Sinonasal malignancies: Clinical and epidemiological aspects

Abstract

Chapter 1 introduces the anatomy of the nasal cavity and paranasal sinuses, and discusses malignancies at these sites, outlining current diagnostic and treatment practices. Chapter 2 presents a population-based cohort study on sinonasal squamous cell carcinoma (SCC) in the Netherlands from 2008 to 2021. It reports a slight increase in annual incidence (1.6% annual percentage change), with a 5-year overall survival (OS) of 43%. These findings highlight poor survival outcomes and the need for better treatments. Chapter 3 focuses on SCC of the nasal vestibule, noting distinct differences in clinical presentation, treatment, and outcomes. It found a downward trend in incidence and higher 5-year OS and disease-free survival (DFS) rates compared to SCC of the nasal cavity and paranasal sinuses, stressing the importance of treating these tumors separately. Chapter 4 evaluates brachytherapy for early-stage SCC of the nasal vestibule. A study of 68 patients over 17 years found 5-year locoregional recurrence-free survival (LRFS) of 91.1%, disease-specific survival (DSS) of 96.1%, and OS of 66.2%, suggesting brachytherapy as an effective and safe treatment. Chapter 5 discusses the variability in staging systems for nasal vestibule cancer, proposing the adoption of a new system by Bussu et al., which showed balanced patient allocation and improved staging accuracy. Chapter 6 assesses the Rome classification, which demonstrated statistically significant associations with survival outcomes, supporting its potential as a prognostic tool for nasal vestibule SCC, though further validation is needed. Chapter 7 shifts focus to sinonasal mucosal melanoma (MM), a rare and aggressive cancer with poor survival rates. A study of 320 patients between 2001 and 2021 found stable incidence rates and 5-year OS and relative survival (RS) of 24.5% and 32.4%, respectively, highlighting the need for novel treatments. Chapter 9 investigates PRAME, a potential diagnostic and therapeutic target in sinonasal MM. In a study of 26 patients, PRAME expression was found in all cases, suggesting its utility in diagnosis and as a target for therapy. Chapter 9 also examines sinonasal teratocarcinosarcoma, an aggressive malignancy with a poor prognosis. A retrospective analysis of three patients showed rapid local expansion and high risk of metastasis, underscoring the need for multimodal treatment. Finally, Chapter 10 presents a survey of sinonasal cancer experts, revealing significant variation in management practices and the lack of consensus on treatment approaches. This highlights the need for collaborative research and international guidelines to standardize care. These chapters provide a comprehensive clinical and epidemiological baseline, emphasizing the importance of multidisciplinary research and the need for innovative treatment strategies for sinonasal malignancies.