Details of Disease

General Information of Disease (ID: DISIYS9W)

• Disease Name: Angiosarcoma
• Synonyms: angiosarcoma (disease); sarcoma of blood vessel; hemangiosarcoma; angiosarcoma; vascular sarcoma; hemangiosarcoma, malignant; malignant hemangioendothelioma; malignant angioendothelioma; blood vessel sarcoma
• Disease Class: 2B56: Angiosarcoma
• Definition: A malignant tumor arising from the endothelial cells of the blood vessels. Microscopically, it is characterized by frequently open vascular anastomosing and branching channels. The malignant cells that line the vascular channels are spindle or epithelioid and often display hyperchromatic nuclei. Angiosarcomas most frequently occur in the skin and breast. Patients with long-standing lymphedema are at increased risk of developing angiosarcoma.
• Disease Hierarchy:
  DISNSLVG: Vascular cancer
  DISSN8XB: Soft tissue sarcoma
  DISZDG3U: Sarcoma
  DISIYS9W: Angiosarcoma
• ICD Code:
  ICD-11: ICD-11: 2B56
  Expand ICD-11: 'XH6264
• Disease Identifiers:
  MONDO ID: MONDO_0016982
  MESH ID: D006394
  UMLS CUI: C0018923
  MedGen ID: 42385
  HPO ID: HP:0200058
  Orphanet ID: 263413
  SNOMED CT ID: 33176006

Drug-Interaction Atlas (DIA) of This Disease

This Disease is Treated as An Indication in 1 Clinical Trial Drug(s)

Drug Name	Drug ID	Highest Status	Drug Type	REF
TRC105	DM1874J	Phase 3	Monoclonal antibody	[1]

Molecular Interaction Atlas (MIA) of This Disease

This Disease Is Related to 19 DTT Molecule(s)

Gene Name	DTT ID	Evidence Level	Mode of Inheritance	REF
ANGPT2	TTKLQTJ	Limited	Biomarker	[2]
CCND1	TTFCJ7S	Limited	Biomarker	[2]
COL18A1	TT63DI9	Limited	Biomarker	[3]
FLT1	TT1VAUK	Limited	Altered Expression	[4]
JUN	TTS7IR5	Limited	Biomarker	[2]
MYC	TTNQ5ZP	Limited	Biomarker	[5]
SLC29A1	TTLXAKE	Limited	Altered Expression	[6]
VEGFC	TT0QUFV	Limited	Biomarker	[2]
CSF3	TT5TQ2W	moderate	Therapeutic	[7]
PECAM1	TT4EZB2	moderate	Altered Expression	[8]
ADRB1	TTR6W5O	Strong	Altered Expression	[9]
KDR	TTUTJGQ	Strong	Genetic Variation	[10]
KRAS	TTM8FR7	Strong	Genetic Variation	[11]
PLCG1	TT6T4JI	Strong	Genetic Variation	[12]
PRKCI	TTWJTHX	Strong	Altered Expression	[13]
PTPRB	TT64I9Q	Strong	Genetic Variation	[14]
TIE1	TTT4236	Strong	Altered Expression	[15]
GJA4	TTQO1VY	Definitive	Genetic Variation	[16]
HRAS	TT28ZON	Definitive	Genetic Variation	[17]

This Disease Is Related to 1 DTP Molecule(s)

Gene Name	DTP ID	Evidence Level	Mode of Inheritance	REF
SLC43A3	DTGBPR5	Strong	Biomarker	[18]

This Disease Is Related to 20 DOT Molecule(s)

Gene Name	DOT ID	Evidence Level	Mode of Inheritance	REF
CEP85L	OTSHJFOT	Disputed	Genetic Variation	[19]
SFRP2	OT8GZ0CA	moderate	Biomarker	[20]
ADAM19	OTH88TXU	Strong	Biomarker	[21]
CAMTA1	OTAN1S5B	Strong	Biomarker	[22]
CIC	OTFXCHNZ	Strong	Biomarker	[23]
COPA	OTZ1DTXU	Strong	Biomarker	[24]
CRKL	OTOYSD1R	Strong	Genetic Variation	[17]
DLC1	OTP8LMCR	Strong	Biomarker	[25]
DRAP1	OTRWLF6D	Strong	Biomarker	[24]
EFNA3	OTJGOUMZ	Strong	Biomarker	[26]
EFS	OT06O7XL	Strong	Biomarker	[27]
EMCN	OTS03CZC	Strong	Altered Expression	[28]
ERRFI1	OT7VZ2IZ	Strong	Biomarker	[24]
MAML1	OTQA4DDN	Strong	Biomarker	[29]
NUP160	OTUKVV3I	Strong	Altered Expression	[18]
POT1	OTNBXJCQ	Strong	Biomarker	[30]
PRRX2	OT8UR4AU	Strong	Altered Expression	[31]
SIRT7	OT5M4OT4	Strong	Altered Expression	[32]
TFE3	OTM99ZWH	Strong	Genetic Variation	[33]
ERAS	OT528IZO	Definitive	Biomarker	[34]

