From previous IRS studies the following was observed:
Group I patients in IRS III had a progression free survival of 70 - 80% at 3 years. Only histology had a significant effect on survival (alveolar tumors had a poorer survival than the botryoid/embryonal lesions).
Group II and III patients had 5 year survivals of about 60 to 70%.
An orbital primary is associated with a favorable outcome.
Group IV - Survival is poor (5 year survival IRS-I = 20%, IRS-II = 27%, IRS-III = 32%).
Patients with GU tumors had a significant survival advantage.
For patients with extremity RMS involvement of regional lymph nodes is associated with a poor outcome.
The translocation t(2;13)(q35;q14) or less commonly t(1;13)(p36;q14) occurs in about 70% of alveolar RMS but not in embryonal RMS.
In disseminated alveolar RMS, the translocation t(2;13)(q35;q14) is associated with a poorer prognosis.
High dose chemotherapy with bone marrow rescue is still considered experimental and to date has shown no advantage to chemotherapy alone.