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?
A. Oral tazemetostat
B. Surgery alone
C. Surgery, followed by radiation and doxorubicin
D. Lymph node radiation, followed by doxorubicin
E. Unsure
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?
A. QT prolongation risk
B. Lisinopril use
C. Proton pump inhibitor use
D. Verapamil use
E. Unsure
3. What drug may need to be added to MP's medication list?
A. Prochlorperazine
B. Aspirin
C. Calcium carbonate
D. Phenytoin
E. Unsure
4. What should MP's provider monitor while he is on tazemetostat?
A. Complete blood count, transaminases, and triglycerides
B. Cholesterol levels, thyroid function, and weight gain
C. Renal function, triglycerides, and complete blood count
D. Thyroid function, blood glucose, and transaminases
E. Unsure
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?
A. Advise him to retake the dose now, as he likely vomited it up
B. Advise him to check the vomit for the tablets, and retake only if he sees them
C. Advise him to take his dose at the next scheduled time
D. Advise him to take half of the regular dose now
E. Unsure
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?
A. They should use a reliable form of contraception while MP is on tazemetostat and for 1 month after he stops therapy
B. They should use a reliable form of contraception while MP is on tazemetostat and for 3 months after he stops therapy
C. They should use a reliable form of contraception while MP is on tazemetostat and for 6 months after he stops therapy
D. It is safe for MP's partner to become pregnant, there is only risk to the fetus if MP's partner takes tazemetostat
E. Unsure
7. Which of the following is seen with most pathologic diagnoses of epithelioid sarcoma?
A. Loss of the SWI/SNF complex
B. Loss of EZH2 expression
C. Loss of INI1 expression
D. Loss of methylation
E. Unsure
8. A basket trial evaluated tazemetostat for epithelioid sarcoma, allowing the investigators to assess which of the following?
A. Tazemetostat use in several different cancers with the same mutation
B. Different dosing regimens of tazemetostat in epithelioid sarcoma
C. Use of different drugs for any benefit in epithelioid sarcoma
D. Tazemetostat's use in different soft tissue sarcomas, regardless of mutation
E. Unsure
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?
A. 600 mg twice daily
B. 600 mg once daily
C. 400 mg twice daily
D. 800 mg once daily
E. Unsure
10. What is the role of SMARCB1 in cancer development?
A. Inhibits EZH2
B. Encodes INI1
C. Inhibits Hedgehog pathway
D. Induces apoptosis
E. Unsure
Evaluation Questions
11. How confident are in your treatment choices for patient MP?
A. Not at all confident
B. Somewhat confident
C. Confident
D. Highly confident
12. How confident are in your treatment choice for patient AK?
A. Not at all confident
B. Somewhat confident
C. Confident
D. Highly confident