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Objective  This study aimed to describe the impact of adverse clinical and pathologic features in sinonasal squamous cell carcinoma (SCC). Design  This study is designed with retrospective chart review. Setting  The present study is conducted at a tertiary care institution. Participants  All patients treated surgically for sinonasal SCC at our tertiary care institution between January 2006 and December 2013. Main Outcome Measures  Overall survival (OS) and disease free survival (DFS) are the final measurement of this study. Results  Forty-eight patients were identified. Mean age at surgery was 65.8 years, and mean follow-up time was 40.7 months. Eighteen patients (38%) had T1-T3 disease, while 30 patients (63%) had T4 disease. Seven patients (8.3%) had nodal disease at presentation. At 2, 5, and 10 years, OS was 71, 54, and 48%, respectively, while DFS was 64, 51, and 45%, respectively. Twelve patients (25%) experienced local recurrences with mean time to recurrence of 15.3 months. Twenty-five patients (52%) had positive margins, 24 (50%) had high-grade tumors, 18 (38%) had perineural invasion (PNI), and 15 (31%) had lymphovascular invasion (LVI). In the univariate analysis, T4 disease (risk ratio [RR] = 2.7) and high grade (RR = 2.4) had a significant association with DFS. In the multivariate analysis, high grade (RR = 4.0 and 4.5) and LVI (RR = 4.1 and 4.7) had a significant association with OS and DFS. Conclusion  Our single-institution experience of 48 patients suggests that high grade and LVI are independently associated with survival outcomes in sinonasal SCC, while PNI and microscopically positive margins do not have a significant impact., Competing Interests: Conflict of Interest None declared., (Thieme. All rights reserved.)

Objectives  This article examines a national cohort of patients with nasopharyngeal adenoid cystic carcinoma (ACC) for incidence, skull base invasion, overall survival, and treatment paradigms. Design, Setting, and Participants  Retrospective national population-based study using Surveillance, Epidemiology, and End Results program data of patients with ACC of the nasopharynx (NACC) and skull base between 2004 and 2016. Main Outcomes and Measures  Primary outcomes included 5-year overall survival and odds of radiation treatment. Statistical analysis was performed using STATA 15.0 (STATACorp). p -Values < 0.05 were considered statistically significant. Results  Of the 2,385 cases of ACC, 70 cases were classified as NACC. Twenty-one percent (15) involved invasion of the skull base or posterior pharyngeal wall, and 42% (30) were either stage 3 or stage 4. The 5-year overall survival for patients with NACC without skull base invasion was 67% which dropped to 40% with invasion into the skull base. Radiation was used as the primary form of therapy for 62% of NACC and 73% of NACC invading into skull base. Odds of receiving radiation therapy and 5-year survival were not affected by socioeconomic status or density of providers. Conclusion  NACC is rare in incidence and was most commonly treated with radiation therapy when advanced in stage. Prognosis was dependent on invasion through posterior pharyngeal wall and skull base. Provider density and socioeconomic status did not affect odds of radiation or overall survival for NACC., Competing Interests: Conflict of Interest None declared., (Thieme. All rights reserved.)

Objectives  The main purpose of this article is to examine a single-center cohort of patients with nasopharyngeal adenoid cystic carcinoma (ACC) for pathologic features, skull base invasion, overall survival, and disease-free survival, with a focus on response to proton beam radiation therapy. Design, Setting, and Participants  Single-center institutional cancer registry was used to retrospectively identify and analyze outcomes for 12 patients treated for ACC of the nasopharynx from 2000 to 2016. Main Outcomes and Measures  Primary outcomes included 5-year overall survival and locoregional control. Statistical analysis was performed using STATA 12.0 (STATACorp, College Station, Texas, United States). Spearman's rank order correlation was used for ordinal, monotonic variables with p -values <0.05 considered statistically significant. Survival analysis was performed by Kaplan-Meier method; comparison between groups was performed using log-rank test. Results  Twelve patients with ACC of the nasopharynx were included. All patients presented with advanced disease and were treated with primary radiation therapy, typically proton beam therapy. Only two underwent a surgical attempt at resection. A majority of cases had a cribriform growth pattern. The 5-year survival was 75% and rate of locoregional control rate at 5 years was 50%, comparable to other ACC cohort studies that included earlier stage tumors in various subsites that were surgically resected. Conclusions  Although ACC is traditionally noted to be radioresistant, ACC of the nasopharynx was responsive to radiotherapy in our cohort, despite advanced stage and skull base invasion. Reasons for this improved survival are unclear and suggest the need for further pathologic and genetic characterization of nasopharyngeal ACC., Competing Interests: Conflict of Interest None., (© Thieme Medical Publishers.)

