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The objectives of this article are to describe an unusual clinical and histopathologic presentation of an ameloblastoma affecting the right maxilla, maxillary sinus, and nasal cavity and to discuss the difficulty of establishing a clinical classification based on the most recent edition of Head and Neck Tumours in the WHO Classification of Tumours series (2022). A 74-year-old man presented with a 6 × 6-cm expansile, ulcerated mass on the right lateral palate. A clinical diagnosis of squamous cell carcinoma was rendered. A biopsy was performed, and the specimen showed multiple histologic patterns of ameloblastoma inconclusive of odontogenic or sinonasal origin. Cone beam computed tomographic imaging demonstrated a well-defined unilocular mass in the right maxilla extending up to the nasal cavity. A surgical resection was performed and confirmed the diagnosis of maxillary ameloblastoma with extension into the nasal cavity. This dilemma in delayed diagnosis led to a literature search for similar maxillary ameloblastoma cases with extension into vital structures. In 45 cases previously reported in the literature, the median age of patients with maxillary ameloblastoma was 50 years, and there was extensive involvement of adjacent vital structures. The nasal cavity/sinonasal region (24/45), orbit/orbital floor (12/45), multiple fossae (5/45), and base of the skull (4/45) were the most common extensions of maxillary ameloblastoma. Fifteen patients had lesions with multiple extensions, and 1 patient showed lung metastasis. The most common histologic presentation was the follicular pattern, followed by the plexiform pattern or mixed follicular and plexiform patterns. Surgical interventions were performed on most patients, with the majority undergoing maxillectomy. Differentiating primary sinonasal ameloblastoma from gnathic ameloblastoma with sinonasal extension is challenging, and this article discusses subtle radiographic criteria and symptoms that aid in the distinction of both types. The authors suggest that variants of maxillary ameloblastoma with extensive involvement of the sinonasal region, orbit, or base of the skull be classified with a clinical diagnosis of maxillofacial ameloblastoma, regardless of the tumor origin., Competing Interests: No conflicts of interest reported.

Nasal vestibule squamous cell carcinoma (NVSCC) is an exceedingly rare malignancy, often misclassified due to its anatomical location and lack of a standardized definition. This review aims to consolidate current evidence on NVSCC, focusing on epidemiology, risk factors, classification, clinical presentation, treatment modalities, and prognostic factors. The NV anatomy is delineated, emphasizing the need for a clear definition to avoid misclassification. Risk factors include smoking, sunlight exposure, and debated associations with chalk exposure or viral factors. Clinical presentation includes symptoms like nasal obstruction, pain, burning, and bleeding, often misdiagnosed as inflammatory conditions. NVSCC exhibits distinct local spread patterns along cartilaginous surfaces, with the facial and submandibular lymph nodes at higher metastatic risk. Current classifications lack consensus, hindering comparison of outcomes. Treatment varies, with surgery or radiotherapy for early-stage tumors and multimodality approaches for advanced cases. The choice between surgery and radiotherapy is debated, with potential advantages and drawbacks for each. Radiotherapy, especially with Interventional RadioTherapy (IRT, previously known as brachytherapy), is gaining prominence, showing promising outcomes in terms of local control and cosmetic results. Prophylactic neck treatment remains controversial, with indications based on tumor characteristics. Prognostic factors include T classification, tumor size, surgical margins, nodal involvement, and histological features. Long-term survival rates range widely, emphasizing the need for further studies to refine management strategies for this rare malignancy. In conclusion, NVSCC poses diagnostic and therapeutic challenges, warranting multidisciplinary approaches and continued research efforts to optimize patient outcomes., (© 2024. The Author(s).)

Hamartomas, as non-true tumors, are commonly found in the lungs, digestive tract, and kidneys, and there are few clinical reports on nasal cases. Nasal histiocytosis accompanied by lymphatic lesions is a common feature of nasal hamartoma. Three patients with nasal cavity hamartoma were treated in our department, including 1 male and 2 females, with nasal obstruction or epistaxis. The imaging (computed tomography, magnetic resonance imaging) of 2 patients showed a soft tissue mass in the nasal cavity and no erosion of bone, while in 1 patient, the imaging was inconclusive. The excised material in all patients was found to be a soft mass. The 3 cases showed the same pattern under histological examination, and all 3 patients were diagnosed with nasal cavity respiratory epithelial adenomatoid hamartoma (REAH). Nasal hamartoma is a rare disease with nonspecific clinical manifestations and imaging features and is often misdiagnosed. Only by a better understanding of this disease can the rate of correct clinical diagnosis be improved., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Background: This study aims to assess the clinical efficacy of elective neck irradiation (ENI) in patients with esthesineuroblastoma (ENB), a rare malignant neoplasm, who are clinically node-negative., Methods: We conducted a retrospective analysis of 178 patients newly diagnosed with ENB at our institution between 2009 and 2021. Propensity score matching (PSM) was employed to compare node-negative patients treated with and without ENI. We extensively examined survival outcomes and treatment failure., Results: Of the 178 participants, 149 (83.7%) were lymph node-negative and staged in Modified Kadish A-C. 96 patients underwent ENI treatment, while 53 did not. At baseline, patients who received ENI differed from those who did not in terms of radiotherapy technique, staging, orbital invasion, surgical mode, and chemotherapy. After PSM, 43 pairs were available for analysis. ENI was observed to extend overall survival (OS, 5-year 73.9% vs. 84.0%; 3-year 76.9% vs. 97.1%, p = 0.022), progression-free survival (PFS, 5-year 38.5% vs. 84.6%; 3-year 50.5% vs. 94.5%, p < 0.001) and locoregional relapse-free survival (LRFS, 5-year 42.7% vs. 84.6%, p = 0.023; 3-year 57.3% vs. 94.5%, p < 0.001) in node-negative ENI patients. Failure pattern analyses revealed that ENI, which included level Ib, II, VIIa, significantly reduced the treatment failure rate. Furthermore, ENI did not significantly impact the prognosis of T1-2 patients, indicating potential clinical value of ENI in T3-4 patients., Conclusions: Our findings suggested that ENI decreased regional failure and significantly enhanced LRFS and PFS. ENI may be considered as an integral part of the initial treatment strategy for locally advanced node-negative ENB patients., (© 2024. The Author(s).)

Objectives: We present an adolescent in whom olfactory neuroblastoma (ONB) was detected on follow-up magnetic resonance imaging (MRI) 2.5 years after SIADH diagnosis. Our case contrasts prior pediatric reports in which ONB and SIADH were diagnosed concurrently., Case Presentation: A previously healthy 13-year-old girl was found to have SIADH during evaluation for restrictive eating. Work-up ruled out adrenal, thyroid and paraneoplastic causes, diuretic use, and vasopressin receptor and aquaporin channel mutations. Brain MRI was normal except for paranasal sinus (PNS) inflammatory changes to the left fronto-maxillary sinuses and frontoethmoidal recess. The sodium levels normalized with fluid restriction (800-900 ml/m 2 /day). Multiple repeated attempts to liberalize fluid intake resulted in recurrent hyponatremia. Follow-up brain MRIs 4 and 11 months after the initial presentation showed persistent PNS inflammatory changes. A subsequent brain MRI 31 months after initial presentation demonstrated a lesion in the left frontoethmoidal recess extending into the left nasal cavity and biopsy showed low grade ONB. The patient underwent surgery with normalization of serum sodium on liberalized fluid intake. Seven days after surgery, she had recurrence of SIADH, and brain MRI showed remnant of the ONB at the fovea ethmoidalis. She completed adjuvant radiotherapy though her SIADH persisted., Conclusions: Our case highlights the importance of considering ONB in the evaluation of children with SIADH. Idiopathic SIADH is rare in children and if no cause is identified, computed tomography of sinuses and nasal endoscopy should be considered earlier in the work-up of these patients, particularly in the absence of sinus symptoms., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2024 Sasidharan Pillai, Boxerman, Groblewski, DeNardo, Faizan, Topor, Robilliard and Serrano-Gonzalez.)

