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227

ICAR SINONASAL TUMORS

TABLE XXIII.A.4 Evidence surrounding role of surgery in adult rhabdomyosarcoma.

Clinical endpoints

Study

Year LOE Study design Study groups

Conclusions

Kanaet al. 1238

1. OS 2. Margin status

1. Surgery associated with improved DMFS 2. Eight of 13 patients had R0 resection 3. Surgical salvage employed for five patients CR: 100% and metastases: 20%

2022 4

Retrospective cohort

Eight of 17 adult RMS patients underwent primary surgery

Barthere

2020 4

Retrospective case series

Surgery with R0

1. CR 2. Metastases

et al. 1249

resection ( n = 5), adjuvant RT in adult PM-RMS Surgery for resectable tumor with adjuvant therapy and possible salvage surgery in Chemo alone ( n = 21) versus surgery alone ( n = 25) versus RT alone ( n = 107) versus surgery and RT( n = 55) CRT( n = 10) versus CRT + surgery ( n = 6) Surgery ( n = 1) versus surgery + RT( n = 3) versus CRT ( n = 10) both adult and pediatric RMS ( n = 40)

Li et al. 1237

OS

46.5% OS in cohort treated with upfront surgical approach for resectable tumors

2019 4

Retrospective case series

Unsal et al. 119

2017 4

Population based database study (SEER)

DSS

1. 5-year DSS: 17.7% versus 50.8% versus 36.7% versus 35.9% ( p = 0.0595) 2. Surgery alone group had best survival but not statistically significant No difference in rate of recurrence or death with surgery in addition toCRT 1. LRC: 0% versus 66% versus 100%; distant control: 100% versus 33% versus 30% 2. Small single-institution case series without statistical analysis

Thompson et al. 1235

1. Recurrence 2. Mortality

2013 4

Retrospective case series

Sercarz et al. 1236

1994 4

Retrospective case series

1. LRC 2. Distant control

Abbreviations: CRT, chemoradiation therapy; DSS, disease-specific survival; LRC, locoregional control; LRFS, locoregional failure/recurrence-free survival; OS, overall survival; RMS, rhabdomyosarcoma.

with sinonasal RMS underwent surgery. Compared to the pre-IMRT era, there was a lower locoregional recur rence rate with IMRT. In the IMRT group, a single sinonasal locoregional recurrence was observed in the study population. 1239 Most other studies involved com bined CRT modalities. In general, chemotherapy protocols for adult sinonasal RMS have been adapted from pediatric clinical trial pro tocols (i.e., vincristine based). Chemotherapy was most frequently administered with radiation. Three studies examined the 5-year OS of adult sinonasal RMS to be between 20.6% and 31.8%, notably lower than that of pedi atric sinonasal RMS. 1240–1242 A majority of patients in these studies received combined chemotherapy and radiation. The remaining studies identified were retrospective case series that examined chemotherapy response or survival and recurrence outcomes. Analysis of these studies was

limited due to variable chemotherapy protocols, small case numbers, and short follow-up periods (Table XXIII.A.5). Role of chemoradiation therapy in adult rhabdomyosarcoma

Aggregate grade of evidence

C (Level 4: nine studies)

Benefit

Definitive treatment option for local, regional, and distant disease. Acute and long-term CRT side effects.

Harm

Cost

Cost of CRT administration. Balance of benefits and harms

Benefits–harm assessment

(Continued)