Robert G. Maki, MD, PhD, FACP, FASCO, clinical director, Sarcoma Program, Abramson Cancer Center, Penn Medicine, discusses the utility of IDH1/2 inhibitors in chondrosarcoma.

The potential advantage of metabolic therapy among patients with cancer has garnered significant research interest, says Maki.

IDH1/2 inhibitors, for example, are available to patients with gliomas and leukemias. Additionally, patients with chondrosarcoma, a rare subtype of sarcoma, frequently harbor 

 alterations may not be driver mutations, IDH1 inhibitors have demonstrated prolonged stability of disease with potential tumor shrinkage in patients with chondrosarcoma, says Maki. 


Studies evaluating IDH1/2 inhibitors in this setting are in early phases, but these data suggest that metabolic therapy may be an effective approach in treating patients with chondrosarcoma in the future, concludes Maki.

Videos

Robert G. Maki, MD, PhD, FACP, FASCO, discusses the utility of IDH1/2 inhibitors in chondrosarcoma.  



Dr. Maki on the Utility of IDH1/2 Inhibitors in Chondrosarcoma 

Robert G. Maki, MD, PhD, FACP, FASCO, clinical director, Sarcoma Program, Abramson Cancer Center, Penn Medicine, discusses the utility of tazemetostat (Tazverik) in epithelioid sarcoma.

Many of the novel drugs that are introduced to the sarcoma paradigm are targeted toward specific disease subtypes, Maki explains.

For example, in January 2020, the FDA granted an accelerated approval to tazemetostat for the treatment of patients with metastatic or locally advanced epithelioid sarcoma that is not eligible for complete resection.

The approval was based on findings from the phase 2 Study EZH-202 trial in which tazemetostat elicited a 15% objective response rate among 62 patients with epithelioid sarcoma.

Although the response rates were modest, epithelioid sarcoma is an exceedingly rare sarcoma subtype that requires new therapeutic options, Maki explains. Moreover, minimal adverse effects were reported with tazemetostat.

Going forward, novel combinations with tazemetostat in other tumor types where 

 mutations are present are anticipated, concludes Maki.

Robert G. Maki, MD, PhD, FACP, FASCO, discusses the utility of tazemetostat in epithelioid sarcoma.



Dr. Maki on the Utility of Tazemetostat in Epithelioid Sarcoma

Robert G. Maki, MD, PhD, FACP, FASCO, clinical director, Sarcoma Program, Abramson Cancer Center, Penn Medicine, discusses the potential utility of immunotherapy in sarcoma.

Immunotherapy, namely checkpoint inhibitors, are a novel and potentially effective therapy in the sarcoma space, Maki says.

A number of sarcoma subtypes, including undifferentiated pleomorphic sarcoma and angiosarcoma, appear to respond to checkpoint inhibitors, says Maki. 

However, checkpoint inhibitors do not appear to induce significant responses in more common subtypes, such as leiomyosarcoma and liposarcoma. Ultimately, additional research is needed to identify the optimal role of immunotherapy in sarcoma, Maki concludes.

Robert G. Maki, MD, PhD, FACP, FASCO, discusses the potential utility of immunotherapy in sarcoma.  



Dr. Maki on the Potential Utility of Immunotherapy in Sarcoma 

Robert G. Maki, MD, PhD, FACP, FASCO, clinical director, Sarcoma Program, Abramson Cancer Center, Penn Medicine, discusses the evolving treatment landscape of soft tissue sarcoma. 

In the current treatment paradigm, primary treatment for patients with soft tissue sarcoma should be given in the neoadjuvant setting rather than the adjuvant setting, Maki says. 

Historically, radiation and chemotherapy were given after patients underwent surgery, explains Maki.

However, neoadjuvant radiation and chemotherapy is now being utilized more frequently, particularly at centers that treat many patients with soft tissue sarcoma, says Maki. 

Although trials evaluating adjuvant therapy are ongoing, the paradigm appears to be shifting to a neoadjuvant approach, Maki says.

In the metastatic setting, the combination of doxorubicin and ifosfamide offers a reasonable option for patients who require a rapid response. Alternatively, patients who are not overly symptomatic should be considered for single-agent treatment, concludes Maki.

Robert G. Maki, MD, PhD, FACP, FASCO, discusses the evolving treatment landscape of soft tissue sarcoma.  



Robert G. Maki, MD, PhD, Penn Medicine

Articles

Dr. Maki on the Utility of IDH1/2 Inhibitors in Chondrosarcoma

Dr. Maki on the Utility of Tazemetostat in Epithelioid Sarcoma

Dr. Maki on the Potential Utility of Immunotherapy in Sarcoma

Dr. Maki on the Evolving Treatment Landscape in Soft Tissue Sarcoma