Purpose: Mesenchymal neoplasms composed of vascular, smooth muscle, and adipocytic components are uncommon in the nasal cavity. While angioleiomyoma (AL) is a smooth muscle tumor in the Head & Neck WHO classification, it is considered of pericytic origin in the Skin as well as Soft Tissue and Bone classifications. For nasal AL with an adipocytic component, the terms AL with adipocytic differentiation and angiomyolipoma (AML) have been applied, among others. AML is a type of perivascular epithelioid cell tumor (PEComa), most often arising in the kidney, sometimes associated with the tuberous sclerosis complex (TSC). It is uncertain whether nasal cavity AML and AL are best considered hamartomas or neoplasms, as their genetics are largely unexplored., Methods: We performed a multi-institutional retrospective study of nasal cavity mesenchymal lesions. Patient demographics, clinical histories, and histologic and immunohistochemical findings were collected. DNA and RNA were extracted from formalin-fixed, paraffin-embedded tissue and analyzed by SNP-based chromosomal microarray, targeted RNA fusion sequencing, and whole-exome sequencing., Results: Fifteen lesions (3-42 mm) were identified, predominantly in male (87%) patients with a median age of 60. Patients typically presented with obstructive symptoms, and none had a history of TSC. One AL was a recurrence from six years prior; 11 cases showed no recurrence (median 4.7 years, range: 0.88-12.4). Morphologically, 11 AML contained 30-80% smooth muscle, 10-25% vasculature, and 2-60% adipose tissue, while four AL contained 70-80% smooth muscle and 20-30% vasculature. Other histologic observations included ulceration, thrombosis, inflammation, myxoid change, senescent nuclei, and extramedullary hematopoiesis; no well-developed epithelioid cell morphology was identified. Immunohistochemically, all cases were positive for smooth muscle markers (actin, desmin, and/or caldesmon) and negative for melanocytic markers. Molecular analysis revealed loss of 3p and 11q in a single AML. No other known pathogenic copy number or molecular alterations were seen, including in TSC1/2, TFE3, or NOTCH2., Conclusion: Nasal cavity AML lacks morphologic, immunophenotypic, and genetic features of PEComa family AML. The significant histologic overlap between nasal AML and AL without distinguishing molecular features in either entity suggests "sinonasal angioleiomyoma with adipocytic differentiation" may be the most appropriate terminology for hybrid vascular and smooth muscle lesions containing adipocytic components., (© 2024. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Purpose: This study aimed to investigate the clinical and histopathological characteristics of sinonasal seromucinous hamartomas (SHs)., Methods: Eight patients with sinonasal SH and treated at a tertiary hospital between November 2005 and September 2023 were included. Additionally, a systematic review of published articles was conducted, analyzing 48 cases of SH described in the literature., Results: Among the eight patients treated at our institution, tumors originated from the posterior nasal cavity in four patients and middle turbinate and middle meatus were the primary origin in two patients each. Coexistence of inflammatory nasal polyps (NPs) was observed in four cases. Histopathologically, four patients exhibited focal respiratory epithelial adenomatoid hamartoma (REAH) features, and low-grade dysplasia was found in one patient. A combined analysis with previous literature revealed that 46.3% of all cases originated in the anterior nasal cavity. The proportions of cases accompanied by NPs and those with focal REAH features were 20.5% and 39.1%, respectively. Additionally, the frequencies of cases exhibiting dysplastic features (5.4%) and recurrence (2.1%) were low. Remarkably, tumors originating from the anterior region tended to have a higher frequency of dysplasia than those originating from the posterior region, although this difference was not statistically significant (p = 0.0996)., Conclusion: Patients with sinonasal SH showed favorable treatment outcomes following surgical resection. Focal REAH features and accompanying NPs were frequently observed. A substantial proportion of cases originate in the anterior nasal cavity, and these tumors may exhibit a high tendency for dysplasia., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Objective: Confocal laser endomicroscopy (CLE) is an optical imaging technique that allows in vivo, real-time, microscope-like assessment of superficial lesions. Although there is substantial data on CLE use in the upper GI tract, there is limited information regarding its application in the nasal cavity and paranasal sinuses. This study aims to assess the feasibility and diagnostic metrics of CLE in the nasal cavity and paranasal sinuses regarding differentiation between healthy/benign and malignant tissue. These structures show, however, a wider variety of frequent and concomitant benign and malignant pathologies, which could pose an increased challenge for optical biopsy by CLE., Methods: We performed CLE on a case series of six patients with various findings in the nose (three chronic rhinosinusitis, adenocarcinoma, meningoenzephalozele, esthesionneuroblastoma). Forty-two sequences (3792 images) from various structures in the nasal cavity and/or paranasal sinuses were acquired. Biopsies were taken at corresponding locations and analyzed in hematoxylin and eosin staining as a standard of reference. Three independent examiners blinded to the histopathology assessed the sequences., Results: Healthy and inflamed mucosa could be distinguished from malignant lesions with an accuracy, sensitivity, specificity, positive predictive value, and negative predictive value of 84.1%, 85.4%, 83.1%, 72.5%, and 92.1%, respectively, with a substantial agreement between raters (Fleiss κ = 0.62)., Conclusion: This technique shows, despite its limitations, potential as an adjunctive imaging technique during sinus surgery; however, the creation of a scoring system based on reproducible and defined characteristics in a larger more diverse population should be the focus of further research to improve its diagnostic value and clinical utility., Level of Evidence: NA Laryngoscope, 134:4217-4224, 2024., (© 2024 The American Laryngological, Rhinological and Otological Society, Inc.)

Abstract: Olfactory neuroblastoma (ONB) is an uncommon malignant tumor typically located in the upper nasal cavity. Olfactory neuroblastoma originating in the nasopharynx is extremely rare and tends to be misdiagnosed. The authors describe a rare case of ONB arising ectopically in the nasopharynx. The patient was a 65-year-old woman with recurrent epistaxis and a feeling of fullness in the right ear. After evaluation, endoscopic surgery was performed. The pathological result proved to be ONB. Postoperative magnetic resonance imaging showed that the tumor was completely resected. The patient proceeded to have 66 Gy of postoperative intensity-modulated radiotherapy and was followed for 36 months without tumor recurrence. Olfactory neuroblastoma originating from the nasopharynx is more rare condition compared with ONB located in other areas in the literature. The symptoms of ONB ectopic to the nasopharynx are similar to those of other nasopharyngeal tumors, which were likely to be misdiagnosed. The treatment principle is the same as that of nonectopic ONB, which is surgery combined with radiotherapy. Surgery can be performed using an endoscopic transnasal approach., Competing Interests: The authors report no conflicts of interest., (Copyright © 2024 by Mutaz B. Habal, MD.)

