Table 1.

Criteria of disease response.

A. Complete or Partial Response/Stable or Progressive disease response system (8)
Disease response will be established by changed in at least 1 parameter, coded by using the following criteria  -Response by imaging  -Assessment of other clinical measures (quality of life)  -Clinical criteria and functional impairment Response was established by changes in at least 1 of these parameters
Complete Remission	No evidence of disease on radiologic imaging and
	No evidence of organ dysfunction due to disease and
	Normalization of quality of life criteria
Partial Response	>20% reduction in size of target vascular lesion evident on radiologic imaging or
	Improvement in target organ dysfunction by at least 1 grade or
	Improvement of self-report PedsQL by >4.4 or proxy-report PedsQL by >4.5 compared with baseline; FACT-G by >3.99
Progressive Disease	>20% increase in size of target vascular lesion evident on radiologic imaging or
	Worsening in target organ dysfunction by at least 1 grade or
	Worsening of self-report PedsQL by >4.4 or proxy-report PedsQL by >4.5 compared with baseline; FACT-G by >3.99
Stable Disease	None of the above
B. Good/Intermediate/Poor response system (7)
Response	Description
Good	Improvement in radiologic imaging findings of >70% or remnant lesion in radiologic imaging, but no gross lesion identified
Intermediate	Improvement in radiologic imaging findings of ≤70% and >30%
	Or self-reported improvement of gross lesion
Poor	Improvement in radiologic imaging findings of <30%, or stable disease status
	Or self-reported worsening of gross lesion