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Background: Despites a tripling of recognised occupational sinonasal adenocarcinomas in the past three decades, no comprehensive publications of the epidemiological development and histological distribution of sinonasal malignancies exist in Germany. Therefore, this study aims to analyse population-related data and address gaps in the nationwide cancer registry., Methodology: The German Center for Cancer Registry Data (ZfKD) provided a dataset covering the period from 2003 to 2015. 8332 cases were extracted, morphologically clustered and underwent descriptive analysis. Incidence and mortality rates were standardised, and overall survival (OS) probability was estimated., Results: Standardised incidence rates increased, averaging 1.8 for men and 1.0 for women per 100,000 inhabitants. Sinonasal cancer manifested mostly in the nasal cavity with a 5-year OS of 63%. In contrast, malignancies in the frontal sinus had a less favourable survival probability (p < 0.001). Morphologically, the tumour entities comprised 55% squamous cell carcinomas, 18% adenocarcinomas, 8% mucosal melanomas, 4% esthesioneuroblastomas and 2% sinonasal undifferentiated carcinomas (SNUC). The highest 5-year OS rate was observed in esthesioneuroblastomas (59%), while SNUC (38%) and mucosal melanoma (29%) had the lowest (p < 0.001)., Conclusions: This study provides a comprehensive epidemiological analysis of sinonasal morphologies based on a unique dataset. Findings reveal a higher incidence and lower survival probability among men. The topographical distribution varies by sex and tumour entity. The mortality rate for patients with sinonasal cancer is moderate to high, particularly for SNUC and mucosal melanoma, with incidences of 8.4% and 2%, respectively. Hence, there is an urgent need for an optimised screening for early-stage malignancies., Competing Interests: Declarations. Conflict of interest: The authors declare no competing interests. Ethics approval and consent to participate: An application for an ethical review was submitted to the Ethics Committee of the University of Luebeck, but was not considered necessary due to the anonymization of the dataset (File number: 20-410)., (© 2024. The Author(s).)

Background: With modern treatment paradigms, olfactory neuroblastoma (ONB) has favorable overall survival (OS); however, the incidence of recurrence remains high. The primary aims of this study were to delineate the prognosis of recurrence of ONB and explore how recurrence subsites are associated with OS, disease-specific survival (DSS), and further recurrence., Methods: A retrospective chart review of ONB cases from nine academic centers between 2005 and 2021 was completed. Tumor characteristics, recurrence subsites, timelines to recurrence, additional recurrences, and survival estimates were determined using descriptive and time-to-event analyses., Results: A final cohort of 233 patients was identified, with 70 (30.0%) patients recurring within 50.4 (standard deviation ±40.9) months of diagnosis on average, consisting of local (50%), neck (36%), intracranial (9%), and distant (6%) recurrence. Compared with subjects without recurrence, patients with recurrence had significantly different primary American Joint Committee on Cancer T stage (p < 0.001), overall stage (p < 0.001), and modified Kadish scores (p < 0.001). Histopathology identified that dural involvement and positive margins were significantly greater in recurrent cases. First recurrence was significantly associated with worse 5-year DSS (hazard ratio = 5.62; p = 0.003), and subjects with neck or local recurrence had a significantly better DSS compared to intracranial or distant recurrence., Conclusions: Recurrent cases of ONB have significantly different stages and preoperative imaging factors. Patients with local or neck recurrence, however, have better DSS than those with intracranial or distant recurrence, independent of initial tumor stage or Hyams grade. Identifying specific factors that confer an increased risk of recurrence and DSS is important for patient counseling in addition to surveillance planning., (© 2024 ARS‐AAOA, LLC.)

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., Conclusion: 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., (© 2024 The Authors. Head & Neck published by Wiley Periodicals LLC.)

Objective: Sinonasal malignant tumors (SNMT) are relatively rare among head and neck malignant tumors. Most are squamous cell carcinomas, and malignant melanomas, olfactory neuroblastomas, adenoid cystic carcinomas, sarcomas, and others also occur. The most common primary site of nasal sinus squamous cell carcinoma is the maxillary sinus. In recent years, a decrease in incidence of maxillary sinus squamous cell carcinoma (MSSCC) has been reported along with a decrease in the incidence of sinusitis. MSSCC is treated with a combination of surgery, radiation, and chemotherapy. Treatment decisions are made according to the progression of the disease, the patient's general condition, and the patient's own wishes. There are variations in treatment policies among facilities due to the specialty of staff and cooperation with other departments at each facility. We conducted a multi-institutional retrospective study to compare outcomes by treatment strategy., Methods: In this study, 340 patients with SNMT who were treated at 13 Hospitals (Head and Neck Oncology Group (Kyoto-HNOG) ) during the 12-year period from January 2006 to December 2017 were included. There were 220 patients with squamous cell carcinoma, 32 with malignant melanoma, 21 with olfactory neuroblastoma, and 67 with other malignancies. Of the squamous cell carcinomas, 164 were of maxillary sinus origin. One hundred and forty cases of MSSCC that were treated radically were included in the detailed statistical analysis., Results: There were 5 cases of cStage I, 9 cases of cStage II, 36 cases of cStage III, 74 cases of cStage IVa, and 16 cases of cStage IVb. There were 92 cases without clinical lymph node metastasis (cN(-)) and 48 cases with clinical lymph node metastasis(cN(+)). Primary tumors were treated mainly by surgery in 85 cases (Surg) and by radical radiation therapy (with or without chemotherapy) of 6-70 Gy in 55 cases(non-Surg). The 5-year overall/disease-free survival rate (OS/DFS) for MSSCC was 65.1%/51.6%. Old age, renal dysfunction, and clinical T progression were independent risk factors for OS, and renal dysfunction was an independent risk factor for DFS. In cN(-) patients, OS and DFS were significantly better in Surg group than in non-Surg group. In cN(+) patients, there was no significant difference in OS and DFS between Surg and non-Surg groups., Conclusion: For patients with MSSCC without lymph node metastasis, aggressive surgery on the primary tumor contributes to improved prognosis., Competing Interests: Declaration of Competing Interest The authors declare that they have no competing interests, (Copyright © 2023. Published by Elsevier B.V.)

Objectives: Extraosseous plasmacytoma (EOP) is a rare plasma cell neoplasm that tends to convert to plasma cell myeloma (PCM) in about 11% to 35% of cases. It has a predilection for the upper respiratory tract, prototypically affecting the nasal cavity and paranasal sinuses. Contemporary first-line treatment is radiotherapy, with more recent studies showing an added benefit of combining radiation with surgery. In this cohort study, we aimed to examine clinical presentation, treatment, and prognosis for all patients nationwide from 1980 through 2017. Furthermore, we determined the size and extension of tumors, investigating the rate at which minimally invasive surgery would have been possible., Methods: Patients were found in the national pathology registry, and all biopsies were collected for pathology review by a hematopathologist. We performed survival statistics for overall survival (OS), progression-free survival (PFS), and the cumulative incidence of conversion to PCM., Results: Twenty-three patients were included. The median age was 65, and patients were primarily men (78%). Tumors were located in either the nasal cavity (57%), maxillary sinus (39%), or sphenoid sinus (4%). In most cases, the tumor was <5 cm (65%) without extension to adjacent structures (60%). The national incidence was 0.02/100,000 person-years, the median symptom duration until diagnosis was 5 months, and none of the patients presented with contiguous spread to regional lymph nodes. Stand-alone radiotherapy was the predominant treatment (61%). In the entire cohort, one patient died from the initial disease, and six patients died from either relapse of EOP or PCM. The 5-year OS, PFS, and conversion rate to PCM were 78%, 56%, and 23%, respectively., Conclusion: SN-EOP responds well to radiotherapy, but relapse and conversion to PCM were not uncommon and entailed a poor prognosis. Most tumors were endoscopically resectable and non-invasive, making the majority of tumors suitable for surgery as an addition to radiation.