Objective: Olfactory neuroblastoma is a rare sinonasal malignancy with comparatively positive prognosis and survival, but with a range of biological behaviors that can be difficult to prognosticate with current means of risk stratification. Neutrophil-to-lymphocyte ratio (NLR) has been found across a diverse range of malignancies to be associated with poorer outcomes. This paper aims to elucidate the relationship of NLR with olfactory neuroblastoma to assess its prognostic value in this setting., Study Design: Retrospective chart review., Setting: A single tertiary care academic hospital., Methods: The study cohort included all patients treated for initial presentation of olfactory neuroblastoma from 2004 to 2020. NLR was calculated from preoperative labs, and each patient was evaluated for Kadish staging, Hyams grade, intraoperative positive margin, use of adjuvant therapy, posttreatment recurrence, and death. All statistical analysis was conducted using R and relationship between NLR and variables was assessed via binomial logistic regression., Results: Forty-four patients were included, 24 were male. Average age 52.8, average length of follow-up was 9.6 years. Patients were grouped by low (Kadish A/B) and advanced (Kadish C/D) stage, n = 23 and n = 21, respectively, and low (Hyams I/II) and high (Hyams III/IV) risk, n = 15 and n = 11, respectively. Advanced Kadish stage was associated with elevated NLR, odds ratio 5.69 [2.30, 20.7], P = .001. No other variables were associated with elevated NLR including Hyams grade, margin status, recurrence, and mortality., Conclusion: Higher Kadish grade is associated with elevated NLR which may provide novel prognostic value to current risk-stratifying systems., (© 2024 The Author(s). Otolaryngology–Head and Neck Surgery published by Wiley Periodicals LLC on behalf of American Academy of Otolaryngology–Head and Neck Surgery Foundation.)

Although rhinoliths are rare, they deserve attention and should be considered in the differential diagnoses of calcified lesions that can affect the nasal cavity and maxillary sinus region. This article reports a case of a large rhinolith in the nasal cavity caused by cocaine use. Initially, the patient denied drug use, leaving several uncertainties regarding the diagnosis, including recurrent sinusitis. However, after the histopathologic results, the patient admitted to previous cocaine use, emphasizing the importance of honesty in the medical history to guide diagnostic hypotheses, as well as the awareness of the diagnostic possibility of a rhinolith to assist in treatment., Competing Interests: The authors report no conflicts of interest., (Copyright © 2024 by Mutaz B. Habal, MD.)

Background and Objectives: Empty nose syndrome (ENS) is a debilitating condition that often results from traumatic or iatrogenic causes, such as nasal surgery. There are various conservative and surgical treatments for ENS, but their effectiveness remains uncertain. Therefore, the development of animal models that accurately mimic human ENS is essential for advancing effective treatment strategies. Materials and Methods: To investigate ENS development, turbinoplasty was performed in the nasal cavity of swine, entailing partial removal of the ventral turbinate using turbinectomy scissors followed by electrocauterization. After 56 days, samples were obtained for histological and morphological analyses. Results: A significant reduction in the volume of the ventral turbinate in the ENS model led to an expansion of the nasal cavity. Histological analysis revealed mucosal epithelial changes similar to those observed in ENS patients, including squamous cell metaplasia, goblet cell metaplasia, submucosal fibrosis, and glandular atrophy. Biomarkers related to these histopathological features were identified, and signals potentially contributing to squamous cell metaplasia were elucidated. Conclusions: The swine ENS model is anticipated to be instrumental in unraveling the pathogenesis of ENS and may also be useful for evaluating the effectiveness of various treatments for ENS.

The clinical data of five patients diagnosed with olfactory neuroblastoma (ONB) who were admitted to the Department of Pediatrics, Beijing Tongren Hospital Affiliated to Capital Medical University from January 2012 to January 2024 were retrospectively analyzed. Two males and three females aged 6.2 (5.7-15.8) years were included. The symptoms mainly covered nasal congestion, increased nasal secretions, headache, decreased vision and so on. Pathological grade Ⅱ, Ⅲ and Ⅳ was identified in two cases, one case and two cases, respectively. Modified Kadish stage B, C and D was detected in one case, two cases and two cases, respectively. All patients underwent surgery, chemotherapy, and radiation therapy. Among the five patients, four survived and one died. The follow-up time was 22.3 (10.4-56.4) months, and the recurrence rate was 0. ONB should be suspected when tumors are presented in the upper and middle parts of the nasal cavity, especially dumbbell shaped masses that grow towards the nasal cavity and intracranial area based on imaging. The multimodality therapy of ONB comprising of surgery and chemotherapy, can achieve good therapeutic effects and prognosis, but long-term follow-up is required.

Background: Non‑intestinal adenocarcinoma of the nasal cavity and paranasal sinuses (non‑ITAC) is a heterogeneous tumour that has rarely been reported in previous studies. We compared and analysed the symptoms, radiographic and pathological features, treatment methods, and prognosis of patients with low-grade (G1) and high-grade (G3) tumours., Methods: This was a retrospective study included 22 patients with pathologically confirmed non-ITAC of the nasal cavity and paranasal sinuses who were treated between January 2008 and December 2021 at a single centre. Of these, 11 patients had G1 tumours, and 11 patients had G3 tumours. Clinicopathological features, treatment methods, and survival outcomes were analysed., Results: The median follow-up period was 48.5 months. Nasal congestion was the most common initial symptom, and the nasal cavity was the most frequently involved site. For G1 tumours, the main treatment was simple surgery, 1 and 3‑year overall survival (OS) rates were 100 and 88.9%, while the 1 and 3‑year local control (LC) rates were 100 and 100%, respectively. For G3 tumours, the main treatments were surgery combined with radiotherapy and/or chemotherapy,1 and 3‑year OS rates were 72.7 and 72.7%, while the 1 and 3‑year LC rates were 100 and 90.91%, respectively. G3 tumours was associated with significantly shorter overall survival than G1 tumours (P = 0.035). Patients with stage III-IV showed shorter overall survival compared to stage I-II patients (P = 0.035)., Conclusions: Non-ITAC of the nasal cavity and paranasal sinuses may frequently occur in the nasal cavity. The main treatment modality is surgery, supplemented by radiotherapy and chemotherapy. Pathological grade and tumour stage were poor prognostic factors for the disease., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

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.)

Purpose of Review: The purpose of this review is to analyze the diagnosis and treatments of the sinonasal malignant tumors throw systematic reviewed literature. The systematic review of the literature was performed according to PRISMA guidelines., Recent Findings: Total 11,653 cases of five article were analyzed. The cohort of 3824 cases received appropriate treatment. The most frequent histotype of the group of sinonasal malignancies was squamous cell carcinoma. Squamous cell carcinoma was represented by 54%. The other histopathological subtypes were esthesioneuroblastoma with 9,9%, melanoma 9,8%, adenocarcinoma 7,5%, sarcoma 7,3%, adeno cystic carcinoma 7,1%, sinonasal undifferentiated carcinoma 3,9%, sinonasal neuroendocrine carcinoma 2,8% respectively. All 772 cases of total 3824 were treated only surgically. All 62 cases of total 3824 were treated without surgery, 20 cases with proton technique and SFUD, and 42 cases with proton technique and IMRT. The other 2990 cases of total 3824 were treated with multimodality treatment. The diagnosis and treatment of sinonasal cancers require a interdisciplinary approach and multimodality treatment., (© 2024. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Ewing-like sarcomas (ELS) are round cell mesenchymal neoplasms that are highly aggressive to bone and/or soft tissue. However, they rarely occur in the nose, with no reported such cases in the medical literature to date. Here, we reported the case of a two-year-old Chinese boy who presented with a mass in the left nasal cavity. Surprisingly, the final histopathological diagnosis of the nasal mass was determined to be a subtype of ELS, BCOR -rearranged sarcoma. Therefore, we retrospectively analyzed the clinical data of this case and reviewed the relevant literature on ELS and BCOR -rearranged sarcoma. The purpose of this article is to provide new insights into the clinical characteristics of children with BCOR -rearranged Ewing-like sarcoma and to improve the understanding of this disease., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

