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Targeted drugs target specific parts of cancer cells. Each type of targeted therapy works differently, but all of them affect the way a cancer cell grows, divides, repairs itself, or interacts with other cells.

Targeted therapy is becoming an important treatment option for some soft tissue sarcomas.

Pazopanib (Votrient)

Pazopanib blocks several cell enzymes called tyrosine kinases that are important for cell growth and survival. It might be used to treat certain advanced soft tissue sarcomas that have not responded to chemotherapy.

It can help slow tumor growth and ease side effects in patients with sarcomas that cannot be removed with surgery.

Pazopanib is taken in pill form, once a day.

Possible side effects

Common side effects include high blood pressure, fatigue, nausea, diarrhea, headaches, changes in hair color, low blood cell counts, and liver problems. In some patients, this drug causes abnormal results on liver function tests, but it rarely leads to severe liver damage that can be life-threatening.

In rare cases, bleeding, clotting, wound healing, heart rhythm problems, and heart attacks can occur.

If you're taking pazopanib, your doctor will monitor your heart with EKGs and do blood tests to check for liver problems or other changes.

Tazemetostat (Tazverik)

Tazemetostat works by targeting EZH2, a protein that helps some cancer cells grow. This drug can be used to treat epithelioid sarcomas that can’t be removed completely by surgery. It can shrink or slow the growth of some of these cancers, although it’s not yet clear if it can help people live longer.

This drug is taken as pills, typically twice a day.

Possible side effects

The most common side effects of this drug include pain, fatigue, nausea, vomiting, loss of appetite, and constipation. Tazemetostat can also increase the risk of developing some types of blood cancers, including certain leukemias and lymphomas.

Other targeted drugs

Many other targeted drugs might also be helpful in treating certain types of soft tissue tumors. Examples of these drugs include:

• Regorafenib (Stivarga)
• Imabinib (Gleevec) for certain dermatofibrosarcoma protuberans (DFSP)
• Sorafenib (Nexavar) for solitary fibrous tumor
• Tyrosine kinase inhibitors (TKIs), such as sunitinib (Sutent), axitinib (Inlyta), and cabozantinib (Cabometyx)
• Bevacizumab (Avastin)
• Trametinib (Mekinist), a MEK inhibitor, for epithelioid hemangioendothelioma
• mTOR inhibitors, such as sirolimus (Rapamune), everolimus (Afinitor), and temsirolimus (Torisel), for recurrent angiomyolipoma or lymphangioleiomyomatosis or advanced malignant perivascular epithelioid cell tumor (PEComa)
• Larotrectinib (Vitrakvi), entrectinib (Rozlytrek), or repotrectinib (Augtyro), for tumors with an NTRK gene change
• Sirolimus albumin-bound nanoparticles (also known as nab-sirolimus or Fyarro), for advanced PEComas
• Alectinib (Alecensa), brigatinib (Alunbrig), ceritinib (Zykadia), crizotinib (Xalkori), and lorlatinib (Lorbrena) for inflammatory myofibroblastic tumor (IMT) with ALK gene change
• Selpercatinib (Retevmo) for tumors with RET gene change
• Niraogascestat (Ogsiveo), a gamma-secretase inhibitor for desmoid tumors
• Pexidartinib (Turalio) and Vimseltinib (Romvimza), CSF1R inhibitors, for tenosynovial giant cell tumor
• Palbociclib (Ibrance) and Abemaciclib (Verzenio), CDK4/6 inhibitors, for certain liposarcomas