KEGG   DISEASE: AngiosarcomaHelp
Entry
H01666                      Disease                                

Name
Angiosarcoma
Description
Angiosarcomas are rare, generally aggressive, malignant mesenchymal tumours of vascular origin and constitute less than 1% of all sarcomas. Their most frequent site of origin is the head and neck, especially the scalp, of elderly men. Angiosarcomas are likely derived from vascular endothelial cells, possibly resulting from aberrant angiogenesis. Studies have shown relatively increased expression of vascular endothelial growth factor (VEGF) and its receptors (VEGFr1-3) in human angiosarcoma. The most common gene mutation in angiosarcomas is KRAS as well as other RAS mutations. Activation of the RAS pathway is often tumorigenic. Although immortalised murine endothelial cells form benign haemangiomas in vivo, the addition of activated HRAS produces rapidly growing, poorly differentiated angiosarcomas.
Category
Cancer
Brite
Human diseases [BR:br08402]
 Cancers
  Cancers of soft tissues and bone
   H01666  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
      H01666  Angiosarcoma
BRITE hierarchy
Gene
KRAS (mutation) [HSA:3845] [KO:K07827]
HRAS (mutation) [HSA:3265] [KO:K02833]
NRAS (mutation) [HSA:4893] [KO:K07828]
Carcinogen
Vinyl chloride [CPD:C06793]
Thorium dioxide
Arsenic [CPD:C06269]
Anabolic steroids
Comment
ICD-O-3: 9120/3 Angiosarcoma
Other DBs
ICD-11: 2B56
ICD-10: C44.9 C22.3
MeSH: D006394
Reference
PMID:11251578 (drug)
  Authors
Tanioka M, Ikoma A, Morita K, Fujii H, Toda KI, Takahashi K, Tanaka T, Nishigori C, Jin G, Higashi S, Toyokuni S, Miyachi Y
  Title
Angiosarcoma of the scalp: absence of vascular endothelial cadherin in primary and metastatic lesions.
  Journal
Br J Dermatol 144:380-3 (2001)
DOI:10.1046/j.1365-2133.2001.04032.x
Reference
PMID:24685486 (drug)
  Authors
Letsa I, Benson C, Al-Muderis O, Judson I
  Title
Angiosarcoma of the face and scalp: effective systemic treatment in the older patient.
  Journal
J Geriatr Oncol 5:276-80 (2014)
DOI:10.1016/j.jgo.2014.02.004
Reference
PMID:25634014 (carcinogen)
  Authors
Patel SH, Hayden RE, Hinni ML, Wong WW, Foote RL, Milani S, Wu Q, Ko SJ, Halyard MY
  Title
Angiosarcoma of the scalp and face: the Mayo Clinic experience.
  Journal
JAMA Otolaryngol Head Neck Surg 141:335-40 (2015)
DOI:10.1001/jamaoto.2014.3584
Reference
PMID:20537949 (gene, marker, carcinogen)
  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
  Authors
Huntington JT, Jones C, Liebner DA, Chen JL, Pollock RE
  Title
Angiosarcoma: A rare malignancy with protean clinical presentations.
  Journal
J Surg Oncol 111:941-50 (2015)
DOI:10.1002/jso.23918
Reference
PMID:24384851 (marker)
  Authors
Antonescu C
  Title
Malignant vascular tumors--an update.
  Journal
Mod Pathol 27 Suppl 1:S30-8 (2014)
DOI:10.1038/modpathol.2013.176
Reference
PMID:25352641 (marker)
  Authors
Sullivan HC, Edgar MA, Cohen C, Kovach CK, HooKim K, Reid MD
  Title
The utility of ERG, CD31 and CD34 in the cytological diagnosis of angiosarcoma: an analysis of 25 cases.
  Journal
J Clin Pathol 68:44-50 (2015)
DOI:10.1136/jclinpath-2014-202629
Reference
PMID:15817349 (drug)
  Authors
Ohguri T, Imada H, Nomoto S, Yahara K, Hisaoka M, Hashimoto H, Tokura Y, Nakamura K, Shioyama Y, Honda H, Terashima H, Moroi Y, Furue M, Korogi Y
  Title
Angiosarcoma of the scalp treated with curative radiotherapy plus recombinant interleukin-2 immunotherapy.
  Journal
Int J Radiat Oncol Biol Phys 61:1446-53 (2005)
DOI:10.1016/j.ijrobp.2004.08.008

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