References

• [1] ClinicalTrials.gov (NCT02979899) Trial of TRC105 and Pazopanib Versus Pazopanib Alone in Patients With Advanced Angiosarcoma (TAPPAS). U.S. National Institutes of Health.
• [2] Carcinogenic susceptibility of rasH2 mice to troglitazone.Arch Toxicol. 2007 Dec;81(12):883-94. doi: 10.1007/s00204-007-0218-1. Epub 2007 Jun 14.
• [3] The effect of ifosfamide, epirubicin, and recombinant human endostatin therapy on a cardiac angiosarcoma: A case report.Medicine (Baltimore). 2019 Apr;98(17):e15290. doi: 10.1097/MD.0000000000015290.
• [4] Tumor-Associated Protein Profiles in Kaposi Sarcoma and Mimicking Vascular Tumors, and Their Pathological Implications.Int J Mol Sci. 2019 Jun 27;20(13):3142. doi: 10.3390/ijms20133142.
• [5] Post-radiation vascular lesions of the breast.J Cutan Pathol. 2019 Jan;46(1):52-58. doi: 10.1111/cup.13363. Epub 2018 Nov 4.
• [6] Human equilibrative nucleoside transporter 1 gene expression is associated with gemcitabine efficacy in advanced leiomyosarcoma and angiosarcoma.Br J Cancer. 2017 Jul 25;117(3):340-346. doi: 10.1038/bjc.2017.187. Epub 2017 Jun 22.
• [7] Phase II trial of paclitaxel in patients with soft-tissue sarcoma.Cancer Invest. 1998;16(7):442-6. doi: 10.3109/07357909809011697.
• [8] Angiosarcoma and anaplastic carcinoma of the thyroid are two distinct entities: a morphologic, immunohistochemical, and genetic study.Mod Pathol. 2019 Jun;32(6):787-798. doi: 10.1038/s41379-018-0199-z. Epub 2019 Feb 5.
• [9] Effective Management of Advanced Angiosarcoma by the Synergistic Combination of Propranolol and Vinblastine-based Metronomic Chemotherapy: A Bench to Bedside Study.EBioMedicine. 2016 Apr;6:87-95. doi: 10.1016/j.ebiom.2016.02.026. Epub 2016 Feb 17.
• [10] Genomic and transcriptomic comparison of post-radiation versus sporadic sarcomas.Mod Pathol. 2019 Dec;32(12):1786-1794. doi: 10.1038/s41379-019-0300-2. Epub 2019 Jun 26.
• [11] Sequential development of hepatocellular carcinoma and liver angiosarcoma in a vinyl chloride-exposed worker.Hum Pathol. 2016 Nov;57:193-196. doi: 10.1016/j.humpath.2016.07.021. Epub 2016 Aug 18.
• [12] Actionable mutations in canine hemangiosarcoma.PLoS One. 2017 Nov 30;12(11):e0188667. doi: 10.1371/journal.pone.0188667. eCollection 2017.
• [13] Regulation of programmed cell death ligand 1 expression by atypical protein kinase C lambda/iota in cutaneous angiosarcoma.Cancer Sci. 2019 May;110(5):1780-1789. doi: 10.1111/cas.13981. Epub 2019 Mar 21.
• [14] A phase II study of ENMD-2076 in advanced soft tissue sarcoma (STS).Sci Rep. 2019 May 14;9(1):7390. doi: 10.1038/s41598-019-43222-6.
• [15] Consistent MYC and FLT4 gene amplification in radiation-induced angiosarcoma but not in other radiation-associated atypical vascular lesions.Genes Chromosomes Cancer. 2011 Jan;50(1):25-33. doi: 10.1002/gcc.20827.
• [16] Unexpected role for the human Cx37 C1019T polymorphism in tumour cell proliferation.Carcinogenesis. 2010 Nov;31(11):1922-31. doi: 10.1093/carcin/bgq170. Epub 2010 Aug 12.
• [17] Targeted massively parallel sequencing of angiosarcomas reveals frequent activation of the mitogen activated protein kinase pathway.Oncotarget. 2015 Nov 3;6(34):36041-52. doi: 10.18632/oncotarget.5936.
• [18] NUP160-SLC43A3 is a novel recurrent fusion oncogene in angiosarcoma.Cancer Res. 2015 Nov 1;75(21):4458-65. doi: 10.1158/0008-5472.CAN-15-0418.
