3.2. Treatment
Systemic and local treatment given to patients in both protocols are
detailed in Table 2 . One hundred and forty-five (145) patients
who were enrolled in RMS2005 (94%) received chemotherapy according to
protocol, 8 patients received IVA without doxorubicin, and 1 patient
received IVA followed by VA. All patients included in ARST0531 received
chemotherapy according to protocol: 44 received VAC and 41 VAC/VI.
Radiotherapy to the site of primary tumor and nodes was administered in
132 (86%) and 82 (97%) patients enrolled in RMS2005 and ARST0531,
respectively (Table 2). A significantly higher proportion of patients in
EpSSG did not receive RT for the following reasons: parent refusal (2),
early progression (6), amputation (2), very young age (1) and treatment
center decision (7). Four patients received nodal RT only. The most
common type of radiotherapy in both series was photon external beam, and
a minority of patients in both protocols received proton beam
radiotherapy.
In the EpSSG cohort, 75 out of 154 patients (49%) underwent surgical
resection of the primary tumor: up-front primary resection in 10/75
(13%) and delayed primary resection in 65/75 (87%). Results of surgery
included complete local resection (R0) in 55 (73%), microscopic
residual disease (R1) in 14 (19%), and macroscopic residual (R2) in 6
(8%). In the COG cohort, 22 out of 85 patients (26%) underwent
surgical resection of the primary tumor: up-front primary resection in
17/22 (77%) and delayed primary resection in 5/22 (23%). R0 was
achieved in 20 (91%) and R1 in 2 (9%).