Background: Aim of this study is to investigate the prevalence of cervical nodal metastasis at presentation and as disease relapse in primary, treatment-naive olfactory neuroblastoma (ONB), and to review treatment modalities, risk factors for regional failure and survival outcomes according to nodal status., Methods: A systematic review and proportion meta-analysis were conducted following PRISMA guidelines based on PubMed, Web of Science, and Scopus., Results: Eighteen articles were examined. The pooled proportion of patients with nodal metastasis at presentation (11.5%) was comparable to that of cN0 patients not receiving elective neck treatment developing nodal metastasis during follow-up (12.3%). Of the latter, most were Kadish stage C tumors (85.5%)., Conclusions: Cervical involvement is common both at presentation and during follow-up of cN0 ONB. The highest risk of developing late nodal metastasis is seen in cN0 patients with Kadish stage C tumors not receiving elective neck treatment. Elective cN0 neck treatment should be encouraged in selected patients to increase regional control., (© 2023 Wiley Periodicals LLC.)

Background: In China, reports on the epidemiology of adenocarcinomas of the nasal cavity and paranasal sinuses are limited., Aim: This study aimed to describe the experience of a single institution in China in treating these malignant tumours., Materials and Methods: We conducted a retrospective chart review of patients with adenocarcinoma of the nasal cavity and paranasal sinuses admitted between January 2019 and December 2021. Tumour staging was based on the American Joint Committee on Cancer, 8th edition, for sinonasal malignancies., Results: The series included 10 men and 4 women (mean age, 54.5 [range, 14-80] years). Epistaxis and nasal obstruction were the most common clinical manifestations in 10 (71.4%) patients. Ten (71.4%) had stage T4 disease at diagnosis, but no patient had lymph node or distant metastasis. The posterosuperior septum (100.0%) and middle turbinate (92.8%) were the two sites most vulnerable to tumour invasion. All patients underwent endoscopic resection as the initial treatment; one patient died., Conclusions and Significance: In China, these malignancies are related to exposure to certain substances; however, diagnosis can be delayed. Endoscopic resection is a suitable treatment option for adenocarcinomas of the nasal cavity and paranasal sinuses.

Sinonasal malignancies make up <5% of all head and neck neoplasms, with an incidence of 0.5-1.0 per 100,000. The outcome of these rare malignancies has been poor, whereas significant progress has been made in the management of other cancers. The objective of the current review was to describe the incidence, causes, presentation, diagnosis, treatment, and recent developments of malignancies of the sinonasal tract. The diagnoses covered in this review included sinonasal undifferentiated carcinoma, sinonasal adenocarcinoma, sinonasal squamous cell carcinoma, and esthesioneuroblastoma, which are exclusive to the sinonasal tract. In addition, the authors covered malignances that are likely to be encountered in the sinonasal tract-primary mucosal melanoma, NUT (nuclear protein of the testis) carcinoma, and extranodal natural killer cell/T-cell lymphoma. For the purpose of keeping this review as concise and focused as possible, sarcomas and malignancies that can be classified as salivary gland neoplasms were excluded., (© 2022 The Authors. CA: A Cancer Journal for Clinicians published by Wiley Periodicals LLC on behalf of American Cancer Society.)

Objectives: To examine the epidemiology, subtypes, trends over time, and predictive factors for recurrence and malignant transformation of sinonasal papillomas., Methods: A retrospective chart review of 118 patients with sinonasal papillomas from 2009 to 2019 was conducted at the University of California, Los Angeles. This study is a follow-up to a previously published study from 2000 to 2009 at the same academic center., Results: The mean age was at presentation was 58.5 years, with a 2:1 male to female ratio, and average follow-up of 30.1 months. The rate of recurrence after complete resection was 19% with an average of 32.6 months to recurrence. The time to recurrence followed a bimodal distribution with 57% of cases recurring within 24 months (mean = 10) and 43% from 40 to 103 months (mean = 61). The proportion of the inverted papillomas rose from 38% in 2000-2004 to 89.6% in 2015-2019. Patients presenting at a younger age had a higher chance of recurrence (mean age 52 with recurrence vs. 61 without recurrence). Age did not correlate with histopathologic transformation in surgical pathology. Furthermore, histopathological transformation did not raise the chance of recurrence. Smoking, alcohol use, chronic rhinosinusitis, and allergic rhinitis were not associated with any of the outcome measures in this study. The most significant factor predicting recurrence, beside age at presentation, was the history of two or more prior sinus surgeries for papillomas or other reasons (OR = 3.52 and 5.81)., Conclusion: This study explored the features of sinonasal papillomas as well as the risk factors for recurrence and transformation. Younger age at presentation and two or more prior surgeries for papillomas were associated with recurrence. Time to recurrence followed a bimodal distribution, with late recurrences happenning from 40 to 103 months after surgery, emphasizing the importance of long-term follow-up for timely resection of tumors and prevention of malignancy.

Background: Non-squamous cell carcinoma sinonasal malignancies (NSCCSM) are relatively rare. Neoadjuvant radiotherapy and/or chemotherapy (NTx) have been proposed to improve outcomes compared to surgery alone. In this study, we aim to examine the prevalence of NTx utilization and associated outcomes., Methods: A retrospective study utilizing the National Cancer Database, 2004 to 2015. The study population included adult patients diagnosed with primary NSCCSM., Results: A total of 574 patients were included. The mean age of the study population was 61.7 ± 16.5 years. The median follow-up time was 40.4 months (interquartile range: 15.3-81.3 months). The histopathological diagnoses identified included: (i) 37.0% adenocarcinoma, (ii) 22.8% adenoid cystic carcinoma, (iii) 20.0% mucosal melanoma, (iv) 11.9% esthesioneuroblastoma, and (v) 8.2% sinonasal undifferentiated carcinoma (SNUC). NTx was utilized in 70 (12.20%) of the study population. Patients who received NTx were more likely to have SNUC or esthesioneuroblastoma ( P  < .01 each) and to have stage III or IV disease ( P  < .01 each). NTx was most likely to be administrated in a high-volume center [OR: 3.94, 95%CI: (1.47, 10.53), P  = .006]. Patients who received NTx had a significantly lower prevalence of positive margin postoperatively [OR: 0.48, 95%CI: (0.26, 0.87), P  = .016]. In patients with NSCCSM, negative margin was associated with improved overall survival [HR: 0.55, 95%CI: (0.36, 0.82), P  = .004]., Conclusions: This study provides an epidemiological perspective regarding NSCCSM and related practice patterns and survival outcomes. Neoadjuvant radiotherapy and/or chemotherapy is likely to decrease the risk of positive margin which ultimately could improve survival in this population.

Background: Since adenocarcinoma of the ethmoid sinuses was first described as an occupational disease in the woodworkers of High Wycombe, over the 50 subsequent years there has been a gradual decrease in the numbers to none over the last 12 years. Although this mirrored the decline in local industry, it seems the causative factor was first seen and then disappears over a 50-year period., Methods: A total of 146 cases have been traced historically over this time as well as personal experience of 33 cases; these cases are reviewed and success with a new modality of treatment is discussed., Conclusion: This paper outlines how the disease was initially recognised, both its diagnosis and treatment development. It also describes how both the appearance and disappearance were seemingly caused by changes in manufacturing practice.

Objectives/hypothesis: To analyze the clinical features, classification, and treatment of adult nasopharyngolaryngeal hemangioma (ANPLH)., Study Design: Retrospective study., Methods: From February 2009 to May 2020, 101 patients with ANPLH were reviewed and analyzed., Results: Symptoms of ANPLH were frequently displayed as abnormal pharyngeal sensation and functional defection. According to lesion location, ANPLH was divided into five categories including nasopharyngeal, oropharyngeal, hypopharyngeal, laryngeal, and mixed types. The mixed type constitutes the highest portion, and the nasopharyngeal type is the least in our cohort. Most lesions could resect through natural cavity under endoscopy. Patients with mixed lesions had a higher rate of postoperative recurrence and planned multiple surgeries. Acceptable but not severe intraoperative and postoperative complications occurred in our patient cohort., Conclusions: Patients with ANPLH are always symptomatic and even functional defective, which can be classified into five categories based on lesion location. For these patients, endoscopic surgery through natural cavity is recommended to remove lesions with fewer complications and favorable clinical outcomes., Level of Evidence: 4 Laryngoscope, 131:2724-2728, 2021., (© 2021 The American Laryngological, Rhinological and Otological Society, Inc..)