Objective  To investigate the incidence and degree of contralateral sinus disease following treatment of sinonasal malignancy (SNM) using radiological findings as an outcome measure. Study Design  Retrospective case series. Setting  Tertiary referral academic center. Participants  Patients with SNM treated with surgical and adjuvant radiotherapy with or without concurrent chemotherapy. Main Outcome Measures  Pre- and posttreatment computed tomography and magnetic resonance imaging scans, Lund-Mackay (LM) score, date of diagnosis, chemotherapy, and dosage of radiation therapy (RT). Results  There was a significant difference between pre- and posttreatment LM scores ( p  < 0.001) of the contralateral paranasal sinuses. There was no statistical significance between the change in pre- and posttreatment LM scores and patient age, gender, chemotherapy, RT dosage, and time lapse between diagnosis and when we performed this study in the univariate and the multivariable analyses. Conclusions  Following treatment of SNM with surgical resection and RT, there was a significant increase in incidence and degree of mucosal thickening of the contralateral sinus by LM grading that persisted after the completion of treatment. This indicates the need for long-term follow-up to monitor for chronic rhinosinusitis as a delayed complication following treatment of SNM.

Objectives  Review outcomes of lateral temporal bone resections for ear and temporal bone malignancy. Design, Setting, and Participants  Retrospective review of all lateral temporal bone resections performed from 2008 to 2015 at a single tertiary care center. Main Outcome Measures  Patient demographics, perioperative variables, overall survival, disease-free survival (DFS), and comparison of Kaplan-Meier curves. Results  Overall, 56 patients were identified with a mean follow-up period of 2.3 ± 1.8 years. The predominant histopathologic diagnosis was squamous cell carcinoma (SCC, 54%), followed by salivary gland tumors (18%), and basal cell carcinoma (9%). Tumor stages were T1-T2 in 23%, T3-T4 in 73%, and two unknown primary lesions. Mean overall survival was 4.6 ± 0.4 years. Comparison of tumors with and without lymph node involvement or perineural invasion approached statistical significance for overall survival ( p  = 0.07 and 0.06, respectively). DFS was 2.5 ± 0.3 years. Stratification by lymph node status had a statistically significant difference in DFS ( p  = 0.03). Subgroup analysis of SCC patients did not reveal significant differences. Conclusions  Based on our cohort, most patients with temporal bone malignancies present with advanced disease, making it difficult to achieve negative margins. Overall, lymph node status was the strongest predictor of survival in this group.

Objective: To collect data on skull base surgery training experiences and practice patterns of otolaryngologists that recently completed fellowship training., Design: A 24-item survey was disseminated to physicians who completed otolaryngology fellowships in rhinology, head and neck oncology, or neurotology between 2010 and 2014., Results: During a typical year, 50% of rhinologists performed more than 20 endoscopic anterior skull base cases, 83% performed fewer than 20 open cases, and were more confident performing advanced transplanum (p = 0.02) and transclival (p = 0.03) endoscopic approaches than head and neck surgeons. Head and neck surgeons performed fewer than 20 endoscopic and fewer than 20 open cases in 100% of respondents and were more confident with open approaches than rhinologists (p = 0.02). Neurotologists performed more than 20 lateral skull base cases in 45% of respondents during a typical year, fewer than 20 endoscopic ear cases in 95%, and were very comfortable performing lateral skull base approaches., Conclusion: Many recent otolaryngology fellowship graduates are integrating skull base surgery into their practices. Respondents reported high confidence levels performing a range of cranial base approaches. Exposure to endoscopic ear techniques is minimal in neurotology training, and rhinology training appears to offer increased exposure to skull base surgery compared with head and neck training.

