Key points

Introduction

Sinonasal tumors are rare diseases, accounting for 3% to 5% of head and neck malignant neoplasms and the 0.2% to 0.8% of all tumors. There are several histologic subtypes with different natural histories. The most frequent tumors of this region have epithelial origin and poor prognosis, such as squamous cell carcinoma, intestinal-type adenocarcinoma (ITAC), undifferentiated carcinoma, and neuroendocrine carcinoma (NEC). Although there are several staging systems, none are ideal or universally used. However, stage at presentation is generally highly predictive of survival and, despite a maximum treatment of the primary tumor, local, regional, or distant recurrences can occur even after many years. From a surgical standpoint, the introduction of craniofacial resection (CFR) in the 1960s represented a significant advance in the care of these patients and has served as the mainstay for their treatment for the past 50 years. However, this approach has been associated with perioperative mortality and major complications in 0% to 13% and 35% to 63% of patients, respectively. The advances in endoscopy have revolutionized the management of sinonasal and skull base lesions. Many complex cancers that traditionally required open approaches are now amenable to purely endoscopic endonasal resection, providing less invasive surgery with lower morbidity but with comparable oncologic outcomes in terms of survival rates. The endoscopic endonasal approach has become accepted with precise indications for the treatment of selected skull base cancers. Therefore, at present, external traditional and endoscopic approaches should not be considered as two competing techniques, but rather as different approaches useful for suitable cases, performed in centers with extensive experience, according to the oncologic principle of radicality. At present, the surgical strategy has to be driven by the cancer histology and its extension rather than the available surgical expertise and equipment, and therefore surgeons have to be equally comfortable in managing patients by open craniofacial as well as endoscopic approaches. So far, no standard and uniform protocols of treatment of such aggressive tumors have been reported, given their rarity, heterogeneity in histology and stages of diseases, and in the absence of prospective studies. Surgery followed by radiotherapy (RT) has been generally adopted as the usual treatment strategy. However, some studies also explored the role and feasibility of induction chemotherapy and the prognostic value of the response to it in several histotypes. Recently, heavy-ion therapy using proton or carbon ion beams has been introduced in the treatment of these tumors as exclusive therapy or in the postoperative setting with encouraging outcomes. Proton/carbon ion beam therapy, compared with conventional photon therapy, provides a more accurate and intense dose to the tumor area, with potentially greater control of disease. Moreover, this therapy may produce less toxic side effects in particularly critical areas exposed to late RT toxicities and potentially can help in organ preservation strategies for locally advanced cases, especially to avoid orbital exenteration. In this scenario, even in the absence of prospective data, the integration of multiple modalities of treatment tailored to the histology; molecular profile; and, in selected cases, to the response to induction chemotherapy seems to be the best approach for these rare and aggressive cancers. This article discusses the current evidence for the multimodal management of sinonasal and anterior skull base (ASB) cancers, focusing on the different treatment protocols driven by histologic subtypes. Preoperative work-up, indications and exclusion criteria, surgical techniques, and postoperative management are analyzed. Oncologic outcomes stratified according to histology are presented and future directions for the management of these cancers are discussed.