Objective: To assess clinical outcomes of newly diagnosed extranodal NK/T-cell lymphoma nasal-type (ENKTL) patients treated with L-asparaginase-containing chemotherapy as first-line chemotherapy., Materials and Methods: We retrospectively reviewed data of new ENKTL patients in Siriraj Hospital, Thailand during 2012-2016., Results: Among 27 patients, 16 were men and 11 were women, and their median age was 52 (range, 25-83) years. The primary sites were the aero-nasal area (23) and skin (4). Clinical stages of ENKTL were I, II, III and IV in 10, 8, 1 and 8 patients, respectively. Patients classified according to the NK cell-lymphoma prognostic index with PINK (27) and PINK-E (19) as low, intermediate, and high were 14, 8, 5, respectively and 11, 4, and 4, respectively. Chemotherapy regimens used were SMILE (24) and AspaMetDex (3). Fourteen patients received post- chemotherapy local radiation. The overall response rate for 26 assessable patients was 69%, with complete response in 14 patients (52%). Median progression-free and overall survival were 32 and 33 months, respectively. The most common L-asparaginase regimen related complications were hypofibrinogenemia (24) and hypersensitivity (9). The overall mortality at follow-up was 14/27 (52%) owing to sepsis (11) and disease progression (3)., Discussion: L-asparaginase-based regimens showed similar results as other study results. including those in which hematopoietic stem cell transplantation was performed, suggesting that transplantation can be avoided if it is unaffordable. Thrombosis or bleeding, the regimen side effects, should be carefully monitored during treatment., Conclusions: L-asparaginase-based regimens offer a good outcome as a front-line treatment for ENKTL.

Background: Nasal intestinal-type adenocarcinomas (ITAC) are strongly related to chronic wood dust exposure: The intestinal phenotype relies on CDX2 overexpression but underlying molecular mechanisms remain unknown. Our objectives were to investigate transcriptomic and methylation differences between healthy non-exposed and tumor olfactory cleft mucosae and to compare transcriptomic profiles between non-exposed, wood dust-exposed and ITAC mucosa cells., Methods: We conducted a prospective monocentric study (NCT0281823) including 16 woodworkers with ITAC, 16 healthy exposed woodworkers and 13 healthy, non-exposed, controls. We compared tumor samples with healthy non-exposed samples, both in transcriptome and in methylome analyses. We also investigated wood dust-induced transcriptome modifications of exposed (without tumor) male woodworkers' samples and of contralateral sides of woodworkers with tumors. We conducted in parallel transcriptome and methylome analysis, and then, the transcriptome analysis was focused on the genes highlighted in methylome analysis. We replicated our results on dataset GSE17433., Results: Several clusters of genes enabled the distinction between healthy and ITAC samples. Transcriptomic and IHC analysis confirmed a constant overexpression of CDX2 in ITAC samples, without any specific DNA methylation profile regarding the CDX2 locus. ITAC woodworkers also exhibited a specific transcriptomic profile in their contralateral (non-tumor) olfactory cleft, different from that of other exposed woodworkers, suggesting that they had a different exposure or a different susceptibility. Two top-loci (CACNA1C/CACNA1C-AS1 and SLC26A10) were identified with a hemimethylated profile, but only CACNA1C appeared to be overexpressed both in transcriptomic analysis and in immunohistochemistry., Conclusions: Several clusters of genes enable the distinction between healthy mucosa and ITAC samples even in contralateral nasal fossa thus paving the way for a simple diagnostic tool for ITAC in male woodworkers. CACNA1C might be considered as a master gene of ITAC and should be further investigated., Trial Registration: NIH ClinicalTrials, NCT0281823, registered May 23 d 2016, https://www.clinicaltrials.gov/NCT0281823 ., (© 2021. The Author(s).)

We evaluated the outcome of 65 French patients with Extranodal NK/T-cell lymphoma, nasal type (ENKTL) undergoing hematopoietic stem cell transplantation (HSCT) (19 allogeneic and 46 autologous). Fifty-four patients (83%), most of which receiving L-asparaginase (L-aspa) containing regimens (81%), achieved complete or partial response at time of HCST. After a median follow-up of 79.9 months, 4-years progression-free survival (PFS) and overall survival (OS) were similar in both autologous and allogeneic groups (PFS: 34% vs. 26%, p = .12 and OS: 52% vs. 53%, p = .74). Response status at HSCT was the major independent prognostic factor on survival (OS: HR: 4.013 [1.137; 14.16], p = .031 and PFS: HR: 5.231 [1.625; 16.838], p = .006). As compared to control patients receiving chemotherapy and/or radiotherapy containing regimens only, upfront HSCT did not improve the outcome of responder patients, including those treated by L-aspa. However, it tends to provide survival benefit for relapsed patients with initial high-risk clinical features who achieved second remission. Whereas the place of HSCT in upfront therapy has still to be clarified, these data confirm that HSCT should be considered for consolidation in selected patients with relapsed ENKTL. Based on a large non Asian ENKTL cohort since the L-aspa era, this study provides some insight into the survival patterns of ENKTL patients with HSCT in the Western hemisphere and may give future direction for the next clinical trial design., (© 2021 Wiley Periodicals LLC.)

The nose is highly vulnerable to skin cancers due to the unavoidable sun exposure. The most common localization of skin cancers on the face is nose. Although the nose appears to be a single structure, it comprises many aesthetic units with different histological and anatomical properties. Our aim was to determine the relationship between the prevalence of basal cell carcinoma (BCC) and squamous cell carcinoma (SCC), histologically and anatomically distinct nasal subunits. The study included patients who underwent excision and repair due to BCC or SCC of the nose. The lesions were classified according to their location in the following topographic subunits: tip, alar lobule, dorsum, sidewall, and medial canthal region. Patients were analyzed according to age, sex, topographic subunit, tumor type, and repair technique. There was no statistically significant difference in tumor location according to etiology ( p  > 0.05). The alar subunit was the most common location of BCC, while the dorsum was the most common location for SCC. There is no statistical relationship between the two most common skin cancers, BCC and SCC, and the aesthetic subunits of the nose. The only factor associated with the reconstruction method used was the subunit in which the tumor was located., Competing Interests: None declared., (Thieme. All rights reserved.)

Inflammatory and nasal-sinus tumor pathology is a field of great interest in rhinology worldwide. The aim of the paper is to determine the prevalence of nasal and nasal-sinus inflammatory diseases, as well as nasal and nasosinusal rhinosinusitis tumors, in association or not with inflammatory diseases, using histopathological (HP) examination. It is also desired to identify the association of chronic inflammatory pathology with the tumor one, considering inflammation and immunodeficiency as local susceptibility factors. A retrospective study was performed on a group of 254 patients hospitalized between 2018-2019 in Department of Otorhinolaryngology, Emergency County Hospital, Târgu Mureş, Romania. Based on the clinical and HP examination, the distribution by inflammatory pathologies was made as follows: 175 nasal polyposis, 108 chronic rhinitis, 39 sinusitis - strictly affecting the sinus and 28 chronic polyposis rhinosinusitis - nasal and sinus association. Considering the evaluation of the incidence of benign tumor pathology, the following were found: out of the total examined cases, 4% squamous papilloma, 4% exophytic papilloma, 44% Schneiderian papilloma, 4% benign fibrous histiocytoma, 18% hemangioma, 4% hamartoma, and 4% osteoma were identified. The incidence of malignant tumors is 26% squamous cell carcinoma, 12% intestinal adenocarcinoma, 2% nonintestinal type adenocarcinoma, 2% large B-cell lymphoma, 2% plasma cell, 2% olfactory neuroblastoma, 7% malignant melanoma, 16% basal cell carcinoma. The paper draws attention to the increased incidence of tumor and inflammatory pathology both individually and in combination, considering the involvement of the clinical correlation with the HP result completed, if necessary, with immunohistochemical examinations, for a precise diagnosis.