In the sinonasal tract, two tumor types are defined by neuroectodermal differentiation. Olfactory neuroblastoma (ONB), the traditional member of this category, is a keratin-negative neuroectodermal neoplasm that has lobulated architecture and variable neurofibrillary matrix. Olfactory carcinoma (OC), a newly-recognized diagnosis in the sinonasal tract, has keratin-positive neuroectodermal elements frequently intermixed with well-formed glands. These two neuroectodermal entities can show substantial overlap with other sinonasal small round blue cell tumors that express neuroendocrine markers. This review provides a practical overview of the key clinical and diagnostic features of ONB and OC and their differential diagnosis., Competing Interests: Disclosure The authors have nothing to disclose., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Long noncoding RNAs (lncRNAs) participate in transcriptional, epigenetic, and post-transcriptional regulation of gene expression and may influence carcinogenesis. MALAT1 is a lncRNA that is expressed in endocrine and many other neoplasms and it has been shown to have oncogenic and/or tumor suppressor effects in tumor development. Olfactory neuroblastomas arise in the nasal cavity while sympathetic neuroblastomas are present mainly in the adrenal and periadrenal regions. These neoplasms have overlapping histopathological features. Rare cases of sympathetic neuroblastomas metastatic to the nasal cavity have been reported. PHOX2B has been shown to be relatively specific for sympathetic neuroblastomas, but only a limited number of cases of olfactory neuroblastomas have been examined for PHOX2B expression. This study aimed to explore the potential utilization of MALAT1 and PHOX2B in distinguishing these two entities. Tissue microarrays (TMA) were created for olfactory neuroblastomas (n = 26) and sympathetic neuroblastomas (n = 52). MALAT1 lncRNA expression was assessed by in situ hybridization using RNAScope technology. TMA slides were scanned by Vectra multispectral imaging system and image analysis and quantification were performed with inForm software. PHOX2B expression was analyzed by immunohistochemistry. MALAT1 showed predominantly nuclear expression in both tumor types and MALAT1 expression was 2-fold higher in olfactory neuroblastomas compared to sympathetic neuroblastomas (p < 0.0001). PHOX2B showed nuclear staining in most sympathetic neuroblastomas (51/52, 98 %) while only 1 olfactory neuroblastoma (3.8 %) was focally positive for this marker. These findings suggest immunostaining of PHOX2B could be an excellent marker in distinguishing between these two tumor types., Competing Interests: Declaration of competing interest None., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Olfactory neuroblastoma is a rare sinonasal malignancy arising from the olfactory epithelium that is characterized by skull base involvement and a modest natural history. Because of its rarity and long course, identification of independent prognostic factors is dependent on multivariate analysis of large, long-term data. In this review, we outline evidence for the evaluation and treatment of olfactory neuroblastoma obtained from recent large-scale population-based studies, meta-analyses and multicenter studies. Hyams grade is currently the only pathological grade system for olfactory neuroblastoma. The modified Kadish staging and Dulguerov classification are available for clinical staging. The results of large-scale studies have confirmed Hyams, the modified Kadish and Dulguerov as independent prognostic factors. Surgery followed by radiotherapy provides the best overall survival and recurrence-free survival for resectable disease. The question of whether postoperative radiotherapy should be administered for all cases or only for those at risk of recurrence remains unanswered. Exclusively endoscopic resection is indicated for modified Kadish A/B cases without any increase in the risk of death or recurrence, and is also indicated for modified Kadish C cases if a negative surgical margin is ensured. For more advanced cases, such as those with extensive brain infiltration, the open approach is indicated. Elective nodal irradiation prevents late nodal recurrence of N0 patients. Chemotherapy has failed to show a benefit in survival or disease control. Current needs for olfactory neuroblastoma include the development and validation of refined staging systems suitable for current practice; expansion of indications for endoscopic surgery; less invasive surgery; definitive radiotherapy and novel systemic therapy., (© The Author(s) 2024. Published by Oxford University Press. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.)

Competing Interests: Declaration of Conflicting InterestsThe authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Olfactory neuroblastomas rarely secrete adrenocorticotropic hormone, leading to ectopic adrenocorticotropic hormone syndrome. However, the prevalence, timing, and triggers of ectopic adrenocorticotropic hormone syndrome in patients with olfactory neuroblastomas remain unclear. This study aimed to investigate these factors and conduct a literature review. Fifteen patients with olfactory neuroblastomas who underwent surgery at our institution were included. The prevalence of ectopic adrenocorticotropic hormone syndrome development was assessed by evaluating adrenocorticotropic hormone expression using immunohistochemistry. Furthermore, 26 patients with olfactory neuroblastomas who developed ectopic adrenocorticotropic hormone syndrome from previous reports were reviewed. Among the 15 patients, three (20%) showed adrenocorticotropic hormone-positive tumor cells at the time of initial surgery, and two (13%) developed ectopic adrenocorticotropic hormone syndrome. The timing of developing ectopic adrenocorticotropic hormone syndrome was 2.5 and 10 years following the initial treatment of olfactory neuroblastoma. Based on the literature review, nine patients with recurrent and metastatic olfactory neuroblastoma developed ectopic adrenocorticotropic hormone syndrome after the initial surgery, of whom, three had confirmed disease after developing ectopic adrenocorticotropic hormone syndrome, three developed during disease progression, two developed after receiving chemotherapy, and one developed after undergoing a biopsy. The timing of ectopic adrenocorticotropic hormone syndrome was 2.5-15 years after initial treatment. Our study revealed that acknowledging olfactory neuroblastomas can manifest as ectopic adrenocorticotropic hormone syndrome with a certain low prevalence is crucial. Moreover, our study speculated that tumor stimulation, such as biopsy or chemotherapy, as well as disease progression, could trigger ectopic adrenocorticotropic hormone syndrome onset. Thus, olfactory neuroblastomas can develop into ectopic adrenocorticotropic hormone syndrome, even long after the initial treatment.

