Abstract
Angiosarcoma is a malignant tumor of vascular origin. It is also known as hemangiosarcoma, malignant hemangioendothelioma, malignant angioendothelioma, lymphedema-associated angiosarcoma, and lymphangiosarcoma.
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Notes
- 1.
MYC: this is a proto-oncogene and encodes a nuclear phosphoprotein (c-Myc) that plays a key role in cell cycle progression, apoptosis, and cellular transformation. The encoded protein forms a heterodimer with the related transcription factor MAX: then, this complex binds to the E-box DNA consensus sequence and regulates the transcription of specific target genes. Amplification of this gene is frequently observed in numerous human cancers, including radiation-induced angiosarcoma. Translocations involving this gene are associated with Burkitt lymphoma and multiple myeloma.
- 2.
Sunitinib: tyrosine kinase inhibitor targeting the following: PDGFR, VEGFR, KIT, FLT3, CSF1R, RET.
- 3.
Sorafenib: tyrosine kinase inhibitor targeting the following: BRAF, KIT, RET, FGFR1, FLT3, VEGFR1, VEGFR2, VEGFR3, PDGFRB.
- 4.
Bevacizumab: monoclonal antibody blocking VEGFA (vascular endothelial growth factor A).
- 5.
Pazopanib: tyrosine kinase inhibitor targeting the following: VEGFR, PDGFR, KIT.
- 6.
PIK3CA: this gene encodes for phosphatidylinositol-4,5-bisphosphate 3-kinase catalytic subunit alpha, the catalytic subunit of phosphatidylinositol 3-kinase (PI3K) that phosphorylates phosphatidylinositol to generate phosphatidylinositol 3,4,5-trisphosphate (PIP3), which is dephosphorylated by the protein product of tumor suppressor gene PTEN (a negative regulator of the PI3K-AKT pathway). PIP3 plays a key role by recruiting PH domain-containing proteins to the membrane, including AKT1 and PDPK1, activating signaling cascades involved in cell growth, survival, proliferation, motility, and morphology in response to various growth factors (e.g., EGF, insulin, IGF1, VEGFA, and PDGF). PIK3CA is the most recurrently mutated gene in breast cancer and has been found to important in a number of cancer types (PIK3CA is considered an oncogene).
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Mocellin, S. (2021). Angiosarcoma. In: Soft Tissue Tumors . Springer, Cham. https://doi.org/10.1007/978-3-030-58710-9_25
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