International Multicenter Study of Clinical Outcomes of Sinonasal Melanoma Shows Survival Benefit for Patients Treated with Immune Checkpoint Inhibitors and Potential Improvements to the Current TNM Staging System.

Objectives Sinonasal mucosal melanoma (SNMM) is an extremely rare and challenging sinonasal malignancy with a poor prognosis. Standard treatment involves complete surgical resection, but the role of adjuvant therapy remains unclear. Crucially, our understanding of its clinical presentation, course, and optimal treatment remains limited, and few advancements in improving its management have been made in the recent past. Methods We conducted an international multicenter retrospective analysis of 505 SNMM cases from 11 institutions across the United States, United Kingdom, Ireland, and continental Europe. Data on clinical presentation, diagnosis, treatment, and clinical outcomes were assessed. Results One-, three-, and five-year recurrence-free and overall survival were 61.4, 30.6, and 22.0%, and 77.6, 49.2, and 38.3%, respectively. Compared with disease confined to the nasal cavity, sinus involvement confers significantly worse survival; based on this, further stratifying the T3 stage was highly prognostic ( p < 0.001) with implications for a potential modification to the current TNM staging system. There was a statistically significant survival benefit for patients who received adjuvant radiotherapy, compared with those who underwent surgery alone (hazard ratio [HR] = 0.74, 95% confidence interval [CI]: 0.57-0.96, p = 0.021). Immune checkpoint blockade for the management of recurrent or persistent disease, with or without distant metastasis, conferred longer survival (HR = 0.50, 95% CI: 0.25-1.00, p = 0.036). Conclusions We present findings from the largest cohort of SNMM reported to date. We demonstrate the potential utility of further stratifying the T3 stage by sinus involvement and present promising data on the benefit of immune checkpoint inhibitors for recurrent, persistent, or metastatic disease with implications for future clinical trials in this field.

'Juvenile' nasal angiofibroma presenting in adulthood.

Juvenile nasopharyngeal angiofibromas (JNAs) are rare hyper vascular, benign tumours typically demonstrating a locally aggressive growth pattern. The cardinal presenting symptoms are unilateral nasal obstruction and recurrent, spontaneous epistaxis. Cases outside the adolescent male population are exceedingly rare and present a diagnostic challenge. We present the case of a man in his 30s referred to our tertiary skull base centre, presenting with left nasal obstruction. Examination showed left nasopharyngeal fullness without a discrete mass. Cross-sectional imaging detailed a 2.5×2.1×1.3 cm mass localised to his left sphenoid sinus with bony erosion. Due to the suspicion of malignancy, multidisciplinary consensus was to perform a diagnostic excisional biopsy and this revealed a JNA. He remains clinically well and asymptomatic following surgery. This case highlights the potential for subtle symptomatology in the presentation of these tumours and the challenge in diagnosing a JNA outside the adolescent male population.

Safe surgical training: evaluation of a national functional endoscopic sinus surgery model simulation course using the Kirkpatrick evaluation model.

BACKGROUND: Simulation is a rapidly developing field in modern undergraduate skills education and postgraduate surgical training. AIM: We aim to evaluate simulation training as a tool for higher surgical training in functional endoscopic sinus surgery (FESS) using the Kirkpatrick evaluation model. METHODS: This was a prospective cohort study in which a qualitative survey and multiple-choice questionnaire were distributed to otolaryngology trainees pre- and post-FESS training course using simulation models. Participants' reactions and interpretations of the models were assessed. Pre- and post-simulation knowledge and subjective skills were assessed. RESULTS: A total of 21 trainees completed the course. Trainees reported simulation models to be accurate representations of human anatomy 95% and easy to use 90%. There was an improvement in anatomical 54 to 62% (Z = 76, p0.03) and procedural 65 to 72% (Z = 87, p0.03) knowledge overall. CONCLUSION: Simulation training is an effective method of postgraduate education. This has been particularly useful following reduced operative exposure in the COVID-19 era.

Management of Temporal Bone Malignancy in Ireland.