Background: .-Sinonasal malignancies are rare, aggressive, deadly and challenging tumors to diagnose and treat. Since 2000, age-adjusted incidence rates average less than 1 case per 100,000 per year, male and female combined, in the United States. For the entire cohort, 2000-2017, overall median age-onset was 62.6 years. Carcinoma constitutes over 90% of these upper respiratory cancers and most cases are advanced, more than 72% (regional or distant stage) when the diagnosis is made. Composite mortality at 5 years was 108 excess deaths/1000/year with a mortality ratio of 558%, and 41% of deaths occurred in this time frame. As a consequence, observed median survival was approximately 6 years with 5-year cumulative observed survival (P) and relative survival rates (SR) 53% and 60%. This mortality and survival update study follows the World Health Organization International Classification of Diseases for Oncology-3rd Edition (ICD-O-3)1 topographical identification, coding, labeling and listing of 13,404 patient-cases accessible for analysis in the United States National Cancer Institute's Surveillance, Epidemiology and End Results program (NCI SEER Research Data, 18 Registries), 2000-2017 located in 8 primary anatomical sites: C30.0-Nasal cavity, C30.1-Middle ear, C31.0-Maxillary sinus, C31.1-Ethmoid sinus, C31.2-Frontal sinus, C31.3-Sphenoid sinus, C31.8-Overlapping lesion of accessory sinuses, C31.9-Accessory sinus, NOS., Objectives: .-1) Utilize national population-based SEER registry data for 2000-2017 to update cancer survival and mortality outcomes for 8 ICD-O-3 topographically coded sinonasal primary sites. 2) Discern similarities and contrasts in NCI-SEER case characteristics. 3) Identify current risk pattern outcomes and shifts in United States citizens, 2000-2017., Methods: .-SEER Research Data, 18 Registries, Nov 2019 Sub (2000-2017)2,3 are used to examine the risk consequences of 13,404 patients diagnosed with sinonasal malignancies, 2000-2017, in this retrospective population-based study employing prognostic data stratified by topography, age, sex, race, stage, grade, 2 cohort entry time-periods (2000-06 & 2007-17), and disease-duration to 15 years. General methods and standard double decrement life table methodologies for displaying and converting SEER site-specific annual survival and mortality data to aggregate average annual data units in durational intervals of 0-1, 0-2, 1-2, 2-5, 0-5, 5-10, and 10-15 years are employed. The reader is referred to the "Registrar Staging Assistant (SEER*RSA)" for local-regional-distant Extent of Disease (EOD) sources used in the development of staging descriptions for the Nasal Cavity and Paranasal Sinuses (maxillary and ethmoid sinuses only) and Summary Stage 2018 Coding Manual v2.0 released September 1, 2020. Cancer staging & grading procedural explanations, statistical significance & 95% confidence levels4 are described in previous Journal of Insurance Medicine articles5,6 and other publications.7,8 Poisson confidence intervals at the 95% level based on the number of observed deaths are used in this study but not displayed here to conserve space on the mortality tables. Excluded were all death certificate only and those alive with no survival time., Results: .-In the SEER 18 registries, a total of 13,404 patient cases (2000-2017) were available for analysis with an incidence of less than one patient per 100,000 people. From this group, analysis for survival and mortality totaled 10,624 patients. Males comprised 59.3% of cases and females 40.7%. Whites represented 80.3% of cases and black, others & unknown patients comprised 19.7%. The most common anatomic site of malignancy was the nasal cavity (49.7%); least common was the frontal sinus (1.2%). From diagnosis, across the span of 8 primary sites, first-year mortality rates q ranged from 14.3% (C30.0-nasal cavity) to 30.2% (C31.8-overlapping sinus) with corresponding excess death rates (EDR) of 118/1000/year and 279/1000/year. For single sites, the 5-year cumulative survival ratio (SR) was highest for the nasal cavity (69.5%) and lowest for overlapping lesions of the accessory sinuses (47.2%) with EDRs of 76 and 169 per 1000 per year respectively Overall, 5-year relative survival (SR) for all sinonasal tract malignancies combined was 60.3%, excess mortality (EDR) 108 per 1000 per year and mortality ratio 558%., Conclusions: .-The 8 sinonasal cancer primary sites are characterized by a low percentage of cases in the localized stage (28%). Since excess mortality is high even in the localized stage, overall prognosis is very poor for all patients. Excess mortality persists in cancer of the sinonasal tract as long as 10-15 years after diagnosis and treatment. EDR in the 15-year durational-interval, all sinonasal sites combined remained significant at 27.6 per 1000 per year with continuing decrease in cumulative survival ratio (SR) to 43.9%., (Copyright © 2024 Journal of Insurance Medicine.)

Objective: Our aim was to explore the diagnostic value of dynamic contrast-enhanced magnetic resonance imaging (DCE-MRI)-related quantitative parameters for benign and malignant nasal cavity and sinus tumors., Methods: A total of 78 patients with nasal sinus tumors admitted to People's Hospital of Qingdao Chengyang District in China were enrolled in our study, Of the patients, 41 were diagnosed as having benign tumors and 37 as having malignant tumors by pathological diagnosis. All patients received DCE-MRI scans before surgery to derive time-intensity curves (TICs) and related quantitative parameters (flux rate constant [Kep], transfer constant [Ktrans], extravascular volume fraction [Ve]). The diagnostic surgical pathology results were used as the gold standard to analyze the diagnostic effect of TIC and related quantitative parameters of DCE-MRI, and the receiver operating characteristic (ROC) curves were plotted to determine the values of each parameter in predicting nasal sinus tumors., Results: The percentage of class I in the benign group was significantly higher than in the malignant group (P < .05); the percentage of class III in the benign group was significantly lower than in the malignant group (P < .05); the percentage of class II in the 2 groups was comparable (P > .05). Kep, Ktrans and Ve in the benign group were 0.338±0.124, 0.061±0.035 and 0.532±0.138, respectively; Kep, Ktrans and Ve in the malignant group were 0.785±0.211, 0.441±0.125 and 0.327±0.048, respectively. The levels of Kep and Ktrans were significantly lower in the benign group than in the malignant group (all P < .05); the levels of Ve were significantly higher in the benign group than in the malignant group (P < .05). The optimal Kep cut-off value for predicting malignant nasal sinus tumors was 0.510 min-1, with a sensitivity of 81.4% and specificity of 89.5%; the optimal Ktrans cut-off value for predicting malignant nasal sinus tumors was 0.206 min-1, with a sensitivity of 84.3% and specificity of 89.7%; the optimal Ve cut-off value for predicting malignant nasal sinus tumors was 0.384 min-1, with a sensitivity of 71.8% and specificity of 82.4%., Conclusion: DCE-MRI-related quantitative parameters are ideal for the diagnosis of benign and malignant nasal sinus tumors. This modality provides more data for the identification of the nature of the tumor, and thus merits clinical promotion and application.

Olfactory neuroblastoma is a rare malignant tumour arising from the olfactory nerve and extending into the nasal cavity. In this case report, the case of a 42-year-old male is presented. The patient had a two-month history of progressive nasal blockage and episodes of epistaxis. No complaint of anosmia or facial pain was reported. All the necessary examinations were performed. Upon investigation, the CT scan and MRI showed a polypoid mass involving the right maxillary sinus, eroding the medial wall and expanding into the osteo-meatal complex. The diagnosis of olfactory neuroblastoma was confirmed through histopathological examination and further validated by immunohistochemistry as it was positive for synaptophysin, chromogranin, gamma enolase, and neurofilament. On staging, the tumour was Kadish B. The mass was excised by lateral rhinotomy. The patient was kept on radiotherapy and was free from recurrence upon follow-up 10 months later. It was concluded that based on the analysis of findings related to olfactory neuroblastomas, clinicians should contemplate the possibility of an ONB when radiographic images depict a dumbbell-shaped mass within the nasal cavity, accompanied by peritumoural cysts. Using a multimodal treatment approach is advisable.

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.)

Rosai-Dorfman disease (RDD) is a rare non-malignant disorder, characterized by painless multiple cervical lymphadenopathy, fever, and elevated inflammatory markers. Its diagnosis is difficult due to its rare incidence and various clinical presentations, especially in extranodal involvement. In this report, we demonstrate a patient with RDD who presented with a nasal septum and laryngeal tumor that caused dyspnea. We achieved a successful treatment outcome with combined surgical resection of the laryngeal mass and corticosteroid medication. The symptoms and tumors were resolved within 3 weeks after treatment. We reported our experiences with review of literatures., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Objective: To explore the imaging features of rare tumors of nasal cavity and sinuses, and to improve the understanding of these diseases, thereby aiding clinical diagnosis and treatment. Methods: The CT and MRI findings of 79 cases of rare neoplasm of nasal cavity and sinuses confirmed by pathology were retrospectively analyzed, and the imaging features were summarized. Results: Among the 79 cases, there were 16 cases of neuroendocrine carcinoma, most showing expansive and infiltrative bone destruction without hyperosteogeny and sclerosis. The sphenoid sinus exhibited a "pigeon" shape. In 28 cases of malignant melanoma, MRI signals were diverse, typical signals were rare, but mixed signals were more common. In 12 cases of rhabdomyosarcoma, MRI enhancement mostly showed "grape-like" enhancement and partial ring enhancement; There were 10 cases of olfactory neuroblastoma, the lesions were consistent with the distribution area of olfactory mucosa, most of them were lobulated, marginal nodules, and "flower ring" enhancement, and 2 cases grew across intracranial and external, with multiple cystic lesions and surrounding flaky edema bands. In 5 cases of solitary fibrous tumor, Benign tumors had regular shape and uniform density, while malignant tumors had irregular shape and uneven density, The enhancement was obviously uneven and showed a "pattern" change. There were 2 cases of sarcomatoid carcinoma, both with lobed appearance, uneven density, lamellar low-density shadow, and osteolytic bone destruction. In 4 cases of schwannoma, the enhancement showed obvious inhomogeneous enhancement. One case showed cystic necrosis, one case showed calcification, and the surrounding structure was compressed without damage. There was 1 case of neurofibroma, with many cystic components, low signal separation and compartmentalized enhancement. One case of paraganglioma showed moderate enhancement in the arterial phase and progressive enhancement in the venous phase, accompanied by significant swelling bone destruction. Conclusion: Rare tumors of nasal cavity and paranasal sinuses have distinctive imaging features. CT and MRI can effectively show the extent of the lesions and the degree of infiltration into adjacent tissues and organs, which is helpful for early clinical diagnosis and staging. However, definitive diagnosis still depends on pathology and immunohistochemistry., Competing Interests: The authors of this article and the planning committee members and staff have no relevant financial relationships with commercial interests to disclose., (Copyright© by the Editorial Department of Journal of Clinical Otorhinolaryngology Head and Neck Surgery.)