Objective: To characterize our experience with the surgical management of anterior skull base malignancy, and to propose an algorithm for surgical approach to anterior skull base malignancies., Design: Retrospective review., Setting: Academic cranial base center., Participants: Sixty-seven patients who underwent resection of paranasal sinus or anterior skull base malignancy with an endoscopic, cranioendoscopic, or traditional anterior craniofacial approach., Outcome Measures: Complications, recurrence, and survival., Results: There were 48 males and 19 females, ranging from 6 to 88 years old. There were three groups: endonasal endoscopic resection (n = 10), cranioendoscopic resection (n = 12), and traditional craniofacial resection (n = 45). The most common tumor pathologies included esthesioneuroblastoma, squamous cell carcinoma, and sinonasal undifferentiated carcinoma. Patients with T3/T4 disease were less likely to undergo endoscopic resection (p = 0.007). The 5-year disease-free survival was 82.1% overall, with no statistically significant differences among approaches. There were no differences in orbital complications, meningitis, or cerebrospinal fluid leak. Use of a transfacial incisions predisposed patients to surgical site infection and sinocutaneous fistulae. An algorithm for surgical approach was developed based on these results., Conclusion: Sinonasal and skull base malignancies can be safely surgically addressed via several approaches. Surgical approach should be selected algorithmically based on preoperative clinical assessment of the tumor and known postoperative complication rates.

Objectives To characterize the temporal distribution and resolution rate of postoperative complications from endoscopic skull base surgery. Design Retrospective review of patients undergoing endoscopic resection of paranasal sinus or skull base neoplasm from 2007 to 2013. Setting Massachusetts General Hospital/Massachusetts Eye and Ear Infirmary Cranial Base Center. Participants Fifty-eight consecutive patients. Main Outcome Measures Postoperative complications were categorized as cerebrospinal fluid (CSF) leak, pituitary, orbital, intracranial, or sinonasal. Complications were temporally categorized as "perioperative" (within 1 week), "early" (after 1 week and within 6 months), or "delayed" (after 6 months). Results The most common perioperative complications were diabetes insipidus (19.0%), CSF leak (5.2%), and meningitis (5.2%), with resolution rates of 75%, 100%, and 100%, respectively. Overall, CSF leak occurred in 13.8% of patients and resolved in all cases. A total of 53.8% of all complications were evident within 1 week of surgery. Chronic rhinosinusitis was the most common delayed complication (3.4%). Hypopituitarism and delayed complications were less likely to resolve (p = 0.014 and p = 0.080, respectively). Conclusions Monitoring of complications after endoscopic skull base surgery should focus on neurologic complications and CSF leak in the early postoperative period and development of chronic rhinosinusitis in the long term. Late-onset complications and hypopituitarism are less likely to resolve.

Introduction The radial forearm flap has fallen out of favor in lateral skull base reconstruction in recent literature. However, especially when used in a double layer, a radial forearm may be able to provide the thickness of a large flap while taking advantage of the pliability for which the flap is renowned. Objective To report the results of the double-layer technique of radial forearm free flap reconstruction of lateral temporal bone defects. Design A retrospective chart review. Setting A tertiary care institution. Participants All consecutive patients who underwent lateral temporal bone resections and were reconstructed with free flaps from 2006 to 2012. Major Outcome Measures Flap success rate, complications, and rate of revision surgery. Results A total of 17 patients were identified with free flap reconstruction of the lateral skull base. Seven received reconstruction with a double-layer radial forearm flap. Reconstruction-related complications in this group included one case of facial cellulitis. The flap success rate was 100%. These results were comparable with patients who had other flaps. Conclusions The radial forearm free flap may be an effective reconstruction option for lateral temporal bone defects especially when used in the double-layer technique.

Objective To determine trends in sinonasal undifferentiated carcinoma (SNUC) survival patterns in the United States. Design Retrospective review of national database. Participants All cases of SNUC in the National Cancer Institute's Surveillance Epidemiology and End Results program from 1973 to 2010 were examined. Main Outcome Measures Age-adjusted incidence and survival rates were calculated and stratified by demographic information and treatment modality. Cohort analysis was performed to analyze survival patterns over time. Results A total of 318 SNUC cases were identified. Age-adjusted incidence rate (IR) was 0.02 per 100,000. Incidence was greater in males (IR: 0.03) than females (IR: 0.01; p = 0.03). Overall 5- and 10-year relative survival rate was 34.9% and 31.3%, respectively. Overall median survival was 22.1 months. Median survival following surgery combined with radiation was 41.9 months. Five-year relative survival rate following surgery, radiation, or surgery combined with radiation was 38.7%, 36.0%, and 39.1%, respectively. Median survival from 1973-1986 and 1987-2010 was 14.5 and 23.5 months, respectively. Conclusions This study provides new data regarding survival patterns of SNUC in the United States, confirming survival benefit with surgery and radiation as well as identifying a trend toward improved survival in recent decades.

