724 2017 10 55 10 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] 2013 9,(Chinese Children Cancer Group, CCCG), RMS 5 RMS, [7] :,,, IRSG EpSSG [4-6,8-14], 1. 2. <18 3. 4. DOI:10.3760 /cma.j.issn.0578-1310.2017.10.002 : (2014040) ; ( Z151100004015159) :,100045,Email:mxl1123@vip.sina.com 1. : 2. : B (MRI) CT CT, (PET-CT) 3. : 4. :,,,( ) 5.:,, (WHO) RMS : 1. (ERMS):, 11 2. (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),
2017 10 55 10 Chin J Pediatr, October 2017, Vol.55, No.10 725 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( ) 50.4 36.0 41.4 50.4 :IRS:
726 2017 10 55 10 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) >0.75 10 9 /L, (PLT) >100 10 9 /L 24 ~48 h, 0 0 B CT 4 2 VAC B 10 4 VAC B CT 12 13 5 VA - 16 6 VA B 19 7 VA - 22 8 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[0 3 6 9 h 2-360 mg /( m 2 ) + 20 ~30 min] ; <12, D ; 4, 4, 10 ; B CT ; - 0 0 B CT 4 2 VAC VI B 10 4 VAC VI B 13-16 5 VAC 19 6 VAC VI B 22 7 VAC - 25 8 VAC VI B 28 9 VAC - 31 10 VAC VI B 34 11 VAC - 37 12 VAC VI B 40 13 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)
2017 10 55 10 Chin J Pediatr, October 2017, Vol.55, No.10 727 0 0 B CT 4 2 VI B 10 4 VI B 13-16 5 VDC - 19 6 IE B 22 7 VDC - 25 8 IE B CT 28 9 VAC - 31 10 VI B 33 11 VDC - 36 12 IE B 39 13 VDC - 42 14 IE B 45 15 VAC - 48 16 VI B 51 17 VDC - 54 18 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 1. 1 3 0 0 1 1 VAI - B CT 4 2 VACa B 7 3 VDE - 10 4 VDI 12-13 5 VAI - B CT 16 6 VACa B 19 7 VDE - 22 8 VDI B CT 24-25 9 VAI - 28 10 VACa B 31 11 VDE - 33 12 VDI B 36 13 VAI - 39 14 VACa B 42 15 VDE - 45 16 VDI - :VAI: + D + ;VACa: + D + ;VDE: + +;MRI: ;VDI : + + ;VAI :, D:1.5 mg /m 2, d1, :3 g /m 2, d1 ~3[0 3 6 9 h 2-600 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, 12 24 36,;, B CT ; - X 2. 2 ~3 4 X 3. 4 6 X 4. 5 ~10 5.10 ( ) ( ) : ( ) ;( ) ;
728 2017 10 55 10 Chin J Pediatr, October 2017, Vol.55, No.10 ( ) ; ( ) ; ( ) ;( ) ; ( ) ; ( ) ; ( ) ; ( ) ; ( ) ; ( ) ;( ) ; ( ) ; ( ) ; ( ) [1] Rudzinski ER, Anderson JR, Hawkins DS, et al.the World Health Organization Classification of Skeletal Muscle Tumors in Pediatric Rhabdomyosarcoma: A Report From the Children s Oncology Group [ J].Arch Pathol Lab Med, 2015, 139 ( 10 ) : 1281-1287.DOI: 10.5858 /arpa.2014-0475-oa. [2] PDQ Cancer Information Summaries [ M ]. Bethesda ( MD ) : National Cancer Institute ( US),2002. [3]. [ M]. :, 2001:1-6. [4] Huh W. Vincristine, Dactinomycin, and Lower Doses of Cyclophosphamide With or Without Radiation Therapy for Patients with Newly Diagnosed Low-Risk Embryonal /Botryoid /Spindle Cell Rhabdomyosarcoma [ EB /OL ]. ( 2012-10-12 ) [ 2017-01- [5] Huh W. Intensive Multi-Agent Therapy, Including Dose- Compressed Cycles of Ifosfamide /Etoposide ( IE) and Vincristine / Doxorubicin /Cyclophosphamide ( VDC ) for Patients with High- Risk Rhabdomyosarcoma [ EB /OL ]. ( 2012-10-31 ) [ 2017-01- [6] Huh W. Randomized Study of Vincristine, Dactinomycin and Cyclophosphamide ( VAC ) versus VAC Alternating with Vincristine and Irinotecan ( VI) for Patients with Intermediate- Risk Rhabdomyosarcoma [ EB /OL ]. ( 2012-10-21 ) [ 2017-01- [7] Ma X, Huang D, Zhao W, et al.clinical characteristics and prognosis of childhood rhabdomyosarcoma: a ten-year retrospective multicenter study[ J].Int J Clin Exp Med, 2015,8(10) :17196-17205. [8] Walterhouse DO, Pappo AS, Meza JL, et al.shorter-duration therapy using vincristine, dactinomycin, and lower-dose cyclophosphamide with or without radiotherapy for patients with newly diagnosed low-risk rhabdomyosarcoma: a report from the Soft Tissue Sarcoma Committee of the Children s Oncology Group [ J].J Clin Oncol, 2014,32(31) :3547-3552.DOI: 10.1200 / JCO.2014.55.6787. [9] Rosenberg AR, Anderson JR, Lyden E, et al.early response as assessed by anatomic imaging does not predict failure-free survival among patients with Group III rhabdomyosarcoma: a report from the Children s Oncology Group[ J].Eur J Cancer, 2014,50(4) : 816-823.DOI: 10.1016 /j.ejca.2013.11.031. [10] Weiss AR, Lyden ER, Anderson JR, et al. Histologic and clinical characteristics can guide staging evaluations for children and adolescents with rhabdomyosarcoma: a report from the Children s Oncology Group Soft Tissue Sarcoma Committee[ J].J Clin Oncol, 2013, 31 ( 26 ) : 3226-3232.DOI: 10.1200 /JCO. 2012.44.6476. [11] Dharmarajan KV, Wexler LH, Wolden SL.Concurrent radiation with irinotecan and carboplatin in intermediate -and high-risk rhabdomyosarcoma: a report on toxicity and efficacy from a prospective pilot phase II study[ J].Pediatr Blood Cancer, 2013, 60(2) :242-247.DOI: 10.1002 /pbc.24205. [12] Wolden SL, Lyden ER, Arndt CA, et al. Local Control for Intermediate-Risk Rhabdomyosarcoma: Results From D9803 According to Histology, Group, Site, and Size: A Report From the Children s Oncology Group[ J].Int J Radiat Oncol Biol Phys, 2015,93 ( 5 ) : 1071-1076.DOI: 10.1016 /j.ijrobp.2015.08. 040. [13] Weigel BJ, Lyden E, Anderson JR, et al.intensive Multiagent Therapy, Including Dose-Compressed Cycles of Ifosfamide / Etoposide and Vincristine /Doxorubicin /Cyclophosphamide, Irinotecan, and Radiation, in Patients With High-Risk Rhabdomyosarcoma: A Report From the Children s Oncology Group[ J].J Clin Oncol, 2016, 34 ( 2 ) : 117-122.DOI: 10. 1200 /JCO.2015.63.4048. [14] Ben AM, Minard-Colin V, Mosseri V, et al.does aggressive local treatment have an impact on survival in children with metastatic rhabdomyosarcoma? [ J ]. Eur J Cancer, 2015, 51 (2) :193-201.DOI: 10.1016 /j.ejca.2014.11.009. ( :2017-01-05) (: ),,, 1. : (http:// www.cma.org.cn) (http://www.medline.org.cn) 2. :, 3.:,, ;,