadana U, Singh RA, Ahuja A
  Title
Primary hepatic angiosarcoma.
  Journal
Eur J Surg Oncol 41:1137-43 (2015)
DOI:10.1016/j.ejso.2015.04.022
Reference
PMID:24372769 (description, gene, carcinogen, marker, drug)
  Authors
Zheng YW, Zhang XW, Zhang JL, Hui ZZ, Du WJ, Li RM, Ren XB
  Title
Primary hepatic angiosarcoma and potential treatment options.
  Journal
J Gastroenterol Hepatol 29:906-11 (2014)
DOI:10.1111/jgh.12506
Reference
PMID:20537949 (description, gene, carcinogen, marker, drug)
  Authors
Young RJ, Brown NJ, Reed MW, Hughes D, Woll PJ
  Title
Angiosarcoma.
  Journal
Lancet Oncol 11:983-91 (2010)
DOI:10.1016/S1470-2045(10)70023-1
Reference
PMID:19031953 (marker, drug)
  Authors
Asmane I, Litique V, Heymann S, Marcellin L, Metivier AC, Duclos B, Bergerat JP, Kurtz JE
  Title
Adriamycin, cisplatin, ifosfamide and paclitaxel combination as front-line chemotherapy for locally advanced and metastatic angiosarcoma. Analysis of three case reports and review of the literature.
  Journal
Anticancer Res 28:3041-5 (2008)
Reference
PMID:24707153 (marker)
  Authors
Wang ZB, Yuan J, Chen W, Wei LX
  Title
Transcription factor ERG is a specific and sensitive diagnostic marker for hepatic angiosarcoma.
  Journal
World J Gastroenterol 20:3672-9 (2014)
DOI:10.3748/wjg.v20.i13.3672

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