Betaretrovirus-induced transmissible respiratory tumors in sheep arise at 2 distinct anatomic locations, either deep in the lung tissue caused by jaagsiekte sheep retrovirus (JSRV) or in the nasal cavity induced by ovine enzootic nasal tumor virus (ENTV-1). JSRV and ENTV-1 are found in many countries worldwide and have a significant economic and animal health impact. Although JSRV is endemic in sheep in the British Isles, ENTV-1 has not been reported. We report herein a nasal adenocarcinoma in a cull 8-y-old Belclare ewe from Ireland. The gross and microscopic features and immunohistochemistry results were consistent with an ENTV-1-associated tumor. However, differential PCR, using primers specific to regions of divergent sequence between the viruses, was performed on different parts of the adenocarcinoma and produced consistent results: positive for JSRV and negative for ENTV-1. An association of JSRV with nasal adenocarcinoma in sheep has not been reported previously, to our knowledge. Our case shows the necessity of using PCR in combination with immunohistochemistry to reach an accurate etiologic diagnosis, which is of importance in countries currently free of ENTV-1.

This review of sinonasal adenocarcinoma, both intestinal and non-intestinal type, aims at providing a comprehensive overview of etiological factors, diagnostic workup, histological subtypes, advances in molecular characterization and the genetic basis, current optimal treatment strategies, resulting oncological outcome, and prognostic factors modifying the final treatment results. The current treatment of choice remains surgical resection with a curative intent, using the least invasive approach that allows for removal of the entire tumor with negative margins, supplemented with postoperative high-quality intensity-modulated radiotherapy in the majority of patients. To date, chemotherapy remains reserved for the palliative setting. The progress in understanding the underlying molecular biological mechanisms has not yet translated into standard of care applications., (© 2020 S. Karger AG, Basel.)

Nasal cancer has not been included in the current list of legal occupational diseases in China. There is also a lack of systematic and in-depth study on the relationship between nasal cancer and occupational exposure factors in China. In September 2018, the department for work and pensions of UK released the latest edition of the "List of diseases covered by industrial injuries disablement benefit", which lists nasal cancer and nasopharyngeal carcinoma associated with wood dust exposure on the UK's occupational disease list. In order to better protect the health of workers, the relationship between occupational wood dust exposure and nasal cancer is reviewed, which provides a reference for further revision and improvement of occupational disease catalogue.

Objectives: This paper aims to provide contemporary epidemiological data on squamous cell carcinoma (SCC) of the nasal cavity, which represents a rare type of head and neck cancer., Design, Setting & Participants: A descriptive analysis of people with nasal cavity SCC treated with curative intent from the Head and Neck 5000 study; a multicentre clinical cohort study of people from the UK with head and neck cancer. People with tumours of the nasopharynx, paranasal sinuses and other sub-sites of the head and neck were excluded., Main Outcome Measures: Demographic data and treatment details are presented for all participants. The main outcomes were overall survival and survival according to categories of characteristics (eg, smoker vs non-smoker); these were explored using Kaplan-Meier plots., Results: Thirty people with nasal cavity SCC were included in the study, of which most were male (67%) and current or ex-smokers (70%). The majority (70%) presented with early-stage (T1/2, N0) tumours. Cervical lymph node metastases at presentation were rare, occurring in only one person. Nine people died during the follow-up period (30%). Worse survival outcomes were seen in people with moderate or severe co-morbidities., Conclusions: This paper provides epidemiological data on nasal cavity SCC in the UK. Patterns of disease and survival outcomes are described, identifying high-risk groups. Further studies should explore whether primary treatment modality alters survival., (© 2019 John Wiley & Sons Ltd.)

Introduction: Inverted Papilloma (IP) is a rare benign tumour of the nose and paranasal sinuses histologically characterized by invagination of the outer layer of the epithelium in the underlying chorion. Its most distinctive feature is a strong local aggressiveness, a tendency to recur and an unpredictable risk of association with epidermoid carcinoma. The purpose of this study was to report the epidemiological, clinical, paraclinical data as well as to assess the outcomes of endoscopic endonasal surgery., Methods: We conducted a retrospective study on a series of 13 patients whose data were collected in the Oto-Rhino-Laryngology at the National University Hospital Center of Fann, from 1 st January 2012 to 31 st December 2017. All patients followed up for inverted papilloma confirmed by anathomopathological examination were included in the study., Results: The average age of patients was 44 years, the sex-ratio was 2.25. All patients had nasal obstruction; 53% of them had rhinorrhea followed by epistaxis in 30% of cases. Right-sided symptoms were reported by 69% of patients, left-sided symptoms by 23% and bilateral symptoms by 7% of patients. Anterior rhinoscopy showed endonasal mass in all patients. All patients underwent CT scan; the MRI was performed in a single patient. Endoscopic resection of IP was performed in 10 patients (76.9%) while external approach was used in 23% of cases. Surgery helped to clarify where the tumor had originated: in 46% of cases in the maxillary sinus, in 15% in the lower nasal turbinate, in 15% in the mid-turbinate, in 7% in the ethmoid bulla and in 7% in the lateral wall of the nasal fossa. One patient had recurrence after an average period of 26 months. Malignant transformation to squamous cell carcinoma occurred in one patient., Conclusion: Inverted papilloma is a very aggressive tumor. CT scan is essential to highlight its spread, especially to bone. The advent of endoscopic surgery has revolutionised treatment, providing excellent results. But, there is nevertheless a risk of recurrence after surgery which motivates an indication for long term monitoring., Competing Interests: Les auteurs ne déclarent aucun conflit d'intérêts., (© Moustapha Ndiaye et al.)

Objectives: To further characterize the epidemiology, prognostic disease-specific factors, and treatment outcomes-including newly available chemotherapy data-for patients with sinonasal diffuse large B-cell lymphoma (DLBCL)., Methods: The Surveillance, Epidemiology, and End Results registry was queried from 1973 to 2015 for patients with DLBCL of the nasal cavity and paranasal sinuses. Demographic and disease parameters were collected. Prognostic disease-specific survival and overall survival factors were evaluated with univariate Kaplan-Meier analysis. Significant variables were analyzed with multivariate Cox regression analysis., Results: A total of 1,273 cases of DLBCL of the sinonasal tract were identified. Significant differences in age of diagnosis between men (65.3) and women (71.1) existed (P < .01). Most common primary sites of DLBCL were maxillary sinus (36.1%) and nasal cavity (34.5%), with nasal cavity more common among Asian/Pacific Islands (43.4%) and maxillary sinus more common for Caucasians (36.3%) and African Americans (42.1%). Overall survival was 70% at 2 years, 54% at 5 years, and 38% at 10 years. Disease-specific survival was 81%, 73%, and 67%, respectively. Chemotherapy (hazard ratio [HR]: 0.551; P < .001) and radiation therapy (HR: 0.818; P = .012) were associated with improved prognosis, whereas higher Ann Arbor stage worsened prognosis (HR: 1.21; P < .001). Surgical intervention did not significantly impact survival., Conclusion: This is the first study to include chemoradiation therapy in population-based analysis of sinonasal DLBCL. Chemotherapy and radiation therapy use significantly improve survival in these patients, whereas Ann Arbor staging is significantly associated with poorer outcomes. The mainstay of treatment for DLBCL should remain combination chemoradiation., Level of Evidence: NA Laryngoscope, 129:1259-1264, 2019., (© 2018 The American Laryngological, Rhinological and Otological Society, Inc.)

Different risk factors are suspected to be involved in malignant transformation of sinonasal papillomas and include HPV infection, tobacco smoking, occupational exposure, EGFR/KRAS mutations and DNA methylation alterations. In our study, 25 inverted sinonasal papillomas (ISPs), 5 oncocytic sinonasal papillomas (OSP) and 35 squamous cell carcinomas (SCCs) from 54 patients were genotyped for 10 genes involved in EGFR signalling. HPV-DNA detection was performed by in-situ hybridisation and LINE-1 methylation was quantitatively determined by bisulphite-pyrosequencing. High-risk HPV was observed only in 13% of ISP-associated SCC and in 8% of de novo-SCC patients. EGFR mutations occurred in 72% of ISPs, 30% of ISP-associated SCCs and 17% of de novo-SCCs. At 5-year follow-up, SCC arose in only 30% (6/20) of patients with EGFR-mutated ISPs compared to 76% (13/17) of patients with EGFR-wild-type ISP (p = 0.0044). LINE-1 hypomethylation significantly increased from papilloma/early stage SCC to advanced stage SCC (p = 0.03) and was associated with occupational exposure (p = 0.01) and worse prognosis (p = 0.09). In conclusion, our results suggest that a small subset of these tumours could be related to HPV infection; EGFR mutations characterise those ISPs with a lower risk of developing into SCC; LINE-1 hypomethylation is associated with occupational exposure and could identify more aggressive nasal SCC., (© 2018 UICC.)

