Chondrosarcoma – Ivosidenib works in Chondrosarcoma

Chondrosarcoma and IDH1 – Background

For locally advanced and/or metastatic Chondrosarcoma, no known effective therapy exists. The standard protocols are not effective. Mutations in the IDH1/2 enzymes occur in up to 65% of Chondrosarcoma cases. Ivosidenib (AG-120) is a selective inhibitor of mutant IDH1 approved in the United States for specific cases of acute myeloid leukemia. An ongoing study evaluating Ivosidenib treatment in patients with advanced Chondrosarcoma reported outcomes.

About the study

It is a phase I multicenter study of Ivosidenib monotherapy which enrolled patients with mutant IDH1 advanced solid tumors, including Chondrosarcoma. Ivosidenib was administered orally (100 mg twice daily to 1,200 mg once daily) in continuous 28-day cycles.

Results and Side effects

21 patients with advanced Chondrosarcoma received Ivosidenib.

  • Stable disease was achieved in 52% of patients.
  • The median time until disease progression under the treatment was 5.6 months.
  • In 39.5% of patients, the disease had not progressed after 6 months of treatment.
  • 4 patients received treatment for ≥2.5 years without progressive disease.
  • Results were inferior in dedifferentiated tumors.

Treatment-related side effects were mostly very mild. 12 patients experienced serious side effects; only one event was judged treatment related (hypophosphatemia).


  • In patients with Chondrosarcoma, Ivosidenib showed minimal toxicity and durable disease control.
  • Phase I clinical trials are attractive to cancer patients and should be considered as treatment options.
  • The best treatment for a cancer patient is to get the most advanced cancer drugs in advanced stages of development. There, the hope and the chance to extend life go far beyond the standard protocols.

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What is Sarcoma?

Sarcoma is a name for skeletal and soft tissue tumors. These tumors include over 70 cancer subtypes. These tumors develop from bones, cartilage and soft tissues such as connective tissue, muscle, fat, blood vessels and nerves. Bone sarcomas are usually separated from soft tissue sarcomas. Bone origin sarcoma accounts for about 20% of tumors, and is characterized by a number of different subtypes, most commonly called “Osteosarcoma.” Soft tissue sarcomas make up 80% of all sarcomas, and have about 60 different subtypes. The most common soft tissue sarcomas are “Leiomyosarcoma”, “Liposarcoma” and “Pleomorphic sarcoma”.

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