Objective To report complications occurring at least 6 months after completion of treatment for patients with anterior skull base malignancy undergoing anterior craniofacial resection (CFR). Design Retrospective review of medical records of all patients undergoing traditional CFR for treatment of anterior skull base malignancy from 2002 through 2011. Setting Massachusetts General Hospital/Massachusetts Eye and Ear Infirmary Cranial Base Center. Participants Thirty-one consecutive patients who had at least 18 months of follow-up for analysis were reviewed. All patients underwent traditional CFR. A total of 28 patients received postoperative proton beam radiation therapy. Eleven patients received adjuvant chemotherapy. Main Outcome Measures A delayed complication was any complication occurring at least 6 months after the completion of treatment. Results Seventeen patients had delayed complications. Orbital complications were the most common type (13 patients) followed by issues with wound healing (6 patients). The most common orbital complication was epiphora (7 patients). The most common wound complication was a nasocutaneous fistula (5 patients). Conclusions Patients with anterior skull malignancy can develop complications months to years after the completion of treatment. Therefore, it is important to continue to follow and report complications for several years when deciding on the optimal approach for treatment of these patients.

Objectives To update the Massachusetts General Hospital (MGH) and Massachusetts Eye and Ear Infirmary (MEEI) experience in the management of esthesioneuroblastoma (ENB) with multimodality therapy and to reassess treatment outcomes and complications in a larger cohort with longer follow-up times. Design A retrospective chart review. Setting A tertiary referral center. Participants All patients presenting with ENB and managed at the MGH and MEEI from 1997 to 2013. Main Outcome Measures Disease-free and overall survival. Results Twenty-two patients were identified with an average follow-up of 73 months. Ten patients presented with Kadish stage B disease and 12 with stage C disease. A total of six patients (27%) developed regional metastases. Treatment for all patients included craniofacial resection (CFR) followed by proton beam irradiation with or without chemotherapy. The 5-year disease-free and overall survival rates were 86.4% and 95.2%, respectively, by Kaplan-Meier analysis. Negative margins were a significant factor in disease-free survival. One patient experienced severe late-radiation toxicity. Conclusions ENB is safely and effectively treated with CFR followed by proton beam irradiation. The high incidence of regional metastases warrants strong consideration for elective neck irradiation. Proton beam radiation is associated with lower rates of severe late-radiation toxicity than conventional radiotherapy.

Objectives To describe the presentation, work-up, and management of patients with metastatic renal cell carcinoma (RCC) to the sinonasal cavity and skull base, and to describe our current treatment algorithm of endoscopic surgical resection followed by radiation therapy. Design Retrospective review of two recent cases from our institution over a 1-year period, with a relevant review of the literature. Setting A large regional tertiary care facility. Participants Consecutive cases of RCC with metastases to the sinonasal cavity presenting to our institution. Main Outcome Measures Preoperative and postoperative sinonasal outcome test (SNOT)-22 scores, duration of hospital stay, complications, and local disease control Results Patients in this series underwent preoperative embolization followed by endoscopic resection without complication. Postoperatively they were treated with radiation therapy. They experienced improvement in their SNOT-22 scores and are currently free of local disease. Conclusion Metastatic RCC to the sinonasal cavity can be safely treated with preoperative embolization followed by endoscopic surgical resection and radiation therapy, which can result in improvement in sinonasal quality of life and is a potential adjunct for local control of disease.

Objectives Surgical resection in addition to adjuvant radiation with or without chemotherapy is the mainstay of treatment for esthesioneuroblastoma (ENB). However, management of patients with orbital involvement remains controversial. Historically, orbital exenteration has been advocated when there is evidence of periorbital invasion. Recently, the indications for orbital exenteration have become more selective and orbital preservation has been advocated. We report our experience with anterior craniofacial resection and orbital preservation in patients with ENB. Design Retrospective review of all patients diagnosed with esthesioneuroblastoma who underwent traditional open anterior craniofacial resection at the Massachusetts General Hospital/Massachusetts Eye and Ear Infirmary Cranial Base Center from 1997 to 2008. Results Sixteen patients were identified with a mean follow-up of 76 months. All patients underwent anterior craniofacial resection via an open approach and adjuvant proton beam radiation. Six of the 16 patients had evidence of either periorbital or lacrimal sac involvement at the time of surgery. All of these patients underwent periorbital resection to negative histologic margins with preservation of the orbit. Conclusion In our study, patients with ENB and periorbital invasion-who were treated with anterior craniofacial resection and periorbital resection with orbital preservation-had no evidence of decreased survival. In all patients, negative histologic margins of the periorbital resection were achieved.