Aim: To investigate the characteristics and prognosis of melanoma in a Chinese population., Materials & Methods: Total of 162 advanced melanoma patients were analyzed retrospectively. Kaplan-Meier method and Log rank test were used for survival analysis., Results: The median progression-free survival of mucosal and cutaneous melanoma patients was 13 versus 8 months (p = 0.005), 14 versus 10 months in immunotherapy group (p = 0.022), 6 versus 4 months in chemotherapy group (p = 0.040). Age was an independent risk factor for mucosal melanoma patients. Staging and treatment regimen were independent risk factors for cutaneous melanoma patients. The lungs, liver and brain were most common metastasis locations., Conclusion: The prognosis of patients with advanced mucosal melanoma is better than cutaneous melanoma in China. There are significant differences between the two subtypes of melanoma.

Objective: Nasal and sinonasal inverted papilloma (IP) are rare benign tumors and have the potential to exhibit malignancy in approximately 10% of cases. This study aimed to analyze the clinical features of IP associated with malignancy. Furthermore, we reviewed our therapeutic strategy and the clinical course of malignant IP., Methods: Overall, 70 patients with IP at our institution were retrospectively analyzed from April 2006 to December 2015; of these, six (9%) had associated malignancy. Data was collected on sex, age, presenting symptoms (nasal bleeding, rhinorrhea, facial or cheek pain, and nasal obstruction), bone destruction, and extent of disease on CT and MRI. Categorical data of patients with and without malignancy were compared using the chi-square test. A p value of <0.05 was considered statistically significant. Our therapeutic strategy for IP with malignancy, particularly the surgical procedure, i.e., the external incision or the endoscopic nasal approach, varied based on when the carcinoma was detected. In addition, we considered postoperative radiation therapy depending on histological examination., Results: Nasal bleeding (p<0.001), pain (p=0.040), bone destruction (p<0.001), and extent of disease (p=0.026) on CT and MRI findings were significantly associated with malignancy. Carcinoma was diagnosed preoperatively in two (33%) and postoperatively in four patients (67%). We operated five patients (one case was not treated because of end-stage pancreatic cancer). Two patients underwent endoscopic sinus surgery (ESS) alone, two ESS plus Denker's method, and one ESS plus anterior craniotomy. Three patients underwent surgery only, and two patients received postoperative radiotherapy. The median follow-up period was 69.3 months. One patient died of the disease and the remaining patients are alive without recurrence., Conclusion: For IP patients exhibiting these clinical findings preoperatively, we should suspect complication with malignancy and plan a treatment. Even if postoperative histology does not confirm malignancy, we should ensure careful observation because of metachronous malignant transformation or the possibility to overlook small malignant lesions. Our result suggests that our strategy for malignant IP could be a reasonable option., (Copyright © 2018 Elsevier B.V. All rights reserved.)

Treatment of infantile hemangioma is usually medical. The nose is one of the most important aesthetic and functional units of the face; therefore, surgical management is preferred in persistent lesions (fibro-fatty tissue component) that do not respond to medical treatment. Herein, the authors analyze the results of surgical nasal hemangioma treatment in their center, a literature review and propose an algorithm for surgical management. A retrospective analysis of 23 persistent nasal hemangioma operated between 1996 and 2014 at our institution was made. The authors recollected the following demographic and clinical data: hemangioma subtype, phase of evolution, affected aesthetic nasal subunit, previous treatment, surgery type, complications and follow-up period. The Strasser scale was chosen for assessment of postoperative photographic results. Surgical treatment was performed. Age average was 6.8 years old (range 2-19). Mixed hemangioma was the most common subtype (83%). Surgery was mainly performed in the involution phase (87%). The most affected aesthetic subunit was nasal tip (44%). All lesions received previous medical treatment. The most common surgical technique was open rhinoplasty approach. Follow-up was an average of 26.6 months. Aesthetic results according to the Strasser method included: 3 excellent scores, 15 good, 5 regular, and none poor. Surgery was performed on patients in order to correct nasal persistent hemangiomas sequel. The nose has serious psychosocial impact and mixed infantile hemangiomas appear to have a higher rate of persistent hemangioma requiring surgery, but further studies are needed. Aesthetic outcome with surgical procedures chosen were acceptable and no complications were reported.

Objective: Sinonasal cancers (SNC) are rare, thus limiting previous prognostic studies on a multinational level. The aim of this study is to utilize two population-based datasets to compare prognoses for SNC between the United States (US) and Europe., Methods: The European Cancer Registry (EUROCARE) database and the United States National Cancer Institute's Surveillance, Epidemiology, and End Results (SEER) database were searched to identify survival of patients diagnosed with SNC between 1990 and 2007. Relative survival (RS) data were stratified by age group, gender, geographic location, and diagnostic time period., Results: 12,541 SNC cases were identified in EUROCARE, while SEER identified 4,312. Males comprised the majority in Europe (62.3%) and the US (58.3%). Most patients were over 55 years in Europe (77.0%) and the US (69.5%). Age over 75 was a statistically significant poor prognostic indicator for 5-year RS in the US (48.2%; 95% CI=[43.9, 52.4]) and Europe (38.5%; [34.7, 42.7]). Female gender imparted a favorable 5-year RS in all regions except in Central Europe. By region, the US had the highest 5-year RS (58.8%; [56.4, 61.1]) and Eastern Europe had the lowest 5-year RS (37.1%; [34.0, 40.6]). The aggregate European 5-year RS was 48.1% [46.4, 49.8]., Conclusion: SNC in Europe and the US most commonly affects males and individuals over the age of 55 years. Male gender and age over 75 are poor prognostic factors at 5 years. Five-year RS in the US is higher than the 5-year European aggregate RS., (Copyright © 2017 Elsevier B.V. All rights reserved.)

Introduction: To evaluate the influence of comorbidities and anemia on outcome and survival in patients with sinonasal carcinomas., Materials and Methods: Review of medical records of 121 patients with carcinoma treated at a single quaternary medical care and cancer center between 2002 and 2015. Comorbidities were classified according to the age-adjusted Charlson comorbidity index (ACCI) scoring system., Results: 71 patients were male, 50 were female. Median age was 65 years, and the median interval between tumor diagnosis and date of study inclusion was 71 months. The most common histological subtype was squamous cell carcinoma (n = 64). The median ACCI score was 2 and the most frequent ACCI score was 0 (n = 26). Mean overall survival was 52.2 months (ACCI 0-2) and 39.5 months (ACCI ≥ 3), respectively (p < 0.0001). Mean disease-free survival was 52.9 months (ACCI 0-2) and 45.5 months (ACCI ≥ 3), respectively (p = 0.026). Mean overall survival in patients without anemia was 49.5 months, compared with 39.8 months in patients with anemia (p = 0.043)., Conclusion: Comorbidity is an independent risk factor on overall, disease-free and disease-specific survival in patients with sinonasal carcinoma. Anemia is associated with shorter overall survival. This is the first study evaluating comorbidity using the ACCI scoring system and focusing on patients with sinonasal carcinoma., (Copyright © 2018 European Association for Cranio-Maxillo-Facial Surgery. Published by Elsevier Ltd. All rights reserved.)

Background: Squamous cell carcinoma of the nasal vestibule is a rare entity. In consequence disagreement in etiology, staging system and therapy of primary tumor with or without adjuvant treatment of regional lymph nodes are apparent., Methods: Pubmed-Recherche of relevant literature concerning: lymphatic drainage, metastases, incidence, risk factors (leather, nickel, nicotine, human papillomavirus, Staging system (UICC, AJCC, Wang's system), therapy of the primary tumor, regional lymph nodes and immunohistochemistry., Results: Fifty-five studies were found and analyzed. Results are inconsistent., Conclusion: The Wang-classification is recommended. Radiation and surgery are the treatment of choice for small lesions. Larger lesions (T3-Wang) should be treated with a combined approach. In cT1-cT2cN0-situation after accurate diagnostic, an elective therapy of regional lymph nodes is not necessary., Competing Interests: Die Autoren geben an, dass kein Interessenkonflikt besteht., (© Georg Thieme Verlag KG Stuttgart · New York.)

