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KUANetal.

TABLE XXII.B.2 (Continued)

Clinical endpoints

Study

Year LOE Study design Study groups

Conclusions

Chemotherapy Choussy et al. 1196

2008 3

Multicenter

418 patients with ethmoid

Determination of risk factors and evaluation of treatment

Chemotherapy considered in advance cases

retrospective cohort

adenocarcinoma, divided into two groups: ITAC and poorly differentiated carcinoma ( n = 248) versus well-differentiated carcinoma ( n = 107). 215 recurrencies. 66 received chemo as part of the treatment for first recurrence

Knegt et al. 128

Surgical debulking plus repeated topical fluorouracil therapy, preferable over CFR for adenocarcinomas

1. DFS 2. Local

2001 3

Prospective cohort

62 adenocarcinoma patients (eight

relapse-free survival relapse-free survival

excluded) treated with surgery—no distinction between ITAC/non-ITAC

3. Distant

Abbreviations: CFR, craniofacial resection; DFS, disease-free survival; DSS, disease-specific survival; EEA, endoscopic endonasal approach; ITAC, intestinal-type adenocarcinoma; LRFS, locoregional failure/recurrence-free survival; OS, overall survival; RFS, recurrence-free survival.

although not specifically for the non-ITAC subtype. 1158 Kiliç et al. reported that tumors originating at the nasal cav ity had the greatest DSS. 53 These findings are summarized in Table XXII.B.3. Aggregate grade of evidence : C (Level 2: one study; Level 3: six studies; Level 4: five studies) D Adenoid cystic carcinoma Sinonasal ACC is a salivary gland tumor that originates from the nasal cavity and paranasal sinuses. It comprises 10%–25% of all cases of ACC of the head and neck. 1198 The most common site of origin is the maxillary sinus, followed by the nasal cavity, which together contribute to more than 60% of sinonasal ACC cases. 75,77,79,376,1198 Most patients (50%–80%) present with advanced-stage disease (T3–4) at the time of diagnosis, with high rates of PNI, bone invasion, and ASB involvement. 75,77,376,398,1198,1199 Surgical resection is considered the main treatment, as patients undergoing surgery have better survival. This is commonly followed by RT. 79,376 For unresectable cases, RT combined with or with out chemotherapy is usually recommended. 79 The 5-year OS is reported to range between 56% and 78%. 75,79,398,1200 However, sinonasal ACC is associated with high rates of local recurrence (up to 60%) and distant metastasis, even

compared to other malignant tumors of the paranasal sinuses. 77,79,398,1201–1203 Hence, obtaining local control is a major concern when planning the treatment goal for patients with sinonasal ACC. Due to the rarity of ACC, there are no clear guidelines available for management of these patients, especially in the sinonasal area. This section aims to review the evi dence regarding the importance of total gross resection, achieving negative surgical margins, and the role of adju vant radiotherapy in disease control. It also serves as an update to ICSB 2019 (Section VIII.D). 5,777 Role and extent of surgery It is widely accepted that surgical resection of sinonasal ACC significantly improves survival compared to RT or chemotherapy. 79,376,1200,1203 However, the extent of the resection and importance of achieving clear margins is poorly defined. Given the adjacent vital structures, the extent of surgery is particularly important. A meta-analysis with 99 patients with sinonasal ACC in 2013 revealed that invasion to adjacent structures including the orbit, dura, cavernous sinus, brain, muscles, or skin is com mon (81% of patients) and must be considered in surgical planning. 75 Sinonasal ACCs typically present as advanced- 1

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