Stokken, Janalee K.

AbstractObjective The objective of this study is to describe the clinical presentation, tumor characteristics, natural history, and treatment patterns of sinonasal osteosarcoma.Methods Fourteen patients who had been treated for osteosarcoma of the nasal cavity and paranasal sinuses at a tertiary care center were reviewed. In addition, a systematic review of the literature for osteosarcoma of the sinonasal cavity was performed.Results In a systematic review, including 14 patients from the authors' institution, 53 total studies including 88 patients were assessed. Median follow-up was 18 months (interquartile range: 8–39 months). The most common presenting symptoms were facial mass or swelling (34%), and nasal obstruction (30%). The most common paranasal sinus involved by tumor was the maxillary sinus (64%), followed by the ethmoid sinuses (52%). The orbit (33%), dura (13%) and infratemporal fossa (10%) were the most common sites of local invasion. The majority of patients underwent surgery followed by adjuvant therapy (52.4%). Increasing age was associated with decreased overall survival rate (unit risk ratio [95% confidence interval (CI)] = 1.02 [1.003–1.043]; p = 0.0216) and T4 disease was associated with decreased disease-specific survival rate (hazard ratio [HR] = 2.87; p = 0.0495). The 2- and 5-year overall survival rates were 68 and 40%, respectively, while 2- and 5-year disease-specific survival rates were 71% and 44%, respectively.Conclusion Sinonasal osteosarcomas are uncommon tumors and can pose a significant therapeutic challenge. Increasing age and T4 disease are associated with worse prognosis. This disease usually warrants consultation by a multidisciplinary team and consideration of multimodality therapy.

BackgroundIn this study we aim to describe presenting characteristics and identify prognostic factors for disease resolution in patients with chronic rhinosinusitis (CRS) in the setting of eosinophilic granulomatosis with polyangiitis (EGPA).MethodsPatients evaluated at a tertiary care center with diagnoses of EGPA and CRS were identified. Descriptive statistics were obtained. Univariate analysis was used to search for prognostic factors associated with higher Lund‐Mackay score at presentation and disease resolution.ResultsForty‐four patients were included with a mean age of 52.7 (standard deviation, 14) years. Twenty‐one patients (47.7%) were female, all had a diagnosis of asthma, and 36 (83.7%) had eosinophils >10%. Common presenting symptoms for CRS included nasal discharge (87.9%) followed by nasal congestion (83.9%) and facial pain and pressure (83.8%). Medical management of CRS included systemic corticosteroids (93.2%) and systemic antibiotics (75%). Surgical intervention occurred in 29 patients (67%). Nine patients (20.5%) had resolution of sinus symptoms, including 4 with imaging confirmation. Fourteen patients (31.8%) had continued CRS, but with improved symptoms, whereas 9 patients (20.5%) had continued CRS with no improvement in symptoms. Eleven patients (25%) were lost to follow‐up and 4 (9.1%) died. Univariate analysis did not show antineutrophil cytoplasmic antibody positivity, presence of peripheral eosinophilia, gender, age, or absence of systemic therapy to be predictive of higher Lund‐Mackay score at presentation or predictive of disease resolution.ConclusionCRS in patients with EGPA is often refractory to medical and surgical management. Treatment of these patients should occur in a multidisciplinary setting.

Objective Yellow nail syndrome (YNS) is characterized by the triad of thickened yellow nails, primary lymphedema, and respiratory manifestations including bronchiectasis, chronic cough, pleural effusions, and sinusitis. The purpose of this study is to describe the manifestations of chronic rhinosinusitis (CRS) in patients with YNS and the comorbid manifestations. Methods A retrospective review of patients with YNS with CRS from January 1, 1998 to January 1, 2018 was conducted. Their symptoms, comorbidities, and labs were reviewed. The patient’s sinus computed tomography (CT) scans were then scored using the Lund–Mackay (LM) scoring system. Result Thirty-six patients met the inclusion criteria. The average LM score was 10.2 ± 0.5 (mean ± SD). Sinus involvement included “frothy” dependent sinus secretions in 32 of 36 subjects. The most common location of sinus involvement was the maxillary sinuses (85%), then posterior ethmoid (81%), anterior ethmoid (76%), sphenoid (74%), and frontal sinuses (54%). Comorbid chronic cough (86%), bronchiectasis (65%), lymphedema (39%), and pleural effusion (19%) were also identified. Three patients (8%) had immunodeficiencies. Conclusion This is the largest series describing the sinus manifestations of CRS in patients with YNS. Sinus CT scans showed frothy dependent secretions in the maxillary sinuses and posterior ethmoid sinuses most commonly. YNS patients with sinusitis more commonly presented with chronic cough or bronchiectasis than with pleural effusions or lymphedema. Otolaryngologists should consider the possibility of YNS in patients with CRS, chronic cough, yellow or thickened nail, and CT findings of frothy, dependent sinus secretions.