Introduction: The distribution of extranodal NK/T-cell lymphoma (ENKTCL) is highly inhomogeneous throughout the world. In Sub-Saharan Africa, despite the precocity of Epstein-Barr virus (EBV) infection and its endemicity, ENKTCL remains exceptionally reported. The purpose of this study was to report the epidemiological, clinical, paraclinical, and evolutionary characteristics of ENKTCL at the Aristide LeDantec University Hospital in Dakar, Senegal., Methodology: A 5-year retrospective review of all patients with histopathological, immunohistochemical, and in situ hybridization proven cutaneous lymphomas RESULTS: We collected seven cases corresponding to a frequency of 1.4 cases per year. ENKTCL accounted for 10.5% of all cutaneous lymphomas, ranking second after T-cell lymphomas. Men were predominantly affected (M : F ratio of 6), and the mean age was 38.5 years ± 4.06. The mean time before consultation was 7.3 months. The lymphomas affected primarily the nasal cavity in five cases and the skin in two cases. At admission, six patients had nasal mucosa involvement, which was isolated in three cases, associated with cutaneous lesions in three cases and lymph node involvement in three cases. CD56 was positive in only one case, and Eber transcribed RNA of EBV was expressed by in situ hybridization in all patients., Discussion: To our knowledge, we have reported the first and largest series of ENKTCL in Sub-Saharan Africa. Our study shows an intermediate prevalence between that reported from Asia, Latin America, and the West. It was also noted a young age of patients, a prolonged diagnostic delay, a frequent negativity of CD56 marker, and a very poor prognosis of the disease in our region., (© 2018 The International Society of Dermatology.)

Background: Sinonasal cancer (SNC) is a rare tumor with predominant occupational etiology associated with exposures to specific carcinogens. The aim of this study is to describe SNC cases recorded in Italy in the period 2000-2016., Methods: Clinical information, occupational history, and lifestyle habits of SNC cases collected in the Italian Sinonasal Cancer Register were examined. Age-standardized rates were estimated., Results: Overall, 1529 cases were recorded. The age-standardized incidence rates per 100 000 person-years were 0.65 in men and 0.26 in women. Occupational exposures were predominant among the attributed exposure settings, primarily to wood and leather dusts. Other putative causal agents included chrome, solvents, tannins, formaldehyde, textile dusts, and pesticides. Many cases had unknown exposure., Conclusions: Epidemiological surveillance of SNC cases and their occupational history is fundamental for monitoring the occurrence of the disease in exposed workers in industrial sectors generally not considered at risk of SNC as well as in non-occupational settings., (© 2017 Wiley Periodicals, Inc.)

The current study aims to provide stronger evidence to aid in our understanding of the role of cumulative occupational exposure to (softwood-dominated) mixed wood dust in aetiology of nasal cancer. We included broad exposure occurred in a range of wood-processing occupation across varied industries in four Nordic countries. A population-based case-control study was conducted on all male cases with nasal adenocarcinoma (393 cases), other types of nasal cancer (2,446) and nasopharyngeal cancer (1,747) diagnosed in Finland, Sweden, Norway and Iceland between 1961 and 2005. For each case, five male controls, who were alive at the time of diagnosis of the case (index date), were randomly selected, matched by birth-year and country. Cumulative exposures (CE)s to wood dust and formaldehyde before the index date were quantified based on a job-exposure matrix linked to occupational titles derived from population censuses. Hazard ratios (HRs) for the CE of wood dust were estimated by conditional logistic regression, adjusted for CE to formaldehyde and 95% confidence intervals (CIs) were calculated. There was an increasing risk of nasal adenocarcinoma related to wood dust exposure. The HR in the highest CE category of wood dust (≥ 28.82 mg/m 3 -years) was 16.5 (95% CI 5.05-54.1). Neither nonadenocarcinoma of the nose nor nasopharyngeal cancer could be linked to wood dust exposure. CE to softwood-dominated mixed wood dusts is strongly linked with elevated risk in nasal adenocarcinoma but not with other types of nasal or nasopharyngeal cancer., (© 2017 UICC.)

Introduction: Extranodal natural killer/T (NK/T) cell lymphoma, nasal type (ENKTL) represents a rare subtype of T-cell lymphomas with aggressive clinical behavior according to WHO 2016 classification., Aim: ENKTL has distinctive geographic distribution with higher incidence in Asia and Latin America (10% of all non-Hodgkin lymphoma cases), than in Europe and North America (<1%). ENKTL tipically origins from nasopharynx and upper aerodigestive tract. Anthracycline-based chemotherapy regimens are largely ineffective in the treatment of ENKTL., Method: Our aims were to evaluate the incidence and treatment strategies of ENKTL patients in Hungarian Haematological Centres between 2003 and 2015. Altogether 20 patients with ENKTL were treated in the 4 haematological hospitals (male:female ratio 12:8, with median 49.5 years of age)., Results: Ten patients had localized (stage I-II) disease at the time of the diagnosis. Seventeen patients were treated with chemotherapy (11/CHOP, CHOP-like, 2/HyperCVAD, 1/ProMACECytaBom, 1/SMILE, 2/others), which was completed with involved-field radiation therapy (IFRT) (40-46 Gy) in 6 cases were used. After first-line therapy 9 patients achieved complete remission (CR), 3 patients had partial remission (PR), 3 patients had progressive disease (PD), and 2 patients had stable disease (SD). Median follow-up was 32 (3-113) months. Five patients received second-line therapy for progressive or recurrent disease [2/DHAP, 1/VIM, 1/HyperCVAD, 1/ProMACECytaBom]. None of the patients achieved CR after second-line therapy. Two patients have undergone autologous hematopoietic stem cell transplantation (HSCT) after the first CR., Conclusion: ENKTL treatment is more effective with nonanthracycline-containing regimens. L-asparaginase containing chemotherapy and concurrent or sequential chemo-radiotherapy improves survival and CR rates. Orv Hetil. 2017; 158(41): 1635-1641.

Background: The purpose of this study was to analyze the role of p16 INK4a and the prevalence of human papillomavirus (HPV) in squamous cell carcinoma (SCC) of the nasal vestibule., Methods: Patients diagnosed from 1995 to 2014 were included in this study. Assessment of p16 INK4a and HPV-DNA polymerase chain reaction (PCR) was performed and analyzed with respect to baseline, clinicopathological, and outcome parameters. The p16 INK4a positivity was defined as unequivocal nuclear and cytoplasmic staining of ≥70% of the cells, whereas 50%-69% was considered to be a "borderline" result., Results: There were 46 patients with SCCs of the nasal vestibule, of whom 31 (67.4%) were available for p16 INK4a and 30 (65.2%) for analysis of HPV. Expression of p16 INK4a was present in 19.4% and showed coincidence with high-risk HPV (P < .001). Neither p16 INK4a nor HPV-DNA had significant impact on outcome., Conclusion: Significant immunoreactivity for p16 INK4a was present in about one-fifth of the samples and figured as a surrogate marker of high-risk HPV infection. There was no influence on outcome., (© 2017 Wiley Periodicals, Inc.)

Introduction: Pediatric Sinonasal Malignancies (PedsSNM) are rare and usually associated with a poor prognosis. We aim to investigate the epidemiology, tumor characteristics, and survival of PedsSNM using a population-based database to augment the scant literature on this topic., Methods: The Surveillance, Epidemiology, and End Results database was queried for patients ≤18 years diagnosed with PedsSNM between the years of 1973 and 2013. Data on incidence, tumor characteristics, and survival were analyzed., Results: In total, 210 patients with PedsSNM were identified. Demographically, 54.3% were female, 72.6% were white, and the mean age was 10.7 years. Overall incidence was 0.036 per 100,000 individuals between the years of 2000 and 2013. The nasal cavity was the most frequent primary site (37.1%) and rhabdomyosarcoma was the most frequent malignancy (50.5%). Five-, 10-, and 20-year disease-specific survival (DSS) rates were 60.2%, 46.1%, and 20.6%, respectively. Grade IV tumors made up the largest group (37.3%), and such tumors exhibited the worst 5-, 10-, and 20-year survival (P < 0.05). Distant disease predicted the worst 5-, 10-, and 20-year survival, followed by regional, then localized disease (P < 0.01). Patients treated with surgery alone had a higher 20-year survival (P = 0.0425). No significant differences in survival were observed between race, gender, primary site, or histology., Conclusions: PedsSNM frequently presented as Grade IV tumors. The nasal cavity was the most common primary site and rhabdomyosarcoma was the most frequent histology. Patients receiving surgery alone had the highest survival; however, this may be a reflection of smaller, less aggressive tumors preferentially being treated with surgery alone., (Copyright © 2017 Elsevier B.V. All rights reserved.)

