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KUANetal.

TABLE XXII.D.1 Evidence for management of sinonasal mucoepidermoid and acinic cell carcinoma.

Clinical endpoints

Study

Year LOE Study design Study groups

Conclusion

Auger et al. 411

Cases of sinonasal MEC( n = 239)

OS

1. Achieving negative surgical margins is associated with improved OS, although this is not statistically significant 2. In this group, adjuvant RT is associated with improved survival 1. Insurance status, advanced T stage, and nodal stage were associated with worse OS 2. Treatment modality and sequence were not associated with survival DSS of sinonasal AciCC did not significantly differ from parotid AciCC. Large tumor size, high mitotic count, mixed anatomic site, high tumor grade, and higher stage are associated with worse DSS Patients with MEC had improved OS compared to those with ACC

2020 4

Retrospective database analysis (NCDB)

Triantafillou et al. 1217

2019 4

Retrospective database analysis (NCDB)

Cases of sinonasal MEC( n = 164)

OS

Biron et al. 1220

5- and 10-year:

Matched cohorts of cases of parotid gland AciCC and sinonasal AciCC ( n = 18) Cases of sinonasal MEC( n = 19) Comparison of cases of maxillary sinus MEC( n = 7) and ACC( n = 18) Comparison of cases of maxillary sinus non-SCC versus SCC( n = 188)

2014 4

Retrospective

case–control analysis of

1. OS 2. DSS

theSEER database

Wolfish et al. 1218

2011

4

Retrospective case series

DSS

da Cruz Perez et al. 1219

DSS

2006 4

Retrospective case series

Bhattacharyya 1216

2003 4

Retrospective

OS

1. Cases of non-SCC carcinoma had worse OS 2. MEC cases had median OS of 53months

case–control analysis of

theSEER database

Abbreviations: ACC, adenoid cystic carcinoma; AciCC, acinic cell carcinoma; DSS, disease-specific survival; MEC, mucoepidermoid carcinoma; NCDB, National Cancer DataBase; OS, overall survival.

The 5-and 10-year OS was noted to 82.1% and 52.3% and the DSS was 100% and 88.9%, respectively. Biron et al. also performed a grouped analysis of the previously doc umented cases in the literature and found an estimated 10-year RFS of 92.9%. Table XXII.D.1 summarizes evidence for management of sinonasal MEC and AciCC.

teen percent of these occur in the head and neck, compared to 40% of pediatric RMS. 1222 Different subtypes of RMS have been recognized, with new subtypes added over time. In the 5th edition of the WHO classification of soft tis sue tumors there are four recognized subtypes: embryonal, alveolar, sclerosing/spindle cell, and pleomorphic. 17 Scle rosing RMS is exceedingly rare, and pleomorphic RMS is generally treated like a non-RMS soft tissue sarcoma. Fur ther subtypes have been described based on specific gene fusions. 1223 The embryonal subtype is the most common subtype. It usually occurs in children under 5 years old, although it can arise in adults as well. Botryoid RMS is considered a morphological subtype of this subtype, which presents as a polyp and has a better prognosis. Alveolar is the most undifferentiated subtype and is more aggressive than other subtypes. It is most common in adolescents and young adults. 1223

XXIII

SINONASAL SARCOMA

A Rhabdomyosarcoma Rhabdomyosarcoma (RMS) is overall a rare primary tumor. It is more common in the pediatric population, representing 7% of childhood cancers, and it is the most common extracranial soft tissue tumor of children. 1221 In contrast, RMS represents 1% of adult malignancies. Nine