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240

KUANetal.

TABLE XXIV.A.1 Evidence surrounding impact of Hyams grade on outcomes of ONB.

Clinical endpoints

Study

Year LOE Study design Study groups

Conclusions

33 studies; N = 128

DFS

Kadish stage C/D and Hyams grading III/IV significantly affected DFS

Meerwein et al. 1376

2021

2

Systematic

patients with ONB

reviewand meta analysis reviewand meta analysis

Vuong et al. 1377

2021

2

Systematic

33 studies; N = 492 withONB

1. RFS 2. MFS 3. OS

1. RFS and OS were higher in Hyams grade I and II but lower in Hyams III/IV 2. RT offered survival advantage in grades III/IV 3. Chemotherapy did not offer survival benefit to any Hyams subgroup 1. OS rates between low and high grades were as follows: 5-year 81.2% versus 60.9% and 10-year 64.0% versus 40.6% 2. 5- and 10-year OS HR between low versus high grades were 3.39 (95% CI: 2.09–5.49; p < 0.001) and 3.03 (95% CI: 1.82–5.06; p < 0.001) 5-year OS rates in patients with low-grade versus high-grade tumors were 86% versus 63% ( p = 0.1) 1. 5-year cumulative OS and DFS were 83% and 72%, respectively 2. 5-year OS for low Hyams grade versus high Hyams grade was 95% versus 61% ( p = 0.041) 3. High-grade and node-positive patient had worse DFS 1. Recurrence rate was 80% in surgery alone group, which came down to 43.7% if surgery was supplemented with other modalities 2. Survival rates significantly dropped with increasing tumor stage (63.6% in stages A and B vs. 30% in stages C and D) and grade (100% in grades 1/2 vs. 31.25% in grades 3/4) 1. PFS at 5, 10, and 15 years was 69%, 48%, and 27%, respectively 2. OS at 5, 10, and 15 years was 97%, 92%, and 83%, respectively Stage and Hyams grade did not influence survival

Goshtasbi et al. 1342

37 studies; N = 1088 withONB

OS

2019 2

Systematic

reviewand meta analysis

Ziai et al. 1345

2021

4

Retrospective case series

N = 43withONB

OS

Wolfe et al. 1346

2020 4

Retrospective case series

N = 39withONB

1. OS 2. DFS

Singh et al. 1347

2019 4

Retrospective case series

N = 21withONB

1. Recurrence 2. OS

Wertz et al. 248

2018 4

Retrospective case series

N = 41withONB

1. PFS 2. OS

Woods et al. 1378 5-year survival was 65% Stage and Hyams grade prognostic for survival Abbreviations: DFS, disease-free survival; ONB, olfactory neuroblastoma (esthesioneuroblastoma); OS, overall survival; PFS, progression-free survival. 2018 4 Retrospective case series N = 32withONB OS