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KUANetal.

TABLE XXIII.A.1 Evidence surrounding role of surgery in pediatric rhabdomyosarcoma.

Clinical endpoints

Study

Year LOE Study design Study groups

Conclusion

Kanaet al. 1238

1. OS 2. Margin status

Four had R0 resection, one underwent debulking Two had positive margins

2022 4

Retrospective case series

Five of 12 pediatric RMS patients

underwent primary surgery

Siddiqui

2019 4

Retrospective database study (NCDB) Retrospective case series

CRT( n = 83) versus CRT + surgery ( n = 48) CRT( n = 6) versus CRT + primary surgery ( n = 8)

OS

1. No OS difference between those receiving surgery and those who didnot 2. Timing of surgery not specified Patients with sinonasal RMS who underwent surgery had higher OS, but this was not statistically significant

et al. 1229

Fyrmpas

OS

2009 4

et al. 1230

Abbreviations: CRT, chemoradiation therapy; OS, overall survival; RMS, rhabdomyosarcoma.

tionation of RT have differed according to study group protocol. RT was given to all patients with the exception of certain patients in MMT-84 and MMT-89 trials who were very young, had a low risk of meningeal involve ment, or had a complete response after chemotherapy. In a systematic review of these trials, there was significant survival difference between those who received RT and those who did not. 1225 Additionally, the IRS-IV trial ran domized IRSG group 3 parameningeal RMS (PM-RMS) to conventional and hyperfractionated regimens. There was no significant survival difference between the two arms. 1226 A single study examined whole-brain irradiation, which was used according to high-risk PM-RMS features. In the IRS trials, there was a gradual move away from whole brain irradiation. In the early trial period of IRS-II, patients with cranial nerve palsy, skull base erosion, or intracranial extension received whole-brain and spine RT. In late IRS II and IRS-III trials, whole-spine RT was omitted. Also, in IRS-III, IRSG groups 1 and 2 had whole-brain RT omitted, unless there was intracranial extension. In IRS-IV, whole brain RT was omitted for all. Raney et al. demonstrated no survival benefit with the addition of whole-brain RT for cranial nerve palsies, skull base erosion, or intracranial extension (Table XXIII.A.2). 1224 Role of radiation therapy in pediatric rhabdomyosarcoma

Harm

Acute and long-term radiation complications. Risk of secondary malignancy for pediatric patients.

Cost

Additional cost of RT.

Benefits–harm assessment

Preponderance of benefits over harms.

Value

Vast majority of sinonasal RMS are higher risk (IRSG 2 or 3) and unlikely to have complete tumor clearance with surgery alone. Failure to show survival benefit with

judgments

use of whole-brain radiation despite hypothetical benefit of reducing local recurrence.

Policy level Recommendation. Intervention Primary RT, with or without chemotherapy, for pediatric sinonasal RMS is first-line therapy. Whole-brain radiation for high-risk PM-RMS is not recommended.

4 Role of chemotherapy in pediatric rhabdomyosarcoma

The body of evidence for treatment of pediatric RMS has come from large clinical trial groups: initially IRSG, later Children’s Oncology Group (COG, North America), Coop erative Weichteilsarkom Studiengruppe (CWS, Europe), and International Society of Pediatric Oncology Malignant Mesenchymal Tumors group (MMT, Europe). These trials have often used different risk-stratified chemotherapy pro tocols according to recurrence risk, and RMS tumors with similar risk were grouped together into a stage. Staging has differed between trial groups and has changed over time within the same trial group. Most often, sinonasal RMS has been grouped with other PM-RMS or with RMS from other body sites with a similar histology and IRS group.

Aggregate grade of evidence

B (Level 2: one study, Level 3: three studies)

Benefit

Improved survival with use of RT in primary treatment modality.

(Continued)