Sarcoma CME ACCREDITED Watch Time: 22 mins

touchEXPERT OPINIONS Early recognition and emerging therapies for epithelioid sarcoma

Watch an international sarcoma expert discuss the diagnosis of and developing treatment options for epithelioid sarcoma, a rare sarcoma subtype.

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Expert Interview
What are the fundamental challenges in advanced epithelioid sarcoma?

Dr Ravin Ratan introduces epithelioid sarcoma and describes the diagnostic and treatment challenges facing patients and clinicians.

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Interview Questions

In this interview, Dr Ravin Ratan answers the following questions:

  • What is the presentation and prognosis of epithelioid sarcoma?
  • What are the diagnostic challenges associated with epithelioid sarcoma?
  • What are the current treatment options for localized and operable advanced epithelioid sarcoma?
  • What are the unmet needs in inoperable advanced/ metastatic epithelioid sarcoma?
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Expert Interview
What is the pathophysiology of epithelioid sarcoma and how is it used for diagnosis and treatment?

Dr Ravin Ratan describes the biology of epithelioid sarcoma and discusses how its etiology provides therapeutic opportunities.

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Interview Questions

In this interview, Dr Ravin Ratan answers the following questions:

  • What are the roles of INI1 and EZH2 in healthy cells?
  • How is INI1 expression lost in epithelioid sarcoma?
  • What is the effect of INI1 loss on EZH2 and how can EZH2 be targeted therapeutically?
  • Are there other molecular changes in epithelioid sarcoma that could be useful for future treatment options?
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Expert Interview
What are the new and emerging therapies for patients with epithelioid sarcoma?

Dr Ravin Ratan assesses recent progress and future treatment options for patients with epithelioid sarcoma.

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Interview Questions

In this interview, Dr Ravin Ratan answers the following questions:

  • What are the most recent advances in the treatment of epithelioid sarcoma?
  • What do clinicians need to know about using tazemetostat for epithelioid sarcoma?
  • What clinical evidence supports checkpoint inhibitors in the treatment of epithelioid sarcoma?
  • The treatment landscape of epithelioid sarcoma is evolving. Which approaches are you most excited about?
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Overview & Learning Objectives
Overview

Dr Ravin Ratan, a sarcoma expert from the MD Anderson Cancer Center, discusses challenges surrounding the diagnosis of epithelioid sarcoma and provides an update on the latest treatment options.

This activity has been jointly provided by Oakstone and touchIME ONCOLOGY. Oakstone Publishing is accredited by the ACCME to provide continuing medical education to physicians. read more

Target Audience

This activity has been designed to meet the educational needs of oncologists involved in the management of patients with epithelioid sarcoma.

Disclosures

Oakstone Publishing has assessed conflict of interest with its faculty, authors, editors, and any individuals who were in a position to control the content of this CME activity. Any identified relevant conflicts of interest were resolved for fair balance and scientific objectivity of studies utilized in this activity. Oakstone Publishing’s planners, content reviewers, and editorial staff disclose no relevant commercial interests.

Faculty

Dr Ravin Ratan discloses: Grants/research support from Epizyme and Springworks Therapeutics. Consultant/Advisory Boards for Epizyme and Springworks Therapeutics.

Content Reviewer

Walter Murray Yarbrough, MD, FACP, has no financial interests/relationships or affiliations in relation to this activity.

Touch Medical Director

Hannah Fisher has no financial interests/relationships or affiliations in relation to this activity.

Requirements for Successful Completion

This activity has been planned and implemented in accordance with the accreditation requirements and policies of the Accreditation Council for Continuing Medical Education (ACCME) through the joint providership of Oakstone Publishing and touchIME. Oakstone Publishing is accredited by the ACCME to provide continuing medical education for physicians.

The European Union of Medical Specialists (UEMS) – European Accreditation Council for Continuing Medical Education (EACCME) has an agreement of mutual recognition of continuing medical education (CME) credit with the American Medical Association (AMA). European physicians interested in converting AMA PRA Category 1 Credit™ into European CME credit (ECMEC) should contact the UEMS (www.uems.eu).

Oakstone Publishing designates this enduring material for a maximum of 0.25 AMA PRA Category 1 Credits™. Physicians should claim only the credit commensurate with the extent of their participation in the activity.

In order to receive credit for this activity, participants must review and complete the post-test and evaluation form. Statements of credit are awarded upon successful completion of the post-test and evaluation form.

Date of original release: 18 February 2021. Date credits expire: 18 February 2022.

