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ICAR SINONASAL TUMORS

TABLE XXIII.A.2 Evidence surrounding role of radiation therapy in pediatric rhabdomyosarcoma.

Clinical endpoints

Study

Year LOE Study design Study groups

Conclusions

Merks et al. 1225

OS

1. 5-year OS 71.4% versus 49.6% ( p < 0.0001) 2. RT as part of initial treatment is beneficial for PM-RMS

10 studies, RT

2014 2

Systematic

( n = 1004) versus no RT( n = 89)

reviewand meta analysis

Raney et al. 1224

2002 3

Prospective cohort

Risk-stratified

OS

1. 5-year OS 73% for patients receiving RT 2. 5-year EFS 67%

protocol, all received localRT

Whole-brain RT variably

administered for high-risk meningeal involvement ( n = 526) Whole-brain RT versus no whole-brain RT in PM-RMS with high-risk meningeal involvement ( n = 360)

Raney et al. 1228

OS

No additional benefit to whole-brain RT for localized PM-RMS with CNP, SBE, or ICE

2002 3

Prospective cohort

Crist et al. 1228

2001 3

Prospective cohort

Conventional RT ( n = 251) versus

OS

No difference between conventional and hyperfractionated RT for IRSG group 3 RMS

hyperfractionated RT( n = 239)

Abbreviations: CNP, cranial nerve palsy; EFS, event-free survival; ICE, intracranial extension; OS, overall survival; RMS, rhabdomyosarcoma; RT, radiation therapy; SBE, skull base erosion.

Of note, while these are pediatric studies, some trials have included young adults in their treatment with an age cutoff of 21. Table XXIII.A.3 outlines the trials and their most rele vant findings. Within a single trial, patients may be treated with the same chemotherapy protocol (i.e., prospective cohort) or randomized to different chemotherapy proto cols (i.e., RCT). Results specific to tumor site are pre sented when available, and comparisons are summarized below: - VAC versus intensified protocols: two RCTs com pared VAC to intensified chemotherapy protocols that included doxorubicin. 1227,1232 These groups included a majority of PM-RMS and showed no survival benefit to additional chemotherapy. One RCT compared VAC to VAC/VTC for intermediate-risk PM-RMS with no difference in survival. 1233 - VAI/VAIA versus CEVAIE: one RCT compared VAI to an intensified six-drug chemotherapy protocol without difference in survival. 1234 In another RCT, VAIA was compared to CEVAIE. 1231 For PM-RMS tumors, there was a significant difference in EFS (combined outcome including recurrence, progression, or death). There was no difference in OS, and within the entire cohort that

included other sites, there was no significant difference in EFS and OS. - VAC versus VAI: one RCT compared VAC to VAI to a third chemotherapy protocol. 1226 There was no signifi cant difference in survival between the three regiments. - Intrathecal chemotherapy: one study reviewed effects of increased doses of intrathecal chemotherapy for high risk PM-RMS. Intrathecal chemotherapy consisted of methotrexate, hydrocortisone, and cytarabine. It was administered for patients with cranial nerve palsies, intracranial extension, or skull base erosion. From 1978 to 1987 (IRS-II and early IRS-III), the dosing proto col consisted of 13 courses of intrathecal chemotherapy. From 1987 to 1991 (IRS-III), the protocol was adjusted by age and the total duration was decreased to four courses. After 1991 (IRS-IV), no intrathecal chemotherapy was administered. When comparing zero to seven to 13 doses of chemotherapy, there was no difference in OS between the three groups. 1224 In summary, the available evidence shows similar sur vival with VAC- or VAI-based chemotherapy protocols. Intensified chemotherapy protocols have not demon strated additional survival benefit. While sinonasal RMS was represented within PM-RMS, extrapolation should be