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KUANetal.

TABLE XXII.B.3 (Continued)

Clinical endpoint

Study

Year LOE Study design Study groups

Conclusion

5-year OS of 65.9% for non-ITAC patients

Outcomes of endoscopic resection

Bhayani

2014 4

Retrospective case series

66 patients, 31 of whomhad non-ITAC

et al. 1158

Huber et al. 263

2011

4

Retrospective case series

20 patients with AC, five of whom had non-ITAC 29 cases of low-grade non-ITAC

1. RFS 2. DSS

Three patients died of disease (two high-grade, one low-grade), two patients alive without disease 16 patients with follow-up information (median 16 months), none with recurrent disease

Joet al. 1173

2009 4

Retrospective case series

Association of low-grade tubular sinonasal ade nocarcinomas with respiratory epithelial

adenomatoid hamartomas

Orvidas et al. 1159

2005 4

Retrospective case series

24 patients, 14 of whomhad non-ITAC

Histologic

1. 5-year OS of 58% 2. Patients with high-grade tumors 5.4 times more likely to die than patients with low-grade tumors 11 patients alive with no evidence of disease 36–108 months after diagnosis, one dead of other reasons

characteristics and outcomes

Netoet al. 1169

Clinical find

2003 4

Retrospective case series

12 patients with seromucous

ings/pathologic fea tures/histologic differential diagnosis

adenocarcinomas

Abbreviations: DFS, disease-free survival; DSS, disease-specific survival; ITAC, intestinal-type adenocarcinoma; OS, overall survival; RFS, recurrence-free survival; RT, radiation therapy.

stage tumors in the majority of cases and, regardless of surgical technique, the rate of microscopic or gross pos itive margins is very high. In older studies, the surgical strategy for sinonasal ACC was an open approach with aggressive resection using wide margins to obtain total resection of the tumor. RT as the sole treatment was usu ally reserved for unresectable tumors. 377,1198,1203,1204 In a series of 35 patients by Rhee et al., surgical treatment was directed by subsite in the paranasal sinuses and included medial maxillectomy, subtotal maxillectomy, CFR for skull base involvement, or orbital exenteration for periorbital involvement. 1198 Nasopharynx tumors were considered surgically unresectable. The reported 5-year OS and DFS were 86% and 51%, respectively, with 30% local recurrence rate and 25% distant metastasis rate. Time to local recur rence was 51 ± 64 months after treatment, and time to distant metastasis was 37 ± 33 months. 1198 Pitman et al. reported on a series of 35 patients treated with open CFR and adjuvant RT for treatment of sinonasal ACC. 1204 They performed GTR, except for where tumor abutted major neurovascular structures. In these cases, they tolerated positive margins. Overall, 46% had microscopic positive margins. With a 71% overall recurrence and 36% local

recurrence, analysis failed to reveal PNI, margin status, or tumor grade as predictors of survival despite an aggres sive treatment strategy. Only 25% of recurrences survived longer than 24 months with salvage treatment. Given the high recurrence rate despite relatively high local control rate (65%), they considered treatment of sinonasal ACC palliative and recommended avoiding major morbidity during treatment. While complete surgical resection with negative mar gins is often not feasible due to advanced disease at pre sentation and anatomic restrictions, other reports suggest a significant effect of achieving negative margins on the sur vival of patients. An analysis of 51 patients prospectively followed with ACC of the skull base revealed a signifi cant role of achieving negative margins at the first surgical intervention. 1205 Even with piecemeal resection, there was an OS advantage of 20.1 ± 3.3 years compared with resections that left residual disease, even if microscopic (10.3 ± 1.6years, p = 0.035). Notably, microscopic negative margins demonstrated a survival advantage compared to any positive margins. The surgical strategy included open or endoscopic approaches (in more recent cases) with the aim of achieving total resection without causing major