Sinonasal malignant tumors are a group of uncommon malignancies that account for less than 1% of all tumors. These tumors often involve the maxillary sinus and nasal cavity, with less cumulative incidence in the ethmoidal sinus, sphenoidal sinus, and frontal sinus. The lack of consensus on the management of sinonasal malignancies is due to their rarity, diagnostic challenges, and the heterogeneity of treatments. In this paper, we present a case of endoscopic-assisted medial canthus incision combined with radiotherapy in the treatment of sinonasal malignant tumors, with the aim of providing valuable insights to clinicians on the management of these tumors., (© 2024. The Author(s).)

Olfactory neuroblastoma (ONB) is an uncommon malignant tumor and is usually treated by a multidisciplinary approach includes surgery, radiotherapy, and chemotherapy. A 62 years-old male had a tumor in the nasal cavity and diagnosed as ONB with Kadish A stage. Anterior skull base surgery was performed as radical treatment. Since the surgical margin was negative, no postoperative radiotherapy was administered. 14 years after the surgery, bilateral otitis media with effusion (OME) was occurred, we found the recurrence tumor at bilateral retropharyngeal lymph node (RPLN) which surrounded the internal carotid arteries. Since these were unresectable, we planned chemoradiotherapy which was 70Gy of intensity modulated radiotherapy combined with two courses of carboplatin and etoposide. The tumor volume was reduced and bilateral OME were improved. He has been alive for 3 years after salvage treatment. Although ONB has a relatively good prognosis, it is known to often cause cervical lymph node metastasis. Grades III and IV of Hyams classification are considered high risk. This case, initial tumor was limited in the nasal cavity and its clinical classification was early stage, but Hyams classification was grade III. In reference to this case, considering that RPLN metastasis are difficult to radically resect at the salvage surgery, including this area in postoperative radiotherapy was considered an option.

Objective: To summarize and analyze the clinical manifestations, diagnosis and management and prognostic features of solitary fibrous tumor（SFT） in nasal cavity, sinus and skull base. Methods: The clinical data of 12 patients with STF from nasal cavity, sinus and cranial base admitted to the Affiliated Hospital of Qingdao University from April 2014 to January 2022 were retrospectively analyzed, including 4 patients admitted to the department of Otolaryngology head and neck surgery and 8 patients admitted to the department of skull base surgery The clinical characteristics, diagnosis, management and prognosis were analyzed. Results: Twelve patients were included in this research, including 7 males and 5 females. All patients received surgical treatment, and 4 patients also received postoperative adjuvant chemoradiotherapy. After follow-up for 12-60 months, 4 patients with adjuvant radiotherapy and chemotherapy had a good prognosis, and among 8 patients who did not receive radiotherapy and chemotherapy, 6 patients had good prognosis and 2 patients showed relapse. Four patients with a history of recurrence of SFT after surgery were admitted to our hospital for surgical treatment, in which 1 patient had relapse after surgery, and none had metastasis. Nasal cavity and sinus to skull base SFT is rare. The most effective treatment for this disease is surgical resection, and postoperative adjuvant chemoradiation and long-term follow-up can achieve a better prognosis. En bloc resection is the key to treatment success., Competing Interests: The authors of this article and the planning committee members and staff have no relevant financial relationships with commercial interests to disclose., (Copyright© by the Editorial Department of Journal of Clinical Otorhinolaryngology Head and Neck Surgery.)

NUT Carcinoma（NC） is a rare malignant tumor of unknown origin, which is highly aggressive. It is characterized by chromosome rearrangement accompanied by NUTM1 gene. The pathological manifestations were sudden and focal squamous in poorly differentiated or undifferentiated carcinoma. NUTM1 gene rearrangement can be used to diagnose NC. The prognosis of NUT cancer is poor. Clinically, there is no established treatment plan. treatment options mainly comprise surgery, radiotherapy and chemotherapy. A 74-year-old patient with NC of the nasal cavity and sinuses was reported. Her clinical presentation was right nasal congestion with facial swelling. Sinus CT and MRI showed soft tissue density in the right nasal cavity and maxillary sinus with bone destruction. After admission, the patient underwent nasal endoscopic biopsy, and the postoperative pathological FISH staining showed BRD4/NUT fusion t（15, 19）. The tumor was significantly reduced after two courses of sequential chemoradiotherapy. Two months later, the patient underwent a partial maxillary resection due to the rapid regrowth of sinusoidal mass, invading the hard palate. The patient died 2 months after surgery due to multiple organ failure resulted from tumor metastasis, with a survival time of 11 months. The clinical characteristics, diagnosis and treatment of this case were reported and related literature was reviewed., Competing Interests: The authors of this article and the planning committee members and staff have no relevant financial relationships with commercial interests to disclose., (Copyright© by the Editorial Department of Journal of Clinical Otorhinolaryngology Head and Neck Surgery.)

Objectives: To investigate the internal structure of the nasomaxillary complex, including the maxillary sinus, nasal cavity and nasal septum according to the facial asymmetry pattern and to evaluate its correlation with external maxillomandibular asymmetry in Class III patients based on cone-beam computerized tomography (CBCT) images., Materials and Methods: Facial asymmetry was analysed in a total of 100 Class III patients aged 16 years or older using CBCT scans. Patients were categorized into subgroups based on asymmetry pattern. Measurements of the nasomaxillary complex were obtained from the CBCT scans, including the volume and width of the maxillary sinuses and nasal cavities on deviated and non-deviated sides, as well as the displacement of the nasal septum. Statistical analysis was performed to compare the internal nasomaxillary variables within and between groups, and regression analysis was conducted to evaluate the correlation between facial asymmetry and the internal nasomaxillary variables., Results: Group comparisons showed that there were no significant differences in the volume of the maxillary sinus and nasal cavity. However, the direction and extent of nasal septum deviation, as well as the width of the nasal cavity, varied depending on the maxillary asymmetry pattern. Regression analysis indicated a correlation between nasal septum deviation and the difference in maxillary height, while the difference in nasal cavity width was correlated with the difference in maxillary width., Conclusion: A comprehensive evaluation of the internal nasal anatomy is vital for understanding the intricate relationship between nasal structure and maxillary growth., (© 2023 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)

Giant cell tumors of bone (GCT) are rare soft tissue tumors, that account for 3%-5% of primary bone tumors with <2% occurring in the head and neck. The nasal cavity is a highly unusual site of presentation. We reviewed 15 cases of GCT of nasal cavity and paranasal sinuses. We add 1 case to the literature. The case herein reported, appears to be the second nasal fossa GCT described in the literature and the first documented case with multifocal localization. A case of multifocal GCT of the nasal cavity is described. Although rare in the general population, GCT should be included among the possibilities in the differential diagnosis when evaluating tumors of the head and neck. Management of this particular tumor remains challenging; surgical removal is still the gold standard treatment, preferring a minimally invasive trans-nasal approach to reduce intra and post-operative morbidity. Laryngoscope, 134:2774-2778, 2024., (© 2023 The Authors. The Laryngoscope published by Wiley Periodicals LLC on behalf of The American Laryngological, Rhinological and Otological Society, Inc.)

