INI1-Intact Sinonasal Carcinoma with Rhabdoid Features

Sinonasal malignancies are known for their associated poor prognosis and diversity of histologic features. While poor prognosis is largely due to advanced disease at presentation, histologic features also play a significant role. Therefore, accurate pathologic diagnosis is of utmost importance. Here, we describe a 63-year-old male with chronic left-sided nasal obstruction and left-sided epistaxis who was found to have a large mass occupying most of the nasal cavity extending through the nasopharynx to just below the nasopharyngeal surface of the soft palate. During surgical excision, the mass was noted to originate from the floor of the maxillary sinus with erosion of the medial wall of the maxillary sinus. Pathology revealed a diagnosis of INI1-intact poorly differentiated composite carcinoma with rhabdoid phenotype and sarcomatoid and squamous cell carcinoma foci arising within an inverted papilloma. Included in this report is a detailed description of both the patient’s medical course and this pathologically novel sinonasal neoplasm. We aim to elucidate this rare tumor’s complex features in order to improve future diagnosis and stimulate prospective research on sinonasal malignancies with complex histology.

Augmented Reality and Intraoperative Navigation in Sinonasal Malignancies: A Preclinical Study

ObjectiveTo report the first use of a novel projected augmented reality (AR) system in open sinonasal tumor resections in preclinical models and to compare the AR approach with an advanced intraoperative navigation (IN) system.MethodsFour tumor models were created. Five head and neck surgeons participated in the study performing virtual osteotomies. Unguided, AR, IN, and AR + IN simulations were performed. Statistical comparisons between approaches were obtained. Intratumoral cut rate was the main outcome. The groups were also compared in terms of percentage of intratumoral, close, adequate, and excessive distances from the tumor. Information on a wearable gaze tracker headset and NASA Task Load Index questionnaire results were analyzed as well.ResultsA total of 335 cuts were simulated. Intratumoral cuts were observed in 20.7%, 9.4%, 1.2,% and 0% of the unguided, AR, IN, and AR + IN simulations, respectively (p < 0.0001). The AR was superior than the unguided approach in univariate and multivariate models. The percentage of time looking at the screen during the procedures was 55.5% for the unguided approaches and 0%, 78.5%, and 61.8% in AR, IN, and AR + IN, respectively (p < 0.001). The combined approach significantly reduced the screen time compared with the IN procedure alone.ConclusionWe reported the use of a novel AR system for oncological resections in open sinonasal approaches, with improved margin delineation compared with unguided techniques. AR improved the gaze-toggling drawback of IN. Further refinements of the AR system are needed before translating our experience to clinical practice.

Nasopharyngeal mucoepidermoid carcinoma: a case report and review of its current management

<p class="abstract">Salivary gland tumours arising at the nasopharynx is highly infrequent. Among them the commonest is mucoepidermoid carcinoma (MEC). Reports with a larger number of patients are often from Asian countries where nasopharyngeal squamous cell carcinoma is also predominant. Although nasopharyngeal MEC (NMEC) is a disease of adults, sporadic cases amongst children have been reported. We report a case of a 32 years old man presented with complaining of intermittent epistaxis over a year. His nasal endoscopy showed friable polypoid tumour at the right choanae. Histopathology revealed a NMEC and he underwent endoscopic endonasal nasopharyngectomy with adjuvant radiotherapy. The optimal treatment for NMEC is arbitrary due to the lack of evidence. However, unlike most sinonasal malignancies, NMEC has the tendency to manifest itself early and has good response to the treatment. Therefore, in this article we describe the clinical features and justifications for the selection of treatment options including surgical and non-surgical therapies and including the role of neck dissection.</p>

Long-term Outcomes of Endoscopic Resection Versus Open Surgery for Locally Advanced Sinonasal Malignancies in Combination With Radiotherapy

Objective:To compare the long-term oncological outcomes of endoscopic resection versus open surgery in combination with radiotherapy for locally advanced sinonasal malignancies.Methods:Data for continuous patients with sinonasal epithelial tumors treated in our center between Jan 1999 and Dec 2016 were retrospectively reviewed. Those who received surgery (endoscopic or open surgery) combined with radiotherapy were identified, and 1:1 matching with propensity scores was performed. The primary endpoints of overall survival (OS) and progression-free survival (PFS) were evaluated using the Kaplan-Meier method and Cox proportional hazards modeling. The local recurrence rate (LRR) was assessed by competing risk analysis.Results:We identified 267 eligible patients, 90 of whom were included after matching: 45 patients in the endoscopy group and 45 in the open group. The median follow-up time was 87 months. In the endoscopic group, 84.4% of patients received intensity-modulated radiotherapy (IMRT), with a mean gross tumor volume (GTV) dose of 68.28 Gy; in the open surgery group, 64.4% of patients received IMRT, with a mean GTV dose of 64 Gy. The 5-year OS, PFS and LRR were 69.9%, 58.6%, and 24.5% in the endoscopic group and 64.6%, 54.4%, and 31.8% in the open surgery group, respectively. Multivariable regression analysis revealed that surgical approach was not associated with lower OS, PFS or LRR. Age, histopathology and stage were independent risk factors for OS.Conclusion:For patients with locally advanced sinonasal carcinoma, minimally invasive endoscopic resection, in combination with a higher radiation dose and new radiation techniques such as IMRT, yields survival outcomes similar to those of open surgery in combination with radiotherapy.