Background: Benign tumors of the nasal cavity represent a large variety of different histopathological entities. Although advances in nasal endoscopy over the past couple of decades have made it possible to detect the vast majority of these lesions, accurate diagnosis and proper management can be delayed since they are misdiagnosed as inflammatory paranasal sinus disease or simple epistaxis., Objectives: The aims of the present study are to determine the relative incidence of benign tumor of the nasal cavity and to provide typical endoscopic features of common tumors. This information can potentially improve clinicians' comprehension of benign tumors of the nasal cavity and be helpful in making provisional diagnosis., Results: The present study included 32 patients (25 males, 7 females) with benign tumor of the nasal cavity, which was pathologically confirmed. The most common symptom was nasal obstruction (12/37.5%), followed by recurrent epistaxis (7/21.9%). The most common involving site was anterior nasal septum (17/53.1%), followed by nasal vestibule (7/18.8%) and inferior turbinate (4/12.5%). The most common pathology was squamous papilloma (12/37.5%), followed by lobular capillary hemangioma (7/21.9%). The provisional diagnosis was easily made by nasal endoscopy in 19 (59.4%) patients with typical endoscopic features, especially squamous papilloma, lobular capillary hemangioma, and verruca vulgaris., Conclusions: Clinicians should keep in mind that benign tumors of the nasal cavity are included in the differential diagnosis of unilateral nonspecific nasal symptoms such as nasal obstruction and epistaxis. Also, clinicians should be familiar with the endoscopic findings of various benign tumors and perform the diagnostic approaches with the provisional diagnosis on the basis of those findings.

The true incidence of inverted papilloma (IP) is not yet known. From hospital-based studies, its incidence has been estimated to approximately 0.5/100,000 person years. Earlier hospital case studies have shown that IP can undergo a malignant transformation in 1-53 %. The frequency of its malignant transformation on a population basis is unknown. To our knowledge, no standardised incidence ratio (SIR) has been reported for malignancies among IPs. This study aims to investigate these incidences on a population basis. Using the data from the Swedish Cancer Registry (SCR), we have identified patients with IP and patients with Squamous Cell Carcinoma (SCC) diagnosed between 1960 and 2010 in Sweden. Incidence of IP and incidence of SCC among patients with IP and SIR were analyzed. Eight hundred and fourteen patients with IP were identified. The incidence of IPs reported to the SCR increased from 1960 to 2010. In this cohort, SCC was overrepresented, as compared with the general population. The incidence of IP in the Swedish population seems to have increased., Competing Interests: No conflicts of interest for any of the authors. Ethical approval The study has been approved by the Regional Ethics Committee at the Karolinska Institute. This article does not contain any studies with human participants or animals performed by any of the authors.

Background: Occupational exposure to styrene is widespread and has been suggested to be carcinogenic. The aim of this study was to investigate whether occupational exposure to styrene increases the risk of cancer, in particular lymphohematopoietic cancers., Methods: We established a study population of 72,292 workers employed in 443 small and medium-sized companies producing reinforced plastics 1964-2007 by utilizing several national registries, expert assessment, and worker survey data. We identified incident cancer cases from 1968 to 2012 in the national Danish cancer registry and computed standardized incidence rate ratios (SIRs) with 95% confidence intervals (95% CI) based on national rates., Results: Increasing SIRs of Hodgkin lymphoma, myeloid leukemia, and cancer of nasal cavities and sinuses were inconsistently associated with increasing duration of employment, early year of first employment, or styrene exposure probability. No such trends were observed for cancer of the esophagus, pancreas, lung, kidney, or urinary bladder, which have previously been associated with styrene exposure. Lung cancer showed an overall increased risk that decreased by duration of employment., Conclusion: Occupational styrene exposure may be associated with Hodgkin lymphoma, myeloid leukemia, and cancer of nasal cavities and sinuses. Further studies are needed to evaluate if the observed associations are likely to be causal.

Background: Chronic rhinosinusitis (CRS) has a multifactorial etiology, with a debate about the role of inhalant allergy in the pathogenesis of CRS., Objective: This study assessed the impact of allergy status on externalized paranasal sinuses after tumor resection to determine if a predisposition to inhalant allergy brought about additional inflammation after sinus surgery., Methodology: A case-control study was performed on patients who had no history of CRS who underwent paranasal sinus tumor resection. Allergic sensitization was defined by a positive serum ImmunoCAP test result. Outcomes were measured at least 6 months after surgery by using the modified Lund-MacKay endoscopic score and the 22-item Sino-Nasal Outcome test, with rhinitis, sleep, psychological, ear and/or facial, and sinus subscores to assess the impact of allergy status on mucosal inflammation., Results: A total of 103 patients (53.44 ± 17.46 years; 46% women) were assessed. Of these, 61.17% were allergically sensitized at the time of surgery. Postsurgery endoscopic assessment was similar [the modified Lund-Mackay endoscopic score allergic sensitized 0.5 (1.7) versus nonallergic sensitized 0.0 (0.9); p = 0.15]. Sinonasal symptoms were also similar between the groups' 22-item Sino-Nasal Outcome test scores, allergic sensitized versus allergic nonsensitized, (allergic 28.9 ± 20.8 versus nonallergic 33.5 ± 19.7; p = 0.31), rhinitis score (5.9 ± 5.5 versus 6.4 ± 4.7; p = 0.66), sleep score (6.9 ± 5.9 versus 7.7 ± 4.8; p = 0.50), ear and/or facial symptom score (3.4 ± 3.6 versus 4.3 ± 3.3; p = 0.22), psychological score (6.9 ± 6.0 versus 8.3 ± 6.7; p = 0.29), and of nasal symptom score (6.4 ± 5.2 versus 7.0 ± 5.3; p = 0.61)., Conclusions: Externalization of the sinuses in patients with inhalant allergy did not bring about significant additional inflammation in patients after tumor surgery.

Sinonasally located lymphoid malignancies are rare lesions with first symptoms similar to other obstructive conditions. Additionally, they often coexist with nasal inflammation and mucosal necrosis. Therefore, time from the first symptoms to diagnosis tends to be long. Awareness and early diagnosis of this disease entity could improve treatment outcome. Altogether, 142 patients with sinonasal or nasopharyngeal (i.e. sinonasal tract, SNT) lymphoid malignancies, diagnosed and treated at the Helsinki University Hospital, during a 39-year period from 1975 to 2013, were retrospectively reviewed. There were 90 males (63 %) and 52 females (37 %) with a median age of 64 years (range 26-92). Eighty-four percent of the patients had primary diseases and 16 % had relapses of lymphoid malignancies primarily diagnosed at other locations. The mean duration of symptoms prior to diagnosis was 4.8 months (range 0.5-24). The most common histological entity was diffuse large B-cell lymphoma (43 %), followed by plasmacytoma (18 %). The most common location was nasopharynx (58 %) followed by nasal cavity (44 %) and paranasal sinuses (35 %). Sixty-nine percent of the lesions were at a single anatomic location of the sinonasal tract. Fifty-two percent of the cases were of Ann Arbor Stage I. Lymphoid malignancies form an important and diverse group in the differential diagnosis of SNT tumours. They most often present with general obstructive nasal symptoms due to tumour location. Most of them are primary lesions, highlighting the importance of an accurate diagnosis as early as possible.