Objectives: Squamous cell carcinoma of the nasal vestibule (NV-SCC) is a rare but challenging entity, due to the complex anatomy of the region. Consensus on the best treatment strategy is still lacking, as well as a dedicated staging system. Our aim was to analyze oncological outcomes of surgically treated patients and to investigate possible prognostic factors., Methods: We performed a retrospective multi-centric observational study including six Academic Hospitals over a 10-year period, including only patients who underwent upfront surgery for primary NV-SCC. Patients were staged according to all currently available staging systems. The Kaplan-Meier method was used to compute overall, disease-free, and disease-specific survival. Logistic regression models were used to correlate between survival outcomes and clinical and pathological variables., Results: Seventy-one patients with a median follow-up of 38 months were included in the study. Partial and total rhinectomy were the most commonly performed procedures, respectively, in 49.3% and 25.4% of cases. Neck dissection was performed on 31% of patients, and 45.1% of them underwent adjuvant radiotherapy. Three years overall, disease-specific and disease-free survival were, respectively, 86.5%, 90.3%, and 74.2%. None of the currently available staging systems were able to effectively stratify survival outcomes. Factors predicting lower overall survival on multivariate analysis were age (p = 0.021) and perineural invasion (p = 0.059), whereas disease-free survival was negatively affected by age (p = 0.033) and lymphovascular invasion (p = 0.019)., Conclusion: Currently available staging systems cannot stratify prognosis for patients who underwent surgery for NV-SCC., Level of Evidence: 4 Laryngoscope, 134:2634-2645, 2024., (© 2023 The American Laryngological, Rhinological and Otological Society, Inc.)

This case report describes 2 dogs, an Appenzeller Mountain dog and an Irish Wolfhound, with angioleiomyoma within the nasal cavity. Endoscopic surgical resection resulted in cure in both dogs. Macroscopically and on diagnostic imaging, tumor masses may appear malignant because of local turbinate destruction. This highlights the importance of histological examination before any recommendations are made to owners because tumors of the nasal cavity may be benign and surgery curative., Competing Interests: The authors confirm that they do not have any conflict of interest., (Thieme. All rights reserved.)

Nasal obstruction (NO) is a common clinical symptom characterized by a subjective sensation of insufficient airflow through the nasal cavity and may result from various factors, including changes in nasal anatomy, inflammatory conditions, tumoral lesions, and other etiologies. While a thorough medical history and physical examination can often identify its cause, imaging is usually necessary to fully understand the problem. Computed tomography (CT) is the primary imaging modality used to evaluate the nasal cavity and paranasal sinuses, allowing for the identification of potential causes and structural abnormalities. However, when soft tissue characterization is required, magnetic resonance imaging (MRI) is also useful. Understanding the anatomical and pathological basis of NO is crucial for accurate diagnosis and appropriate management. Imaging techniques provide valuable information for identifying the underlying causes of NO and guiding treatment decisions. This article reviews the normal anatomy of the nasal cavity and adjacent paranasal sinuses as well as the several conditions that may affect breathing comfort (tumors, inflammatory diseases, bony and cartilaginous anatomical variants, and "nonobstructive" mucosal thickenings), showing their normal presentation on CT and MRI., Competing Interests: None declared., (Thieme. All rights reserved.)

Background: Nasal polyps and inverted papillomas often look similar. Clinically, it is difficult to distinguish the masses by endoscopic examination. Therefore, in this study, we aimed to develop a deep learning algorithm for computer-aided diagnosis of nasal endoscopic images, which may provide a more accurate clinical diagnosis before pathologic confirmation of the nasal masses., Methods: By performing deep learning of nasal endoscope images, we evaluated our computer-aided diagnosis system's assessment ability for nasal polyps and inverted papilloma and the feasibility of their clinical application. We used curriculum learning pre-trained with patches of nasal endoscopic images and full-sized images. The proposed model's performance for classifying nasal polyps, inverted papilloma, and normal tissue was analyzed using five-fold cross-validation., Results: The normal scores for our best-performing network were 0.9520 for recall, 0.7900 for precision, 0.8648 for F1-score, 0.97 for the area under the curve, and 0.8273 for accuracy. For nasal polyps, the best performance was 0.8162, 0.8496, 0.8409, 0.89, and 0.8273, respectively, for recall, precision, F1-score, area under the curve, and accuracy. Finally, for inverted papilloma, the best performance was obtained for recall, precision, F1-score, area under the curve, and accuracy values of 0.5172, 0.8125, 0.6122, 0.83, and 0.8273, respectively., Conclusion: Although there were some misclassifications, the results of gradient-weighted class activation mapping were generally consistent with the areas under the curve determined by otolaryngologists. These results suggest that the convolutional neural network is highly reliable in resolving lesion locations in nasal endoscopic images., (© 2024. The Author(s).)

Nasal chondromesenchymal hamartoma (NCMH) is a rare benign polypoid mesenchymal tumor arising in the nasal cavity and/or paranasal sinuses. Recognizing these sporadic, rare lesions is crucial, as surgical complete removal of the mass is the common treatment approach. This retrospective study analyzed the demographics, symptoms, and imaging data of 9 patients diagnosed with NCMH between January 2017 and June 2023, possibly representing the largest single-center adult case cohort to date. Diagnostic techniques included nasal endoscopy, CT/MRI scan, immunohistological studies, and morphologic comparisons. Pathologic specimens were subjected to Sanger sequencing of exons 24 and 25 of DICER1. The average age of 9 cases was 24.4 years, and the oldest was 55 years. Four of the patients were children, ranging from 1 year old to 11 years old, with an average of 4.5 years. Nasal congestion is the most common registered symptom. Endoscopic findings showed that most patients had smooth pink neoplasms or polypoid masses in the nasal meatus. Radiologic scanning revealed soft-tissue density masses that occupied the nasal cavity. Histologically, the characteristic structure of NCMHs is immature cellular cartilage nodules and mature cartilage nodules distributed in a loose mucoid matrix. Five of the 9 patients had somatic DICER1 missense mutations. Four of the patients with DICER1-mutated NCMH exhibited a p.E1813 missense hotspot mutation. We also report a case of a rare p.P1836H missense mutation. The detected DICER1 somatic mutations provide compelling evidence of an association with the DICER1 tumor family. We emphasize the importance of pathologic consultation and the need for pathologists to accumulate experience in NCMH diagnosis to avoid misdiagnosis., Competing Interests: Conflicts of Interest and Source of Funding: The authors have disclosed that they have no significant relationships with, or financial interest in, any commercial companies pertaining to this article., (Copyright © 2024 Wolters Kluwer Health, Inc. All rights reserved.)

Introduction: Intravascular papillary endothelial hyperplasia (IPEH) predominantly occurs in the subcutaneous and dermal regions and rarely originates from the sinonasal mucosa., Case Presentation: We report on the case of a 58-year-old male patient who presented with progressive bilateral nasal obstruction, left-sided epiphora, and intermittent epistaxis. Computed tomography revealed a soft tissue opacity in the left maxillary sinus with intersinusoidal nasal wall demineralization, extending into the surrounding ethmoid cells and the right nasal cavity through a contralateral deviation of the nasal septum. Contrast-enhanced T1-weighted magnetic resonance imaging further confirmed these findings. The IPEH originating from the maxillary sinus extended into the contralateral nasal cavity, and it was successfully removed using an endoscopic endonasal approach, avoiding overly aggressive treatment., Conclusion: This case report highlights the diagnostic challenges of IPEH in the sinonasal region and the importance of considering IPEH as a differential diagnosis in patients presenting with nasal obstruction, epiphora, and intermittent epistaxis., (© 2024. The Author(s).)

