BACKGROUND: Squamous cell carcinoma of the nasal vestibule (SCCNV) is a rare disease, distinctly different in presentation, treatment, and outcome from squamous cell carcinoma (SCC) of the nasal cavity and paranasal sinuses. However, these are often not analyzed separately.
METHODS: The Netherlands Cancer Registry (NCR) and pathology reports from the Dutch Nationwide Pathology Databank (PALGA) were used to identify all newly diagnosed SCCNV cases in the Netherlands between 2008 and 2021.
RESULTS: A total of 763 patients were included. The yearly incidence rate displayed a significant downward trend with an annual percentage change (APC) of -3.9%. The 5-year overall survival (OS) and disease-free survival were 69.0% and 77.2%, respectively. The 5-year relative survival was 77.9% and improved slightly over the inclusion period. OS for patients who were staged cT3 appeared to be worse than those staged cT4a, calling the applicability of the TNM-classification into question.
CONCLUSIONS: SCC of the nasal vestibule is rare, with declining incidence rates. Introducing a specific topography code for SCCNV is recommended to enhance registration accuracy. The TNM classification seems poorly applicable to SCCNV, suggesting the need to explore alternative staging methods.

Frontal sinus involvement by malignant tumors is a rare finding. Therefore, a systematic literature review along with a personal case series may contribute to defining more accurately the epidemiology, treatment options, and outcomes of these neoplasms. This is a retrospective review of patients affected by frontal sinus malignancies surgically treated in a tertiary-care referral center over a period of 20 years. Moreover, a systematic literature review of studies describing frontal sinus cancers from 2000 to date was performed according to PRISMA guidelines in order to analyze current evidence about the treatment and outcomes of such a rare disease. Our retrospective review was basedon 84 cases, treated with an exclusive endoscopic approach in 43 cases (51.2%), endoscopic approach with frontal osteoplastic flap in 6 cases (7.1%), and transfacial or transcranial approaches in 35 cases (41.7%). The five-year overall, disease-specific, disease-free, and recurrence-free survivals were 54.6%, 62.6%, 33.1%, and 59.1%, respectively. Age, dural involvement, type of surgical resection, and surgical margin status were significantly associated with the survival endpoints. In conclusion, the involvement of the frontal sinus is associated with a poor prognosis. Multidisciplinary management, including specific histology-driven treatments, represents the gold standard for improving outcomes and minimizing morbidity.

UNASSIGNED: Biphenotypic sinonasal sarcoma (BSNS) is a rare low-grade cancer that was included from the 4th edition of WHO classification of head and neck tumours. The purpose of this study is to analyse clinical behaviour, pattern of recurrences and survival outcomes of this neoplasm.
UNASSIGNED: Retrospective review of patients affected by BSNS who were treated via an endoscopic-assisted approach in 6 European tertiary-care referral hospitals. Cases of BSNS described in literature since 2012 to date were fully reviewed, according to PRISMA guidelines.
UNASSIGNED: A total of 15 patients were included. Seven patients were treated via an endoscopic endonasal approach, 4 with endoscopic transnasal craniectomy, and 4 via a cranio-endoscopic approach. Adjuvant treatment was delivered in 2 cases. After a mean follow-up of 27.3 months, systemic metastasis was observed in 1 case; the 5-year overall survival and disease-free survival rates were 100% and 80 ± 17.9%, respectively.
UNASSIGNED: BSNS is a locally aggressive tumour with a low recurrence rate and encouraging survival outcomes if properly treated with surgical resection and free margins followed by adjuvant radiotherapy for selected cases. Endoscopic-assisted surgery is safe and effective as an upfront treatment within a multidisciplinary care protocol.
Sarcoma bifenotipico nasosinusale: case-series europeo multicentrico e revisione sistematica della letteratura.
UNASSIGNED: Il sarcoma nasosinusale bifenotipico (SNSB) è un raro tumore a basso grado, incluso a partire dalla 4° edizione WHO dei tumori testa-collo. L’obiettivo di questo studio è analizzare i tassi di sopravvivenza e i pattern di recidiva di questa neoplasia.
UNASSIGNED: Revisione retrospettiva dei pazienti affetti da SNSB, trattati mediante approccio endoscopico in 6 centri di riferimento europei. È stata condotta inoltre una revisione sistematica della letteratura dal 2012 ad oggi, secondo le linee guida PRISMA.
UNASSIGNED: Sono stati inclusi 15 pazienti (approccio endoscopico endonasale in 7 casi, craniectomia endoscopica transnasale in 4 casi, approccio combinato transcranico in 4 casi). In 2 casi è stata somministrata radioterapia adiuvante. Dopo un periodo di follow-up medio di 27,3 mesi, è stato riscontrato un caso di metastasi a distanza; i tassi di 5-year Overall Survival e Disease-Free Survival erano 100% e 80 ± 17,9%, rispettivamente.
UNASSIGNED: Il SNSB è un tumore localmente aggressivo con un basso tasso di recidiva e tassi di sopravvivenza incoraggianti se trattato con asportazione chirurgica radicale con radioterapia adiuvante per casi selezionati. La chirurgia endoscopica ha dimostrato di essere sicura ed efficace come trattamento iniziale all’interno di un protocollo di cura multidisciplinare.