BackgroundLymphatic dysfunction is hypothesized to be an underlying factor in the pathophysiology of yellow nail syndrome (YNS) (yellow nails, lymphedema, pleural effusions, and frequently chronic rhinosinusitis [CRS]). It is unclear why some YNS patients develop CRS. We quantified lymphatic and total vasculature of sinonasal mucosa in YNS patients and compared it to controls from CRS patients with (CRSwNP) or without polyps (CRSsNP).MethodsImmunohistochemistry was performed on archival sinonasal mucosal samples from 5 patients with YNS and 14 controls with antibodies against podoplanin and CD31, markers of lymphatics. Morphometric assessment was performed on digital images using ImageJ software.ResultsIn YNS, the number of lymphatics/mm2 ranged from 7 to 18/mm2 (controls: 6 to 43/mm2, p = 0.343), with a mean perimeter between 92 and 201 µm (controls: 42 to 280 µm, p = 0.482). Total vasculature density was higher than lymphatics, ranging between 189 and 1159 vessels/mm2, average 669 (controls: 139 to 1467/mm2, average 503, p = 0.257) with smaller average perimeter, 40 to 117 µm, mean 64.8 µm (controls: 42 to 92 µm, mean 65.3 µm, p = 0.965). Lymphatics constituted only a small fragment of the total vasculature, ranging from 1.15% to 4.76%, average 2.34% (controls: 0.81% to 10.58%, average 4.88%, p = 0.156). CRSwNP patients had significantly higher lymphatic density (p = 0.011) and ratio of lymphatics to total vasculature (p = 0.045) than patients with YNS or CRSsNP.ConclusionThis is the first histological analysis of sinus mucosa in patients with YNS. Vascular type, density, size, and distribution in the sinonasal mucosa of YNS patients are not statistically significantly different from those of the CRSsNP group. Lymphatic density and ratio to total vasculature is higher in CRSwNP patients.

Benign notochordal cell tumors (BNCTs) are considered to be benign intraosseous lesions of notochord origin; however, recent spine studies have suggested the possibility that some chordomas arise from BNCTs. Here, the authors describe two cases demonstrating histological features of BNCT and concomitant chordoma involving the clivus, which, to the best of the authors’ knowledge, have not been previously documented at this anatomical site.An 18-year-old female presented with an incidentally discovered clival mass. Magnetic resonance imaging revealed a 2.8-cm nonenhancing lesion in the upper clivus that was T2 hyperintense and T1 hypointense. She underwent an uneventful endoscopic transsphenoidal resection. Histologically, the tumor demonstrated areas of classic chordoma and a distinct intraosseous BNCT component. The patient completed adjuvant radiation therapy. Follow-up showed no recurrence at 18 months.A 39-year-old male presented with an incidentally discovered 2.8-cm clival lesion. The nonenhancing mass was T2 hyperintense and T1 hypointense. Surgical removal of the lesion was performed through an endoscopic transsphenoidal approach. Histological analysis revealed areas of BNCT with typical features of chordoma. Follow-up did not demonstrate recurrence at 4 years.These cases document histologically concomitant BNCT and chordoma involving the clivus, suggesting that the BNCT component may be a precursor of chordoma.

Background In the discipline of rhinology and endoscopic skull base surgery (ESBS), 3-dimensional (3D) printing has found meaningful application in areas including preoperative surgical planning as well as in surgical education. However, its scope of use may be limited due to the perception among surgeons that there exists a prohibitively high initial investment in resources and time to acquire the requisite technical expertise. Nevertheless, given the ever decreasing cost of advancing technology coupled with the need to understand the complex spatial relationships of the paranasal sinuses and skull base, the use of 3D printing in rhinology and ESBS is poised to blossom. Objective Help the reader identify current or potential future uses of 3D printing technology relevant to their rhinologic clinical or educational practice. Methods A review of published literature relating to 3D printing in rhinology and ESBS was performed. Results Results were reviewed and organized into 5 overarching categories including an overview of the 3D printing process as well as applications of 3D printing including (1) surgical planning, (2) custom prosthetics and implants, (3) patient education, and (4) surgical teaching and assessment. Conclusion In the discipline of rhinology and ESBS, 3D printing finds use in the areas of presurgical planning, patient education, prosthesis creation, and trainee education. As this technology moves forward, these products will be more broadly available to providers in the clinical and educational setting. The possible applications are vast and have great potential to positively impact surgical training, patient satisfaction, and most importantly, patient outcomes.

