Recurrent and Progressive Disease:
There were no differences in rates of recurrent or progressive disease at 3 and 6 months between the Siro and VCR treatment groups (Table 5). Time to recurrent or progressive disease from start of therapy or from initial treatment response were also equivalent between vincristine and sirolimus. Comparing all treatment groups (Supplemental Table 3), the rate of recurrent disease at 3 months was higher in patients treated with steroids alone (p=0.042), but across all treatment groups, there was no difference in rates of recurrent or progressive disease at 6 months. Although not statistically different from other groups, it was notable that of the 17 patients treated with surgery or IR embolization, 100% had a treatment response by 3 months, but five (29.4%) had recurrent disease within 6 months requiring initiation of systemic medical therapy.