Background: Long-term follow-up in clinical reviews often presents significant difficulty with conventional medical records alone. Publicly accessible genealogical databases such as Ancestry.com provide another avenue for obtaining extended follow-up and added outcome information. No previous studies have described the use of genealogical databases in the follow-up of individual patients., Methods: Ancestry.com, the largest genealogical database in the United States, houses extensive demographic data on an increasing number of Americans. In a recent retrospective review of esthesioneuroblastoma patients treated at our institution, we used this resource to ascertain the outcomes of patients otherwise lost to follow-up. Additional information such as quality of life and supplemental treatments the patient may have received at home was obtained through direct contact with living relatives., Results: The use of Ancestry.com resulted in a 25% increase (20 months) in follow-up duration as well as incorporation of an additional 7 patients in our study (18%) who would otherwise not have had adequate hospital chart data for inclusion. Many patients within this subset had more advanced disease or were remotely located from our institution. As such, exclusion of these outliers can impact the quality of subsequent outcome analysis., Conclusion: Online genealogical databases provide a unique resource of public information that is acceptable to institutional review boards for patient follow-up in clinical reviews. Utilization of Ancestry.com data led to significant improvement in follow-up duration and increased the number of patients with sufficient data that could be included in our retrospective study., (© 2016 ARS-AAOA, LLC.)

Background: The objective of this study was to review the published literature related to sinonasal small cell neuroendocrine carcinomas (SCNECs). Clinical presentation, demographics, treatment, and outcomes of this uncommon disease are reported., Methods: A systematic review of studies for sinonasal SCNECs in PubMed and Ovid databases from 1970 to 2014 was conducted. Bibliographies of selected articles were also examined. Articles were examined for patient data that reported disease outcome., Results: Thirty-four articles were included in this analysis, comprising a total of 80 cases. The mean age of presentation was 53.0 years. Nasal obstruction (53.8%) and epistaxis (48.8%) were the most common presenting symptoms. The nasal cavity and septum (32.5%) was the most common site of involvement. Seventy percent of patients presented with American Joint Committee on Cancer (AJCC) stage IV disease. Combination chemotherapy and radiation therapy was the most common treatment modality, used in 21 cases (26.3%). The second and third most common treatment modalities was combination surgery and chemoradiation therapy (21.3%), and surgery alone (18.8%), respectively. A total of 37 patients (46.3%) were alive after a mean follow-up of 30.8 months (median 15.5 months), independent of treatment modality. Of the treatment modalities, multimodality therapy remained the most common therapy., Conclusion: This review contains the largest pool of sinonasal SCNEC patients to date. Sinonasal SCNEC is a rare and aggressive neoplasm, and there is currently no standard of care for treatment. Various treatment modalities have been employed. Our systematic review suggests that multimodality therapy remains the most common treatment of sinonasal SCNEC., (© 2016 ARS-AAOA, LLC.)

Mucosal melanomas constitute 1.3% of all melanomas and they may develop in any mucosal membrane. Conjunctival melanomas (0.5/million/year) and melanomas in the sinonasal cavity (0.5/million/year) are the most common, followed by anorectal melanomas (0.4/million/year) and melanomas in the oral cavity (0.2/million/year). Anorectal melanoma occurs slightly more often in females, whereas oral melanoma has a male predilection. Mucosal melanoma most commonly develops in a patient's sixth or seventh decade of life, and no differences between races have been found except for sinonasal melanoma and conjunctival melanoma, which are very rare in Black people. The symptoms are not tumour-specific and are related to the organ system affected, and the disease is most often diagnosed at an advanced clinical stage. The diagnosis of a primary tumour is difficult, and metastatic cutaneous melanoma and choroidal melanoma must be excluded. Mutations in KIT are frequently found, while BRAF and NRAS mutations are rarely found - except in conjunctival melanomas that carry BRAF mutations. Mutations in the TERT promotor region are also found in mucosal melanomas. Complete surgical resection with free margins is the treatment of choice. The prognosis is poor, with the 5-year survival rate ranging from 0% (gastric melanoma) to 80% (conjunctival melanoma)., (© 2016 APMIS. Published by John Wiley & Sons Ltd.)

Objective: Olfactory neuroblastoma and the management of neck disease has posed considerable challenges to the treating physician. The aims of the study were to determine the incidence and factors influencing neck disease and to identify at-risk patients with cervical node-negative disease at presentation., Study Design: Multicenter case series with retrospective chart review., Setting and Subjects: In sum, 113 patients with a histopathologic diagnosis of olfactory neuroblastoma across 6 tertiary hospitals in Australia and the United States., Methods: Treatment modalities for the primary site and neck included surgery, radiotherapy, and combined therapy. Treatment outcomes were measured in relation to date of primary treatment, and long-term follow-up was recorded. Disease-free survival was calculated as time for patients to develop delayed neck disease following primary treatment., Results: A total of 113 patients (46 females, 49.7 ± 13.2 years) were identified with a median follow-up of 41.5 months (interquartile range, 58.2); 7.1% of patients presented with primary neck disease, while 8.8% of patients presented with delayed neck disease. Neck disease was present in patients with Hyams grade II (22.2%), III (55.6%), and IV (22.2%) lesions (χ(2) = 5.66, P = .13). Histologic grade was higher in patients with primary neck disease (χ(2) = 16.22, P = .001). Positive surgical margins were associated with a higher risk of delayed neck disease as compared with clear surgical margin (17.9% vs 5%, P = .034)., Conclusion: Neck metastasis is an important clinical consideration for olfactory neuroblastoma at presentation and in surveillance. Primary treatment of the neck could be considered in select patients. Long-term surveillance of the neck and primary site is essential., (© American Academy of Otolaryngology—Head and Neck Surgery Foundation 2015.)

Background: Primary fibrosarcoma of the sinonasal region is an infrequently occurring malignant neoplasm. Fibrosarcomas are most commonly found in the extremities, with only 1% of fibrosarcomas reported in the head and neck region. This study analyzes the demographic, clinicopathologic, and survival characteristics of sinonasal fibrosarcoma (SNFS)., Methods: The Surveillance, Epidemiology, and End Results (SEER) database (1973 to 2012) was queried for SNFS cases. Data were analyzed with respect to various demographic and clinicopathologic factors. Survival was analyzed using the Kaplan-Meier model., Results: Fifty-one cases of fibrosarcoma were identified in the sinonasal region. The mean age at diagnosis was 54.5 years and the mean survival was 119.7 months. There was no gender predilection with a male-to-female ratio of 1.04:1. The maxillary sinus was the most common site of involvement (54.9%), followed by the nasal cavity (23.5%). Five-year survival analysis revealed an overall survival rate of 71.7%, disease-specific survival rate of 77.8%, and relative survival (RS) rate of 78.8%. Disease-specific survival was better among those treated with surgery (with [76.2%] or without [87.5%] adjuvant radiotherapy) than those treated with primary radiotherapy alone (33.3%) (p = 0.0069)., Conclusion: SNFS is a rare entity. This study represents the largest series of SNFS to date. The mainstay of treatment for this tumor is surgical resection with or without radiotherapy., (© 2015 ARS-AAOA, LLC.)

Aims: Sinonasal mucosal malignant melanoma (SNMMM) is a rare disease. The aim of the present study was to describe its clinicopathological features and prognosis in a Chinese population., Methods: Data on 83 SNMMM patients were collected and analyzed. A survival analysis was performed using the Kaplan-Meier method and a log-rank test., Results: The most common presenting symptoms of SNMMM were nasal obstruction, epistaxis, and bloody rhinorrhea. Histopathologically, 38 cases (45.78%) were amelanotic. Five cell types (epithelioid, undifferentiated, plasmacytoid, spindle, and clear) were identified. Positive staining for human melanoma black-45 and Melan-A was diagnostic of SNMMM. Advanced age, multiple tumor sites, and amelanotic-type SNMMM indicated a worse outcome (p = 0.008, p = 0.009, and p = 0.013, respectively). Neither adjuvant therapy nor the tumor stage was associated with overall survival., Conclusions: SNMMM is an uncommon disease with atypical symptoms. Its histopathological appearance is variable, especially in the amelanotic type. Thus, immunohistochemistry is important in the diagnosis, and it should be performed according to the histology., (© 2016 S. Karger AG, Basel.)