Historically, comprehensive surgical resection for olfactory neuroblastoma has included the bilateral olfactory epithelium, cribriform plate, overlying dura, olfactory bulbs and tracts. This results in postoperative anosmia that may significantly impact a patient's quality of life without definitive added benefit in survival. The prevalence of occult intracranial disease is low, especially for Hyams grade I and II tumors. A unilateral approach sparing the contralateral cribriform plate and olfactory system can be considered for select cases of early stage, low-grade tumors when the disease does not cross midline to involve the contralateral olfactory cleft or septal mucosa and when midline dural margins can be cleared with frozen pathology. Approximately half of patients who undergo unilateral resection may have residual olfaction even with adjuvant unilateral radiation. Early data suggest favorable disease-free survival and overall survival for patients who underwent the unilateral approach; however, larger sample studies are needed to confirm comparability to bilateral resections regarding oncologic outcomes., (© 2024 ARS‐AAOA, LLC.)

Objectives: Discrimination of nasal cavity lesions using nasal endoscopy is challenging because of the differences in clinical manifestations and treatment strategies. We aimed to investigate the diagnostic accuracy of clinical visual assessment (CVA) of nasal cavity masses using endoscopic images and determine whether there is a difference according to pathologic class and the examiners' experience. Methods: We collected pathologically confirmed endoscopic images of normal findings, nasal polyp (NP), benign tumor, and malignant tumor (each class contained 100 images) randomly selected. Eighteen otolaryngologists, including six junior residents, six senior residents, and six board-certified rhinologists classified the test set images into four classes of lesions by CVA. Diagnostic performance according to the pathologic class and the examiner's experience level was evaluated based on overall accuracy, F1-score, confusion matrix, and area under the receiver operating characteristic curve (AUC). Results: Diagnostic performance was significantly different according to the pathological class of nasal cavity mass lesions with the overall accuracy reported high in the order of normal, NP, benign tumor, and malignant tumor (0.926 ± 0.100; 0.819 ± 0.135; 0.580 ± 0.112; 0.478 ± 0.187, respectively), F1 score (0.937 ± 0.076; 0.730 ± 0.093; 0.549 ± 0.080; 0.554 ± 0.146, respectively) and AUC value (0.96 ± 0.06; 0.84 ± 0.07; 0.70 ± 0.05; 0.71 ± 0.08, respectively). The expert rhinologist group achieved higher overall accuracy than the resident group (0.756 ± 0.157 vs. 0.680 ± 0.239, p  < .05). Conclusion: CVA for nasal cavity mass was highly dependent on the pathologic class and examiner's experience. The overall accuracy was reliably high for normal findings, but low in classifying benign and malignant tumors. Differential diagnosis of lesions solely based on nasal endoscopic evaluation is challenging. Therefore, clinicians should consider further clinical evaluation for suspicious cases., Competing Interests: Declaration of conflicting interestsThe authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Olfactory neuroblastomas are uncommon malignancies that arise from olfactory receptor cells located high in the nasal cavity. Accurate diagnosis plays a crucial role in determining clinical results and guiding treatment decisions. Diagnosis can be a major challenge for pathologists, especially when dealing with tumours with poor differentiation. The discovery of several molecular and immunohistochemical markers would help to overcome classification difficulties. Due to the paucity of large-scale studies, standardisation of diagnosis, treatment and prediction of outcome remains a challenge. Surgical resection by endoscopic techniques with the addition of postoperative irradiation is the treatment of choice. In addition, it is advisable to consider elective neck irradiation to minimise the risk of nodal recurrence. Molecular characterisation will help not only to make more accurate diagnoses but also to identify specific molecular targets that can be used to develop personalised treatment options tailored to each patient. The present review aims to summarise the current state of knowledge on histopathological diagnosis, the molecular biology and management of this disease., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Abstract: Primary mucosal malignant melanoma of the nasal cavity is a rare tumor with aggressive behavior and a dismal prognosis. An extremely rare tumor that accounts for 0.7% to 1% of all melanomas in Caucasian populations and between 4% and 8% of malignant tumors of the nasal cavity and paranasal sinuses. Taking into account the rarity, it is important to note that malignant melanoma should be considered when making a differential diagnosis of tumors of the nose and paranasal sinuses. Two cases of primary malignant melanoma of the nasal cavity both arising in females, one in a 60-year-old and the other in a 64-year-old, both of whom presented with nasal obstruction and brief symptomatic epistaxis are being presented here. The diagnosis being confirmed by a histopathological examination along with an immunohistochemical analysis by using S100 and HMB45., (Copyright © 2024 Copyright: © 2024 Journal of Cancer Research and Therapeutics.)

Small cell carcinoma (SCC) in the nasal cavity and sinuses is extremely rare. The clinical data of 15 patients with primary SCC in nasal cavity and sinuses were analyzed retrospectively. All patients were treated with surgery, radiotherapy, and chemotherapy. Of the 15 patients, 2 patients are alive for more than 6 years, and 5 patients died after the median follow-up period (11 months). Most of our patients represent the later stage (73% presented at stage III or IV) and had surgery combined with radiotherapy and chemotherapy; however, nearly half of patients have tumor recurrence and/or distant metastasis. SCC of nasal cavity and sinuses often invades surrounding tissues, and the long-term curative rate is generally low. Early diagnosis and comprehensive treatment are key to improve survival. Although the overall survival time of SCC is not optimistic, it is still recommended that patients take comprehensive treatment., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Purpose: To describe a novel endoscopic technique to approach the maxillary sinus (MS), the Modified Anterior Medial Maxillary Approach (MAMMA), preserving the inferior turbinate (IT) and the nasolacrimal duct (NLD). To perform radiological measurements and describe a case series to study the feasibility and limits of MAMMA., Methods: Computed tomography (CT) scans (n = 150 nasal cavities) were used to calculate areas of the MAMMA to define surgical limits and extensions. Measurement of distances to critical anatomy landmarks and total area for the MAMMA were calculated. An instructional case illustrating the surgical technique and outcome was also included., Results: Radiological analysis showed a mean distance from the Piriform Aperture (PA) to the anterior limit of the NLD of 1.03 ± 0.18 cm (range 0.59-1.48) and a mean distance from de PA to the posterior limit of the NLD of 1.57 ± 0.22 cm (range 1.02-2.11). The mean distance from the nasal floor to the Hasner's valve was 1.61 ± 0.27 cm (range 1.06-2.52) and the distance from the nasal floor to the insertion of the IT was 2.20 ± 0.36 cm (range 1.70-3.69). Finally, the mean total area for the MAMMA was 4.04 ± 0.52 cm2 (range 3.17-5.53). No complications or recurrence of the pathology were observed in operated patients., Conclusion: The MAMMA provides a wide surgical field of the MS walls comparable to more aggressive techniques, with preservation of the sinonasal and lacrimal function. MAMMA is an effective alternative to treat different MS pathologies including benign recurrent maxillary sinus tumors., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Rhinosporidiosis, an infectious granulomatous disease, is seldom encountered in the United States. We present a case of rhinosporidiosis in a 26-year-old man, who presented with an unusual mass in his nasal cavity. Suspicion for rhinosporidiosis was high due to the patient's travel and activity history. After imaging and proper diagnosis, surgery was performed to excise the lesion. As international travel resumes during the COVID-19 pandemic, the potential for encountering this rare organism is heightened., Competing Interests: Declaration of Conflicting InterestsThe authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.