Esthesioneuroblastoma (ENB) is a rare sinonasal malignant neoplasm with 40% 5-year survival. Because of the rarity of the tumor, the optimal treatment and subsequent prediction of prognosis are unclear. We studied a modern series of patients with ENB to evaluate the association of immunohistochemical (IHC) markers and clinical stages/grades with outcomes.
A single-center retrospective review of patients with ENB treated during a 25-year period was performed. A systematic literature review evaluating the prognostic benefits of current staging systems in evaluating survival outcomes in ENB was undertaken.
Among 29 included patients, 25 (85%) were treated surgically at our institution, with 76% of those endoscopically resected; 7 (24.1%) received chemotherapy, and 18 (62.1%) received radiation therapy. The 5-year overall survival (OS) was 91.3%, and 10-year OS was 78.3%. Progression-free survival at 5 and 10 years was 85.6% and 68.2%, respectively. A total of 36 distinct IHC markers were used to diagnose ENB but were inconsistent in predicting survival. A systematic literature review revealed predictive accuracy for OS using the Kadish, TNM, and Hyams staging/grading systems was 68%, 42%, and 50%, respectively.
This study reports the 5- and 10-year OS and progression-free survival in a modern series of patients with ENB. No traditional IHC marker consistently predicted outcome. Some novel reviewed markers show promise but have yet to enter clinical mainstream use. Our systematic review of accepted staging/grading systems also demonstrated a need for further investigation due to limited prognostic accuracy.

Surgery plays an important role in the treatment of sinonasal cancer. Many surgical approaches have been described, including open, endoscopic, or combined approaches. The choice is based on several criteria: general criteria related to the oncological results and morbidity of each technique, specific criteria related to the tumor (tumor extensions, tumor pathology), the patient, or the surgeon himself. The aims of this review are (i) to provide a complete overview of the surgical techniques available for the management of sinonasal malignant tumors, with a special focus on recent developments in the field of transnasal endoscopic surgery; (ii) to summarize the criteria that lead to the choice of one technique over another. In particular, the oncological outcomes, the morbidity of the different techniques, and the specificities of each histologic subtype will be discussed based on a comprehensive literature review.

OBJECTIVE: To review overall survival (OS), recurrence patterns, and prognostic factors of de novo sinonasal squamous cell carcinoma (DN-SCC).
METHODS: PubMed, Scopus, OVID Medline, and Cochrane databases from 2006 to December 23, 2020.
METHODS: The study followed the Preferred Reporting Items for Systematic Reviews and Meta-Analysis (PRISMA) guidelines. Articles were required to report either recurrence patterns or survival outcomes of adults with DN-SCC. Case reports, books, reviews, meta-analyses, and database studies were all excluded.
RESULTS: Forty-one studies reported on survival or recurrence outcomes. The aggregate 5-year OS was 54.5% (range, 18%-75%) from 35 studies (n = 1903). Patients undergoing open surgery were more likely to receive radiation therapy and present at an advanced stage compared to those receiving endoscopic surgery (all P < .001). Advanced T stage, presence of cervical nodal metastases, maxillary sinus primary site, and negative human papillomavirus (HPV) status were all correlated with significantly worse 5-year OS. Direct meta-analysis of 8 studies demonstrated patients with surgery were more likely to be alive at 5 years compared to those who did not receive surgery (odds ratio, 2.26; 95% CI, 1.48-3.47; P < .001). Recurrence was reported in 628 of 1471 patients from 26 studies (42.7%) with an aggregate 5-year locoregional control rate of 67.1% (range, 50.4%-93.3%).
CONCLUSIONS: This systematic review and meta-analysis suggests that the 5-year OS rate for DN-SCC may approach 54.5% and recurrence rate approaches 42.7%. In addition, various tumor characteristics including advanced T stage, positive nodal status, maxillary sinus origin, and negative HPV status are all associated with decreased survival.

