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.