1. CASE to be used for questions 1-6:
MP is a 42-year-old male referred for a perineal mass that he noticed about 2 months ago after riding his bike. After an initial work up, the mass was biopsied and the pathology sent to a sarcoma center. The center confirmed the diagnosis of epithelioid sarcoma with low-grade features and INI1 expression loss noted on pathology. Scans reveal a 2.1 cm, well-circumscribed, subcutaneous nodule with no regional lymph node involvement noted. MP’s other medications include lisinopril 20 mg daily, verapamil 180 mg daily, citalopram 20 mg daily, omeprazole 20 mg daily, and ibuprofen 200 mg every 4 hours as needed.

What is the MOST appropriate therapy for MP at this time?

2. MP is followed for 6 years but then scans reveal several small pulmonary nodules. His provider biopsied the nodules and found them to be recurrent metastatic epithelioid sarcoma. His provider decides to start him on tazemetostat 800 mg twice daily. Which of these is MOST concerning about MP starting tazemetostat right now?

3. What drug may need to be added to MP's medication list?

4. What should MP's provider monitor while he is on tazemetostat?

5. MP calls 3 days after starting tazemetostat to say that he vomited about 30 minutes after taking his morning dose, and he is unsure if the tablets came up. He wants to know if he should retake the dose. What is the BEST response to this question?

6. MP returns to clinic with his long-term female partner. She is 33 years old and of childbearing potential. She has read the material that MP was provided about tazemetostat and has questions about the pregnancy warnings. What is the BEST recommendation to provide this couple?

7. Which of the following is seen with most pathologic diagnoses of epithelioid sarcoma?

8. A basket trial evaluated tazemetostat for epithelioid sarcoma, allowing the investigators to assess which of the following?

9. AK is a 28-year-old female that began tazemetostat 800 mg twice daily about 4 months ago. She comes into the clinic today for a routine follow-up, and her laboratory values are as follows:

WBC 2.9K/microL (2.1-6.8 K/microL)
ANC 1728 cells/microL (1500-8000 cells/microL)
Hgb 11.2 g/dL (12-15.5 g/dL)
Platelets 26K/microL (150k-450k/microL)

Her provider decides to hold therapy until her platelets are above 75,000/microL and then restart tazemetostat with a reduction of 1 dose level.

What is the appropriate tazemetostat dose to re-initiate AK on at that time?

10. What is the role of SMARCB1 in cancer development?

Evaluation Questions

11. How confident are in your treatment choices for patient MP?

12. How confident are in your treatment choice for patient AK?

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