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ment to the tumor 49 the antiangiogenic agent pazopanib is approved in many countries for use in sarcomas such as epithelioid sarcoma targeted therapies edit given the multiple genetic abnormalities and disrupted biological pathways observed in epithelioid sarcoma drugs targeting unique tumor characteristics are being examine for more effective treatments tyrosine kinase inhibitors edit tyrosine kinase inhibitors such as sunitinib pazopanib and dasatinib have shown some effect against several cancer types one example among sarcomas being imatinib in gastrointestinal stromal tumors gists 50 tyrosine kinase a subclass of protein kinases is an enzyme that transfers a phosphate group from an atp molecule to a protein in a cell 51 it functions as an on or off switch for many cellular functions including signaling within the cell and cell division tyrosine kinases can contain mutations that cause them to become constitutively active 52 or stuck in the on position resulting in unregulated cell division a hallmark of cancer tyrosine kinase inhibitors block the action of these enzymes tyrosine kinase inhibitors have been shown to inhibit the vegf egfr and met 51 pathways that are frequently over expressed in epithelioid sarcoma they also can be used against the kit and jak stat signaling pathways 51 which are involved in many cancers and may be involved in epithelioid sarcoma temsirolimus is a tyrosine kinase inhibitor that blocks the effects of the mtor protein and inhibits the mtor pathway because of crosstalk between cell signaling pathways it has been shown that while interfering with the mtor pathway alone produces only limited results in halting tumorigenesis inhibiting both the mtor and the egfr pathways concurrently shows an increased effect 22 sine edit selective inhibitors of nuclear export sine compounds such as selinexor and cbs9106 are being investigated in several sarcomas and have shown promising results in both hematological malignancies and solid tumors 53 54 however a randomized trial of selinexor in liposarcoma a distant cousin of epithelioid sarcoma was negative selinexor does have approval for other cancer diagnoses hdac inhibitors edit histone deacetylase hdac inhibitors such as vorinostat have shown some promise in epithelioid sarcoma researchers in texas are investigating whether or not hdac inhibitors can reverse the loss of ini1 function that is characteristic of epithelioid sarcoma 8 hdac inhibitors work by blocking events involved in dna replication and therefore in cell division 55 blocking hdac has been shown to encourage cancer cells to enter apoptosis 8 several dietary phytochemicals have been shown to be effective hdac inhibitors 56 these include sulforphane indole 3 carbinol and phenethyl isothiocyanates found in broccoli kale and watercress and epigallocatecehin 3 gallate found in green tea citation needed cdk inhibitors edit because of the association see above with cyclin d1 cdk inhibitors are being studied in a variety of cancers palbociclib is a cdk inhibitor approved for some breast cancer by virtue of its blockade of cdk4 and cdk6 other experimental cdk4 6 inhibitors include abemaciclib and ribociclib targeting the cancer stem cell edit cancer stem cells or cancer initiating cells are thought to be a small population of cells within the tumor that are directly responsible for tumor formation they are thought to be resistant to treatment and to have the ability to form all the cells needed for tumor development they are suspected to be a contributing factor in cancer progression and relapse after treatment certain stem like cells have been found in epithelioid sarcoma that are marked by cd109 cluster of differentiation 109 25 providing a theoretically druggable target for epithelioid sarcoma however cd109 is expressed in many normal cells of the body such as t cells and endothelial cells lining every blood vessel making cd109 a poor target for immunotherapy oncolytic viral therapy edit oncolytic viral therapy is an emerging cancer therapy that attempts to infect cancer cells with a genetically engineered virus that can penetrate the dna of the cell the virus then 1 can do direct damage to the cancer cell 2 is passed on throughout the cells of the tumor via viral reproduction and 3 provides a target for an immune response from the patient 17 57 it has been noted that the therapeutic potential of oncolytic virotherapy is not a simple consequence of the cytopathic effect but strongly relies on the activation of the body s own immune response against infected cells 57 58 superior anticancer effects have been observed when oncolytic viruses are engineered to express or be co administered with immunostimulatory molecules such as gm csf 58 telomelysin obp 301 is an adenovirus that targets telomerase 59 an enzyme that is expressed in practically all cancer cells but not in normal cells obp 301 is not approved for use in cancer patients but it has been studied in epithelioid sarcoma and shown to promote apoptosis and cell death in the laboratory 59 cgtg 102 developed by oncos therapeutics is an adenovirus currently in orphan drug status for soft tissue sarcomas it is modified to selectively replicate in p16 rb defective cells which include most human cancer cells in addition cgtg 102 codes for the granulocyte macrophage colony stimulating factor gm csf 58 60 a potent immunostimulatory molecule while cgtg 102 has shown efficacy as a single agent against several soft tissue sarcomas in the laboratory as of 2023 clinical research on it appears to have come to a halt 61 additional images edit intermed mag high mag high mag smarcb1 see also edit soft tissue sarcoma sarcoma malignant rhabdoid tumour atypical teratoid rhabdoid tumour references edit 1 2 enzinger f m 1970 epithelioid sarcoma a sarcoma simulating a granuloma or a carcinoma cancer 26 5 1029 41 doi 10 1002 1097 0142 197011 26 5 1029 aid cncr2820260510 3 0 co 2 r pmid 5476785 guillou l wadden c 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