Learning Objectives

After watching this activity, participants should be better able to:

  • Recognize the challenges in the diagnosis and treatment of advanced epithelioid sarcoma
  • Discuss the importance of SMARCB1 alterations, INI1 loss and other molecular changes in the diagnosis and treatment of patients with epithelioid sarcoma
  • Outline the efficacy and safety of current and emerging targeted therapies in patients with epithelioid sarcoma
About Dr Ravin Ratan
Dr Ravin Ratan

MD Anderson Cancer Center, Houston, TX, USA

Ravin Ratan is Assistant Professor in the Department of Sarcoma Medical Oncology at MD Anderson Cancer Center, Houston, Texas, USA. His academic and research interests focus on sarcoma; he has been Principal or Co-principal Investigator for several phase I–III trials in different sarcoma subtypes, and he has published extensively in the field of sarcoma.

He is a member of the American Society of Clinical Oncology and the Connective Tissue Oncology Society.

Dr Ravin Ratan discloses: Grants/research support from Epizyme and Springworks Therapeutics. Consultant/Advisory Boards for Epizyme and Springworks Therapeutics.
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This Activity Is Funded By:

An independent medical education grant from Epizyme.
This activity is provided by touchIME.

This activity has been planned and implemented in accordance with the accreditation requirements and policies of the Accreditation Council for Continuing Medical Education (ACCME) through the joint providership of Oakstone Publishing and touchIME. Oakstone Publishing is accredited by the ACCME to provide continuing medical education for physicians.

The European Union of Medical Specialists (UEMS) – European Accreditation Council for Continuing Medical Education (EACCME) has an agreement of mutual recognition of continuing medical education (CME) credit with the American Medical Association (AMA). European physicians interested in converting AMA PRA Category 1 Credit™ into European CME credit (ECMEC) should contact the UEMS (www.uems.eu).

Oakstone Publishing designates this enduring material for a maximum of 0.25 AMA PRA Category 1 Credits™. Physicians should claim only the credit commensurate with the extent of their participation in the activity.

Unapproved products or unapproved uses of approved products may be discussed by the faculty; these situations may reflect the approval status in one or more jurisdictions. The presenting faculty have been advised by touchIME to ensure that they disclose any such references made to unlabelled or unapproved use. No endorsement by touchIME of any unapproved products or unapproved uses is either made or implied by mention of these products or uses in touchIME activities. touchIME accepts no responsibility for errors or omissions.

This content is intended only for healthcare professionals.

Date of original release: 18 February 2021. Date credits expire: 18 February 2022.

This content is intended for healthcare professionals only. Please confirm that you are a healthcare professional.

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Question 1/4
Which of the following complicates the diagnosis and prognosis of epithelioid sarcoma?
 Correct

Epithelioid sarcoma is often diagnosed at an advanced stage because it is rare, and tumours are usually small, slow growing, painless and histopathologically heterogenous.1,2 The immunohistochemical profile of epithelioid sarcomas also often includes expression of both carcinoma markers (e.g. cytokeratin and epithelial membrane antigen) and sarcoma markers (e.g. vimentin).2

References
1. Czarnecka AM, et al. Cancers. 2020;12;2112.
2. Needs T, Fillman EP. Epithelioid Sarcoma. In: StatPearls [Internet], Treasure Island: StatPearls Publishing; 2020.

Question 2/4
How often does loss of integrase interactor-1(INI1) expression occur in epithelioid sarcoma?
Correct

Loss of integrase interactor-1 (INI1) expression occurs in around 90% of epithelioid sarcomas.1

Reference
1. Czarnecka AM, et al. Cancers. 2020;12;2112.

Question 3/4
Which of the following are genetic aberrations associated with epithelioid sarcoma?

ALK, anaplastic lymphoma kinase; BCR-ABL1, breakpoint cluster region-abelson tyrosine protein kinase 1; EGFR, epidermal growth factor receptor; MET, mesenchymal epithelial transition factor; PI3K/AKT/mTOR, phosphatidylinositol-3 kinases/protein kinase-B/mechanistic target of rapamycin; SMARCB1, SWI/SNF related, matrix associated, actin dependent regulator of chromatin, subfamily B, member 1.
 Correct

SMARCB1 alterations occur in around 50% of patients with epithelioid sarcoma; and PI3K/AKT/mTOR activation, EGFR overexpression and MET activation have also been identified in patients with epithelioid sarcoma.1

EGFR, epidermal growth factor receptor; MET, mesenchymal epithelial transition factor; PI3K/AKT/mTOR, phosphatidylinositol-3 kinases/protein kinase-B/mechanistic target of rapamycin; SMARCB1, SWI/SNF related, matrix associated, actin dependent regulator of chromatin, subfamily B, member 1.

Reference
1. Czarnecka AM, et al. Cancers. 2020;12;2112.

Question 4/4
Based on the results of a recent phase II study, in which range was the objective response rate for tazemetostat in adults with locally advanced or metastatic INI1-negative epithelioid sarcoma?

INI1, integrase interactor-1
 Correct

In a recent phase II study of tazemetostat in adults with locally advanced or metastatic INI1-negative epithelioid sarcoma, objective response was observed in 9 of 62 patients (15%).1

INI1, integrase interactor-1

Reference
1. Gounder M, et al. Lancet Oncol. 2020;21:1423–32.

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