• [19] Breakpoint analysis of transcriptional and genomic profiles uncovers novel gene fusions spanning multiple human cancer types.PLoS Genet. 2013 Apr;9(4):e1003464. doi: 10.1371/journal.pgen.1003464. Epub 2013 Apr 25.
• [20] Development of a Novel Humanized Monoclonal Antibody to Secreted Frizzled-Related Protein-2 That Inhibits Triple-Negative Breast Cancer and Angiosarcoma Growth In Vivo.Ann Surg Oncol. 2019 Dec;26(13):4782-4790. doi: 10.1245/s10434-019-07800-2. Epub 2019 Sep 12.
• [21] Biallelic Dicer1 Loss Mediated by aP2-Cre Drives Angiosarcoma.Cancer Res. 2017 Nov 15;77(22):6109-6118. doi: 10.1158/0008-5472.CAN-17-1262. Epub 2017 Sep 15.
• [22] CAMTA-1 Expression in 24 Cases of Hepatic Epithelioid Hemangioendothelioma in a Single Institute: Diagnostic Utility for Differential Diagnosis from Hepatic Angiosarcoma.In Vivo. 2019 Nov-Dec;33(6):2293-2297. doi: 10.21873/invivo.11736.
• [23] ETV transcriptional upregulation is more reliable than RNA sequencing algorithms and FISH in diagnosing round cell sarcomas with CIC gene rearrangements.Genes Chromosomes Cancer. 2017 Jun;56(6):501-510. doi: 10.1002/gcc.22454. Epub 2017 Mar 31.
• [24] Identification of potential target genes associated with the effect of propranolol on angiosarcoma via microarray analysis.Oncol Lett. 2017 Jun;13(6):4267-4275. doi: 10.3892/ol.2017.5968. Epub 2017 Mar 31.
• [25] DLC1 deficiency and YAP signaling drive endothelial cell contact inhibition of growth and tumorigenesis.Oncogene. 2019 Nov;38(45):7046-7059. doi: 10.1038/s41388-019-0944-x. Epub 2019 Aug 13.
• [26] The role of miR-210, E2F3 and ephrin A3 in angiosarcoma cell proliferation.Eur J Dermatol. 2017 Oct 1;27(5):464-471. doi: 10.1684/ejd.2017.3084.
• [27] Endothelial cell malignancies in infants, children and adolescents: Treatment results of three Cooperative Weichteilsarkom Studiengruppe (CWS) trials and one registry.Pediatr Blood Cancer. 2020 Mar;67(3):e28095. doi: 10.1002/pbc.28095. Epub 2019 Dec 8.
• [28] Expression of endomucin, a novel endothelial sialomucin, in normal and diseased human skin.J Invest Dermatol. 2002 Dec;119(6):1388-93. doi: 10.1046/j.1523-1747.2002.19647.x.
• [29] The miR-17-92 cluster and its target THBS1 are differentially expressed in angiosarcomas dependent on MYC amplification.Genes Chromosomes Cancer. 2012 Jun;51(6):569-78. doi: 10.1002/gcc.21943. Epub 2012 Mar 2.
• [30] POT1 and Damage Response Malfunction Trigger Acquisition of Somatic Activating Mutations in the VEGF Pathway in Cardiac Angiosarcomas.J Am Heart Assoc. 2019 Sep 17;8(18):e012875. doi: 10.1161/JAHA.119.012875. Epub 2019 Sep 12.
• [31] Expression of peroxiredoxin II in vascular tumors of the skin: a novel vascular marker of endothelial cells.J Am Acad Dermatol. 2003 Sep;49(3):487-91. doi: 10.1067/s0190-9622(03)01485-3.
• [32] MicroRNA-340 inhibits the growth and invasion of angiosarcoma cells by targeting SIRT7.Biomed Pharmacother. 2018 Jul;103:1061-1068. doi: 10.1016/j.biopha.2018.04.148. Epub 2018 Apr 25.
• [33] Malignant vascular tumors--an update.Mod Pathol. 2014 Jan;27 Suppl 1:S30-8. doi: 10.1038/modpathol.2013.176.
• [34] Susceptibility of transgenic mice carrying human prototype c-Ha-ras gene in a short-term carcinogenicity study of vinyl carbamate and ras gene analyses of the induced tumors.Mol Carcinog. 1997 Nov;20(3):298-307. doi: 10.1002/(sici)1098-2744(199711)20:3<298::aid-mc6>3.0.co;2-h.