ObjectiveTo examine the impact of 2‐dimensional (2D) illustrations and 3‐dimensonal (3D)–printed anatomic models of the frontal sinuses according to the International Frontal Sinus Anatomy Classification in the education of otolaryngology and radiology residents.Study DesignA crossover study design with half of the study participants randomized to the 2D illustration intervention first and the other half randomized to the 3D‐printed model first.SettingRegularly scheduled resident didactic lectures at a tertiary care center.Subjects and MethodsForty‐one otolaryngology and radiology residents were assessed with pre‐ and postintervention questionnaires that included subjective and objective methods of assessment.ResultsOverall, there was a statistically significant improvement in total number of answers correct and in confidence score between the pre‐ and postintervention assessments (P <. 0001). The primary outcome of order of intervention (ie, 2D → 3D vs 3D → 2D) did not result in statistically significant differences in postevaluation scores. In regard to the secondary outcome of learner preference for educational modality, radiology residents favored the 2D illustrations to understand anatomic relationships, while otolaryngology residents preferred the 3D model to be more helpful in surgical planning (P =. 0075).ConclusionThere is no difference between 2D‐illustrated and 3D‐printed International Frontal Sinus Anatomy Classification anatomic models in overall educational outcome, despite the preference of learners. Together, these models can be used as helpful tools in frontal sinus education for otolaryngology and radiology trainees.

Objectives/HypothesisSinonasal mucosal melanoma (SNMM) is a rare malignant neoplasm. No study to date has analyzed the impact that nodal metastases and distant metastases at diagnosis have on overall survival (OS) and disease‐specific survival (DSS).Study DesignRetrospective database review.MethodsThe Surveillance, Epidemiology, and End Results database was queried for cases of SNMM. Descriptive statistics were used analyze patient demographic and clinicopathologic variables. The Kaplan‐Meier model was used to analyze survival, and the Cox proportional hazards model was used for multivariate analysis.ResultsThree hundred twenty‐eight cases of SNMM were identified. The most frequent sinonasal anatomic location was the nasal cavity in 279 (73.0%) patients, followed by the maxillary sinus in 60 (15.7%) patients. Regional nodal metastases at diagnosis occurred in 33 (8.6%) patients, whereas distant metastases at diagnosis occurred in 41 (10.7%) patients. Five‐year OS was 22.7%, and 5‐year DSS was 26.8%. The presence of positive nodes at diagnosis (OS P < .0001, DSS P < .0001), distant metastases at diagnosis (OS P = .0442, DSS P = .0442), primary tumor site (OS P < .0001, DSS P < .0001), and increasing age (OS P = .0012, DSS P = .0016) were found to be significant as negative predictors of OS and DSS.ConclusionsSNMM is a rare pathologic entity with a poor prognosis. The presence of nodal and distant metastases at diagnosis are negative prognostic factors in OS and DSS. These factors can be used in the development of new models of risk stratification and to inform treatment strategies and surveillance patterns.Level of EvidenceNALaryngoscope, 130:622–627, 2020

Objectives Determine the effect of endoscopic skull base surgery (ESBS) on long‐term olfactory outcomes after surgery. Methods An English‐language search was conducted using the Cochrane, MEDLINE, Scopus, and Embase databases from January 2000 to October 2017 for adult patients undergoing ESBS with subjective and objective olfaction outcomes. Two authors independently examined articles to identify those meeting inclusion criteria. Studies examining objective olfactory outcomes after ESBS were included in the meta‐analysis. A random‐effects meta‐analysis of patients undergoing sellar and parasellar ESBS was conducted to compare preoperative and postoperative olfactory outcomes using the University of Pennsylvania Smell Identification Test (UPSIT) and Cross‐Cultural Smell Identification Test (CCSIT). Results Among 339 eligible articles, 29 articles met inclusion criteria. Twenty‐five of these focused on sellar and parasellar tumors. Individual articles not meeting criteria for meta‐analysis were qualitatively reported. Meta‐analysis showed there was no difference in preoperative and postoperative olfactory function after sellar and parasellar ESBS based on the UPSIT (five studies, mean difference [MD] = −1.03; 95% CI: −3.98, 1.93; P = .50) and the CCIST (three studies, MD = −0.77; 95% CI: −3.03, 1.49; P = .50). A pooled overall meta‐analysis revealed similar results (eight studies, effect size = −0.30; 95% CI: −0.79, 0.18; P = .22). However, heterogeneity for all meta‐analyses was high ( I 2 > 95%, P < .01), suggesting significant variation in the included studies. Conclusions Based on published objective olfaction outcomes after sellar and parasellar ESBS, there was no significant difference between preoperative and postoperative olfaction. Further prospective studies using validated objective measures of olfaction are required to improve our understanding on this subject. Level of Evidence 2a Laryngoscope , 129:1998–2007, 2019