Treatment Paradigm

Question 1

What is the Tx paradigm for RMS?

Answer 1

The Tx for RMS in the IRS studies varies based on site, histology, and tumor size. RMS Tx paradigm: generally, max safe resection (or Bx alone) → chemo +/- RT (timing of CRT depends on risk groupings)

Question 2

What chemo regimens are commonly used in RMS?

Answer 2

Vincristine/Actinomycin D/Cytoxan (VAC) and vincristine/actinomycin D (VA) are commonly used. Ifosfamide/Etoposide (IE) is also used in subsets of RMS.

Question 3

How does age factor into the prognosis of metastatic embryonal RMS?

Answer 3

>10 yo is worse than <10 yo (EFS 14% vs. 47%).

Question 4

What factors drive poor prognosis in PM RMS?

Answer 4

Subarachnoid space involvement with skull base erosion, CN palsy, intracranial extension; DFS 51% vs. 81% (without risk factors)

Question 5

Radiation doses for Favorable Histologies?

Answer 5

Question 6

Radiation doses for Unfavorable Histologies?

Answer 6

Question 7

Principles of Radiation therapy

Answer 7

Question 8

Principles of Surgery

Answer 8