The neurosurgical management of sinonasal malignancies involving the anterior skull base: a 28-year experience at The MD Anderson Cancer Center

OBJECTIVE Sinonasal malignancies that extend to the anterior skull base frequently require neurosurgical intervention. The development of techniques for craniofacial resection revolutionized the management of these neoplasms, but modern and long-term data are lacking, particularly those related to the incorporation of endoscopic techniques and novel adjuvant chemotherapeutics into management schema. The present study was performed to better define the utility of surgical management and to determine factors related to outcome. METHODS Patients who underwent surgery between 1993 and 2020 were included in this retrospective cohort study. Only patients with greater than 6 months of clinical and radiological follow-up were included. Outcome measures included progression, survival, and treatment-related complications. RESULTS Two hundred twenty-five patients were included. The mean clinical follow-up was 6.5 years. The most common histological diagnosis was olfactory neuroblastoma (33%). Overall, metastatic disease and brain invasion were present in 8% and 19% of patients, respectively, at the time of surgery. A lumbar drain was used in 54% of patients. When stratified by decade, higher-stage disease at surgery became more frequent over time (15% of patients had metastatic disease in the 3rd decade of the study period vs 4% in the 1st decade). Despite the inclusion of patients with progressively higher-stage disease, median overall survival (OS) remained stable in each decade at approximately 10 years (p = 0.16). OS was significantly worse in patients with brain invasion (p = 0.006) or metastasis at the time of surgery (p = 0.014). Complications occurred after 28% of operations, but typically resulted in no long-term negative sequelae. Use of a lumbar drain was a significant predictor of complications (p = 0.02). Permanent ophthalmological disabilities were observed after 4% of surgical procedures. One patient died during the perioperative period. Finally, major complications (Clavien-Dindo grade ≥ IIIb) decreased from 27% of patients in the 1st decade to 10% in the 3rd decade (p = 0.007). CONCLUSIONS The surgical management of sinonasal malignancies with anterior skull base involvement is effective and generally safe. Surgical management, however, is only one facet of the overall multimodal management paradigms created to optimize patient outcomes. Survival outcomes have remained stable despite more extensive disease at surgery in patients who have presented in recent decades. The safety of such surgery has improved over time owing to the incorporation of endoscopic surgical techniques and the avoidance of lumbar spinal drainage with open resection.

Outcome for sinonasal malignancies: a population-based survey

Purpose Sinonasal malignancies (SNM) represent a rare and complex group of cancers that includes a wide range of histopathological subtypes. Data from population-based cohorts are scarce but warranted as a basis for randomized controlled treatment trials (RCTs). Our aim was to assess overall and histology subset-specific outcomes for SNM patients treated at a tertiary referral centre. Methods A retrospective, population-based, consecutive cohort of patients with SNMs diagnosed from 2001 through 2019 was examined. Outcome was analysed in relation to age, gender, site, stage, histopathology, and treatment. Results Two-hundred and twenty-six patients were identified, whereof 61% presented with stage IV disease. 80% completed treatment with curative intent, which comprised surgery with neoadjuvant (29%) or adjuvant (37%) radiotherapy, monotherapy with surgery (22%), definitive chemoradiotherapy (7%), or radiotherapy (5%). Median follow-up was 106 months. The 5- and 10-year overall survival rates were 57% and 35%, respectively. Median overall survival was 76 months (esthesioneuroblastoma: 147 months; adenocarcinoma: 117; salivary carcinoma: 88; mucosal melanoma: 69; squamous cell carcinoma: 51, undifferentiated carcinoma: 42; neuroendocrine carcinoma: 9; and NUT-carcinoma 5). The 5- and 10-year disease-free survival rates were 63% and 54%, respectively, and disease-specific survival 83% and 66%. Increasing age, stage IVB, melanoma histopathology, and treatment with definitive chemoradiotherapy emerged as significant independent prognostic risk factors for disease-specific mortality (p ≤ 0.001). Conclusion The results indicate a seemingly good outcome in comparison to previous reports, particularly for mucosal melanoma, adenocarcinoma, and undifferentiated carcinoma. The study provides additional background for future RCTs focusing on histology subset-specific treatment for SNM.

RECONSTRUCTION OF LARGE ANTERIOR SKULL BASE DEFECTS AFTER RESECTION OF SINONASAL TUMORS WITH INTRACRANIAL EXTENSION BY USING PEDICLED DOUBLE FLAP TECHNIQUES

Background: Surgical resection of a large anterior skull base (ASB) tumor and sinonasal maglinancies with intracranial extension will result a large skull base defect. Reconstruction of large ASB defects by using traditional techniques may result in high risk of postoperative CSF leakage, meningitis and increase mortality rate. The use of pedicled double flap technique to reconstruct the anterior skull base defect may decrease the complications. In this study, we examine the clinical outcomes of patients who underwent this double flap reconstruction technique after the resection of sinonasal malignancies with significant intracranial extension at Cho Ray hospital, Vietnam. Methods: Case series study was conducted at Cho Ray hospital from 09/2010 to 09/2020. All patients with large sinonasal malignancies with intracranial invasion underwent combined transbasal - EEA approach. Reconstruction of large skull base defect ( > 2 cm ) was followed by using the pedicled double flap technique. Results: There were 75 patients who underwent the modified multi-layer with double flap reconstruction technique after the resection of ASB tumor from 09/2010 to 09/2020. The skull base defects were commonly seen at the horizontal plate of the ethmoid and the roof of the ethmoid ( 98.6%). The large skull base defects ( > 2cm) accounted for 81.3%. The risk of postoperative CSF leakage after double flap repair was very low. In this study, we had 1 patient with postoperative CSF leakage and 1 patient had postoperative meningitis. Conclusion: The use of two vascularize pedicled flap may decrease the incidence of postoperative cerebral spinal fluid (CSF) leakage and meningitis. This technique is an effective method for the reconstruction of the ASB with large defect.