Objectives Lateral temporal bone malignancy remains a challenging rare disease. We report 17 years of multidisciplinary care of these tumors with univariate and multivariate analyses of key prognostic indicators for consideration in contemporary oncological management. Design This is a retrospective cohort study. Setting This is set at a tertiary referral center. Participants All patients presenting with histopathologically newly diagnosed cases of temporal bone malignancy between 2000 and 2017 were included. Main Outcome Measures The main outcome measures are disease-specific and recurrence-free survival rates. Results In this study, 48 cases of temporal bone malignancy were diagnosed. Median age at diagnosis was 69 years (range: 5-88). Fourteen patients were female. Squamous cell carcinoma was the predominant malignancy in 34 cases (71%). Surgical treatment was undertaken in 37 patients. Mean length of follow-up was 32 months (range: 0.7-117). Overall 5-year disease-specific survival was 52.4%, while overall 5-year recurrence-free survival was 53.5%. On univariate analysis, significantly worse survival was seen in females ( p = 0.008), those with distant metastatic disease ( p = 0.041), and in middle ear involvement ( p = 0.012) with no difference for involvement of the external auditory canal ( p = 0.98) or mastoid ( p = 0.78). Only middle ear involvement remained significant on multivariate analysis. Conclusion A wide variety of malignant pathology may present in the temporal bone. Recurrence-free survival is equivalent to international data; however, this figure is low. This emphasizes the need to treat these tumors appropriately with radical resection, where possible, at first presentation. Therefore, multidisciplinary surgical input is recommended. Middle ear involvement was a negative prognosticator for disease-specific and recurrence-free survivals.

COVID-19 pandemic: Effects and evidence-based recommendations for otolaryngology and head and neck surgery practice.

The 2019 novel coronavirus disease (COVID-19) is a highly contagious zoonosis produced by SARS-CoV-2 that is spread human-to-human by respiratory secretions. It was declared by the WHO as a public health emergency. The most susceptible populations, needing mechanical ventilation, are the elderly and people with associated comorbidities. There is an important risk of contagion for anesthetists, dentists, head and neck surgeons, maxillofacial surgeons, ophthalmologists, and otolaryngologists. Health workers represent between 3.8% and 20% of the infected population; some 15% will develop severe complaints and among them, many will lose their lives. A large number of patients do not have overt signs and symptoms (fever/respiratory), yet pose a real risk to surgeons (who should know this fact and must therefore apply respiratory protective strategies for all patients they encounter). All interventions that have the potential to aerosolize aerodigestive secretions should be avoided or used only when mandatory. Health workers who are: pregnant, over 55 to 65 years of age, with a history of chronic diseases (uncontrolled hypertension, diabetes mellitus, chronic obstructive pulmonary diseases, and all clinical scenarios where immunosuppression is feasible, including that induced to treat chronic inflammatory conditions and organ transplants) should avoid the clinical attention of a potentially infected patient. Health care facilities should prioritize urgent and emergency visits and procedures until the present condition stabilizes; truly elective care should cease and discussed on a case-by-case basis for patients with cancer. For those who are working with COVID-19 infected patients' isolation is compulsory in the following settings: (a) unprotected close contact with COVID-19 pneumonia patients; (b) onset of fever, cough, shortness of breath, and other symptoms (gastrointestinal complaints, anosmia, and dysgeusia have been reported in a minority of cases). For any care or intervention in the upper aerodigestive tract region, irrespective of the setting and a confirmed diagnosis (eg, rhinoscopy or flexible laryngoscopy in the outpatient setting and tracheostomy or rigid endoscopy under anesthesia), it is strongly recommended that all health care personnel wear personal protective equipment such as N95, gown, cap, eye protection, and gloves. The procedures described are essential in trying to maintain safety of health care workers during COVID-19 pandemic. In particular, otolaryngologists, head and neck, and maxillofacial surgeons are per se exposed to the greatest risk of infection while caring for COVID-19 positive subjects, and their protection should be considered a priority in the present circumstances.

Changing Trends in the Management of Esthesioneuroblastoma: Irish and International Perspectives.

Objectives Evaluation of the changing trends in esthesioneuroblastoma in an Irish context and review of management options nationally to clarify the best current therapeutic approach by comparing with international research on this uncommon malignancy. Design Retrospective review. Setting Tertiary referral center. Participants All patients presenting with esthesioneuroblastoma in Beaumont hospital or on the National Cancer Registry of Ireland between 1994 and 2013. Main Outcome Measures Recurrence-free and overall survival. Results During the study period, 32 cases of esthesioneuroblastoma were diagnosed (0.4 per million per year). Average age at diagnosis was 57 years; however, two cases were under 20. The majority (62.5%) were male. Patients predominantly presented with epistaxis or nasal congestion (73%), while two cases were identified incidentally on radiological investigations. Twenty-seven cases underwent primary surgical management (two post neo-adjuvant treatment) with seventeen requiring bifrontal craniotomy. Twenty-four of these received postoperative radiation therapy. Overall, 5-year survival was 65%. Kadish A/B patients exhibited 100% 5-year disease-specific survival versus 54% in Kadish C/D ( p = 0.011). Hyams grade I/II patients exhibited 75% 5-year disease-specific survival versus 63% in Hyams grade III/IV ( p = 0.005). Patients treated endoscopically exhibited 100% 5-year disease-specific survival versus 51% in those treated via an open approach ( p = 0.102). Conclusions Many controversies exist in the diagnosis and management of this condition. Despite this, results from Irish data are mostly concordant with the international literature. The rising incidence of this disease may represent improved pathological recognition. An increasing number of esthesioneuroblastoma cases are being successfully treated via endoscopic surgery.

