RMS, RMS. (rhabdomyosarcoma,rms) (Intergroup RMS Study Group,IRSG) ( European Pediatric soft tissue sarcomas study group,epssg)

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1 Chin J Pediatr, October 2017, Vol.55, No.10 ( CCCG-RMS-2016) (rhabdomyosarcoma,rms), 6.5% [1-3], RMS,, (Intergroup RMS Study Group,IRSG) ( European Pediatric soft tissue sarcomas study group,epssg) 20,, RMS 3,,,, RMS [1,4-6] ,(Chinese Children Cancer Group, CCCG), RMS 5 RMS, [7] :,,, IRSG EpSSG [4-6,8-14], < DOI: /cma.j.issn : ( ) ; ( Z ) :,100045, mxl1123@vip.sina.com 1. : 2. : B (MRI) CT CT, (PET-CT) 3. : 4. :,,,( ) 5.:,, (WHO) RMS : 1. (ERMS):, (ARMS): ARMS t(2;13)(q35;q14) t(1;13)(q36;q14) PAX3-FKHR PAX7-FKHR,PAX3-FKHR 3. ( pleomorphic or anaplastic RMS):, ( 1) (IRS) -( 2),

2 Chin J Pediatr, October 2017, Vol.55, No TNM ( cm) 1 a T 1 T 2 5 >5 N 0 N 1 N X M 0 2 b T 1 T 2 5 N 0 N X M 0 3 T 1 T 2 5 >5 N 1 N 0 N 1 N X M 0 4 T 1 T 2 5 >5 N 0 N 1 M 1 : a ( ) ; b, ;T 1 : ;T 2 :, ; N 0 :;N 1 :;N x : ; M 0 : ;M 1 : - Ⅰ Ⅱ Ⅲ Ⅳ,,,(, ) Ⅰa Ⅰb,, a b Ⅱa,, Ⅱb,, Ⅱc,,, Ⅲa Ⅲb, c, (, ) : a ; b ; c TNM, ( 3), TNM IRS 1 Ⅰ ~Ⅲ 2 ~3 Ⅰ ~Ⅱ 2 ~3 Ⅲ 1 ~3 Ⅰ ~Ⅲ 4 Ⅳ a 3 ~4 Ⅲ ~Ⅳ :IRS: ; a 1. :,,,,, ( ) 3 ~6 (4 ~8 ),, 2. :RMS IRS-Ⅰ,Ⅱ ~ Ⅳ, Ⅰ TNM 1, 50.4 Gy,, RMS 4 :,, (Gy) IRS-Ⅰ 0 IRS-Ⅰ 36.0 IRS-Ⅱa 36.0 IRS-Ⅱb Ⅱc ( ) 41.4 IRS-Ⅲ( ) 45.0 IRS-Ⅲ( ) :IRS:

3 Chin J Pediatr, October 2017, Vol.55, No.10 1 ;,,,,, 13, 25, 3. : :(1), ;,, 7 d 1, PAX3-FKHR PAX7-FKHR, (2) D( ACTD) (ADR), (3), ( 5 ~8) 2.0 mg,actd 2.5 mg 4 ~6, 12 12, (4): <12 12 kg, = /30 ( kg), 21 d (ANC) > /L, (PLT) > /L 24 ~48 h, 0 0 B CT 4 2 VAC B 10 4 VAC B CT VA VA B 19 7 VA VA - :VAC: + D +;VA: + D; MRI: ; VAC VA : : 1.5 mg /m 2, d1 d8 d15, D:0.045 mg /( kg ) + 5 min,d1,:1.2 g /m 2 1 h,d1[ h mg /( m 2 ) + 20 ~30 min] ; <12, D ; 4, 4, 10 ; B CT ; B CT 4 2 VAC VI B 10 4 VAC VI B VAC 19 6 VAC VI B 22 7 VAC VAC VI B 28 9 VAC VAC VI B VAC VAC VI B VAC - :VAC: + D +;VI: + ;MRI: ;VAC ;VI :, 50 mg /m 2, d1 ~5, 90 min, 100 mg /d,, UGT1A1 ; 42, 4 ~6, 13, 12 ; 12,; B CT ; - ( G-CSF) ( GM- CSF) >28 d 25% (5) : NCI (CTCAE version 4.0,2009) (ALT) < 2, < 1.5 (Ccr) (6) [25 mg / (kg d), 2 /d], 3 d, 3 1. :(1) :, CT B CT 2 : B, RMS,,, CT (2) : B CT CT, (3) 1, (4)

4 Chin J Pediatr, October 2017, Vol.55, No B CT 4 2 VI B 10 4 VI B VDC IE B 22 7 VDC IE B CT 28 9 VAC VI B VDC IE B VDC IE B VAC VI B VDC IE B CT :VAC: + D +;VI: + ;VDC: + +;IE: + ;MRI: ;VAC VI ;VDC IE :, 30 mg /m 2,d1 ~2, 1.2 g /m 2 1 h,d1, 1.8 g /m 2,d1 ~5, 100 mg /m 2,d1 ~5; 54, 12 12,; - (CR) ( PR) (PD), 6 ~8 2. : CR: 4, PR: 64%, 30%, PD: 40%, 20%, ( SD): PD PR 3.:CR: CR, PR: CR, SD, PR: CR, PR SD, PR: PR, CR PR SD, SD: SD, CR PR SD, PD: PD VAI - B CT 4 2 VACa B 7 3 VDE VDI VAI - B CT 16 6 VACa B 19 7 VDE VDI B CT VAI VACa B VDE VDI B VAI VACa B VDE VDI - :VAI: + D + ;VACa: + D + ;VDE: + +;MRI: ;VDI : + + ;VAI :, D:1.5 mg /m 2, d1, :3 g /m 2, d1 ~3[ h mg /( m 2 ) ] ;VACa : D VAI, 560 mg /m 2,d1;VDE :, : 25 mg /m 2, d1 ~2, 150 mg / ( m 2 ), d1 ~3;VDI : +, 3 g /m 2,d1 ~3; 24,,25 ~48 ; 24,, VDC( + +) IE( + ) ; 48, 12, ,;, B CT ; - X 2. 2 ~3 4 X X 4. 5 ~ ( ) ( ) : ( ) ;( ) ;

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