The impact of elective neck treatment (ENT), whether by irradiation or dissection, on the prognosis of patients with cN0 sinonasal carcinomas (SNCs) remains an understudied issue. METHODS: A systematic review and meta-analysis of the literature were performed according to PRISMA guidelines in order to assess regional nodal relapse rate after ENT compared to observation in cN0 SNCs patients. Twenty-six articles for a total of 1178 clinically N0 patients were analyzed. Globally, the 5-year overall survival was 52%; 34.6% of patients underwent ENT and 140 regional recurrences were registered (5.9% in the ENT cohort and 15% in the observation group). ENT appears to confer a lower risk of regional recurrence compared to observation alone, with a cumulative OR of 0.38 (95% CI 0.25-0.58). Our meta-analysis supports the efficacy of ENT for reducing the risk of regional recurrence, but its overall impact on survival remains uncertain.

Background: Tumors originating from the craniofacial region usually present in a locally advanced stage with frequent involvement of adjacent sites and have a strong tendency for local recurrence in the absence of adjuvant therapy, even when the original surgical resection was presumed to be radical. In the past decades, several advances in the radiological diagnosis and treatment of craniofacial malignancies have been introduced. There are, however, no randomized trials that define the optimal multimodal treatment of these tumors because of their rarity as well as heterogeneity in both histology and site of origin. The aim of this study was to conduct a critical review of the role of adjuvant therapy in the treatment of craniofacial malignancy. Method: We conducted a critical review of the past and contemporary literature available, focusing on adjuvant oncological treatments of the most common craniofacial malignancies. Results: Preoperative radiotherapy can have a documented role in the treatment of olfactory neuroblastoma and soft tissue sarcoma, while preoperative chemotherapy can be advocated in the treatment of sinonasal undifferentiated carcinoma, neuroendocrine carcinoma, olfactory neuroblastoma, and craniofacial sarcoma (both soft-tissue and high-grade osteosarcoma). Postoperative radiotherapy has a well-established role in the treatment of most craniofacial malignancies. The role of postoperative chemotherapy is unclear in most histologies, but is commonly used during the treatment of well-selected cases of paranasal sinus carcinoma, olfactory neuroblastoma, mucosal melanoma, soft tissue sarcoma and high-grade craniofacial osteosarcoma. Discussion: Alongside developments in surgery, there have also been improvements in diagnostics, radiotherapy, and chemotherapy. Implementation of novel radiation techniques allows delivery of higher radiation doses while minimizing irradiation-related morbidity. Better understanding of tumor biology allows the construction of more complex treatment strategies, incorporating adjuvant chemotherapy either pre- or postoperatively. In the era of personalized targeted therapy, rapid strides are being made to identify specific tumor-targets for use of novel biologic agents, with the potential to change current management paradigms.

Postlaryngectomy sinonasal squamous cell carcinoma, being theoretically associated with exposure to the same risk factors or development of precancerous lesions due to altered nasal physiology, should warrant a thorough head and neck examination.

Sinonasal malignancies pose a significant challenge in management due to their low incidence, biologic diversity, and significant symptom burden. Even though surgery remains the primary therapeutic modality, a multi-modality approach has been shown to benefit a significant proportion of patients and its success depends largely on stage and histologic type. Non-surgical approaches such as novel radiation approaches as well as intensification with systemic therapy hold promise in altering the organ preservation rate as well as overall survival for patients. Practice changing randomized trials to test these novel modalities are overdue and desperately needed.