Evaluation of the impact of addition of PET to CT and MR scanning in the staging of patients with head and neck carcinomas.

The addition of whole body positron emission tomography (PET) to the investigation of patients with newly diagnosed head and neck squamous cell carcinoma (HNSCC) was assessed over a 6-month period. Staging investigations included laryngoscopy, oesophagoscopy, CXR, CT and MRI. In addition, all patients had an extended-field (whole body) FDG-PET scan and were restaged. Standardised Uptake Values (SUV) were used to measure FDG uptake. SUV levels above 5 were considered indicative of the presence of tumour, values below 3 indicative of benign aetiology and values equal to and between 3 and 5 were considered equivocal. Forty-eight consecutive patients with biopsy proven HNSCC were included for study. Three patients presenting with neck disease had unknown primary tumours. Of the remaining 45 patients, CT scan correctly identified 40 of the primary tumours (89%). MRI and PET both identified 41 primary tumours (91%). Thirty-two patients underwent neck dissection. Of these patients 12 had pathologically N0 necks and 20 had positive nodal disease. CT scan and MRI each correctly staged pN0 necks in 10 of 12 patients (83%) whereas PET alone had a lower true negative rate of 8 out of 12 patients (67%). PET correctly staged the N+ necks in 14/20 patients (70%) versus 12/20 (60%) for MRI, and 8/20 (40%) for CT alone. All four patients who were judged to have distant metastases by PET had these metastases deemed negative by other investigation. None of the three imaging modalities was able to identify the tumour site in the three patients with unknown primaries. In conclusion, although PET has got a higher sensitivity in detecting nodal disease, it has only slightly improved the classification of N+ necks. The findings of this study cast doubt on the merit of routine addition of PET to the current investigative protocols for HNSCC patients.

Validation of the Composite Laryngeal Recurrence Staging System.

BACKGROUND: The Composite Laryngeal Recurrence Staging System (CLRSS) has been described recently as an improved alternative to the retreatment TNM system (rTNM) for staging patients with recurrent laryngeal squamous cell carcinoma. The objectives of this study were to validate the CLRSS and compare its use with the rTNM system. METHODS: A retrospective chart review was conducted of 67 patients with recurrent laryngeal squamous cell carcinoma who had their initial treatment between 1980 and 1992. The external validity of the CLRSS was assessed, and its ability to stage patients and to predict survival was compared with the rTNM system. RESULTS: The overall 2-year survival rate was 51% (34 of 67 patients). The rTNM system was unable to stage 12 patients (18%), whereas all patients could be staged using the CLRSS. The CLRSS predicted survival better than the rTNM system (c-statistic = 0.79). CONCLUSIONS: The newer CLRSS staging system for recurrent laryngeal carcinoma outperformed the rTNM system in its ability to stage more patients and to predict survival.

Development of a new head and neck cancer-specific comorbidity index.

BACKGROUND: Most patients with head and neck squamous cell carcinoma are older and may have coexistent or comorbid diseases. OBJECTIVES: To determine the prognostic impact of individual comorbid conditions in patients with head and neck cancer, to combine the individual comorbid conditions to form a new a head and neck-specific comorbidity instrument, and to compare it with the Modified Kaplan-Feinstein Index to determine if the new disease-specific instrument offers any improvement in survival prediction over a general comorbidity index. DESIGN: Retrospective review of medical records. POPULATION: The study population comprised 1153 patients with biopsy-proven, newly diagnosed squamous cell carcinoma of the oral cavity, oropharynx, or larynx. RESULTS: Seven comorbid conditions (congestive heart disease, cardiac arrhythmia, peripheral vascular disease, pulmonary disease, renal disease, cancer controlled, and cancer uncontrolled) were significantly related to survival. These comorbid conditions were assigned integer weights to reflect their relative prognostic importance and combined to create the new Washington University Head and Neck Comorbidity Index (WUHNCI). Survival was significantly related to levels of comorbidity severity as defined by the WUHNCI. The WUHNCI predicted survival better than the Modified Kaplan-Feinstein Index despite containing far fewer ailments. CONCLUSIONS: Comorbidity is an important feature of the patient with head and neck cancer. The WUHNCI can be used for retrospective review or prospective outcomes research.