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Background: Past research established that surgery plus adjuvant radiotherapy (S + AR) improves overall survival (OS) in esthesioneuroblastoma (ENB). However, it is unknown if the addition of adjuvant chemotherapy (AC) further improves survival. The primary objective of this study was to compare survival among patients treated with S + AR alone to patients who underwent S + AR + AC., Methods: Retrospective review of patient records., Results: Thirty-eight patients met inclusion criteria for either S + AR or S + AR + AC treatment groups. The S + AR + AC group contained more patients with Kadish stage D disease, dural invasion, and positive histologic margins postsurgery. All S + AR + AC patients received platinum-based regimens, combined with etoposide in 67%. OS and recurrence-free survival did not differ between the two groups, even when restricting the analysis to patients with Kadish stages B and C disease., Conclusion: Patients who received platinum-based AC did not exhibit improved survival compared to S + AR alone. Further investigation, preferably prospective, into the optimal use of systemic therapy in ENB is warranted., (© 2018 Wiley Periodicals, Inc.)

Background: The purpose of this study was to determine the clinical outcomes and review the management strategies for metastatic esthesioneuroblastoma., Methods: We conducted a systematic review and meta-analysis., Results: Forty-eight studies totaling 118 patients met inclusion criteria. Chemotherapy in combination with surgery and/or radiation exhibited the best overall survival when compared to monotherapy and no treatment (P < .001). However, most patients (66%) received either monotherapy or no therapy. The number and location of metastases among the 3 treatment groups did not significantly differ (P = .85). Treatment modality remained significantly associated with overall survival on multivariable analysis (P < .001). Platinum-based chemotherapy was most commonly utilized but did not provide a survival benefit when compared with all other regimens (P = .88)., Conclusion: Distant metastases with esthesioneuroblastoma portend a poor prognosis. Chemotherapy in combination with surgery and/or radiation was associated with improved overall survival. Further research into the optimal systemic therapeutic regimen for patients with distant metastases is critical., (© 2018 Wiley Periodicals, Inc.)

Background: Given the particularities of olfactory neuroblastoma (ONB) and the lack of studies on the subject, a multicenter collaborative study was conducted to assess treatment strategy., Methods: Fifty-three patients with ONB were included from the French Rare Head and Neck Cancer Expert Network (REFCOR) database: 16T1, 8T2, 19T3, and 10T4. All cases were treated endoscopically with skull base removal and repair in 26 cases (49%) and without external craniotomy., Results: The overall survival (OS) and disease-free survival (DFS) rates at 5 years were 87% and 71%, respectively, with mean follow-up of 45.4 ± 26.5 months. The complication rate was 18.8% with 4 cases of meningitis. Pathological analysis showed positive margins in 26.8%, notably on the dura-mater and periorbita, without impairment of OS or DFS. Forty-eight patients received adjuvant radiotherapy on T ± N. Ten patients had a recurrence (18.9%). Six patients died of their disease. Prophylactic neck irradiation seemed to reduce the recurrence rate., Conclusion: Exclusively endoscopic treatment proved efficient and reliable in a large controlled series., (© 2018 Wiley Periodicals, Inc.)

Background: Advanced-stage olfactory neuroblastoma requires multimodal therapy for optimal outcomes. Debate exists over endoscopic endonasal surgery in this situation. Stage-matched open and endoscopic surgical therapy were compared., Methods: Patients from 6 cancer institutions were assessed. Stratification included dural involvement, Kadish stage, nodal disease, Hyams' grade, approach, and margin status. At follow-up, local control, nodal status, and evidence of distant metastases were recorded with any subsequent therapy. Statistical analyses to identify risk factors for developing recurrence and survival differences were performed., Results: One hundred nine patients were assessed (age 49.2 ± 13.0 years; 46% women) representing Kadish A stage (10%), Kadish B stage (25%), and Kadish C stage (65%). The majority of the patients (61.5%) underwent endoscopic resection, 53.5% within Kadish C stage. Within-stage survival analysis favored endoscopic subgroup for Kadish C stage (log-rank P = .017) nonsignificant for Kadish B stage (log-rank P = .39)., Conclusion: Stage-matched survival was better for the endoscopically treated group compared to the open surgery group, with high negative margin resections obtained., (© 2017 Wiley Periodicals, Inc.)

Background: Given the rarity of esthesioneuroblastoma, it is difficult to validate a staging system. The purpose of this study was to investigate the utility of the Kadish staging system in esthesioneuroblastoma using the National Cancer Database (NCDB)., Methods: One thousand one hundred sixty-seven patients with esthesioneuroblastoma were identified from the NCDB., Results: Five-year survival was 80.0% for Kadish A, 87.7% for Kadish B, 77.0% for Kadish C, and 49.5% for Kadish D. Kadish B had higher survival than Kadish A. More Kadish B patients received surgery with adjuvant therapy than Kadish A patients (41.6% vs 32.5%; P = .0038) and also had more positive margins (21.6% vs 11.3%; P = .03). There was no difference in age distribution, sex, race, or neck dissection status between the 2 groups., Conclusion: Kadish B had greater survival than Kadish A, but the treatment characteristics could not account for this difference. The utility of early-stage Kadish staging is uncertain and requires further study., (© 2017 Wiley Periodicals, Inc.)

Background: Esthesioneuroblastoma is a rare cancer of the anterior cranial base that arises in the region of the olfactory rootlets. The purpose of this study was to review the long-term outcomes of patients diagnosed with esthesioneuroblastoma (ENB) treated at a single institution to determine factors associated with improved disease control and survival., Methods: A retrospective review of 70 patients with ENB treated at the University of Texas MD Anderson Cancer Center between 1992 and 2007 was undertaken. Survival and recurrence was analyzed and compared using the Kaplan-Meier method and log-rank statistics., Results: Seventy patients were reviewed. The majority (77%) had T3 or T4 disease at presentation, 38% identified as modified Kadish stage C or D. Ninety percent of patients received surgical resection as part of their treatment, and 66% received postoperative radiation or chemoradiation. The median follow-up was 91.4 months (7.6 years). Forty-eight percent of patients developed recurrent disease and the median time to recurrence was 6.9 years. Overall and disease-specific median survival was 10.5 and 11.6 years, respectively. Patients who were treated with surgery alone had a median disease-specific survival of 87.9 months, whereas those who were treated with surgery and postoperative radiation had a median disease-specific survival of 218.5 months (p = .047)., Conclusion: Patients with ENB can achieve favorable long-term survival, even if disease is locally advanced. Survival is improved considerably when surgical resection is followed by postoperative radiation. However, recurrence rates and mortality remain high, and therefore long-term observation in these patients is warranted., (Copyright © 2013 Wiley Periodicals, Inc.)

Background: Stereotactic body radiotherapy (SBRT) has emerged as a promising salvage strategy for patients with recurrent, previously irradiated head and neck cancer; however, data are limited predominantly to squamous cell carcinomas. Herein, we report the efficacy of SBRT in recurrent, nonsquamous cell cancers of the head and neck (NSCHNs)., Methods: In all, 34 patients with pathologically proven NSCHN were re-irradiated with SBRT to a median dose of 40 Gy in 5 fractions (interquartile range, 30-44 Gy). Toxicity and quality of life were followed prospectively., Results: Median follow-up was 10 months (absolute range, 0-55 months). The 6-month/1-year local control rate was 77/59%, with a 6-month/1-year overall survival of 76/59%. Local control was significantly improved for tumors <25 mL (p = .030). Acute/late grade 3 toxicity was 15/6%, with no grade 4-5 toxicity., Conclusions: SBRT for previously irradiated, locally recurrent NSCHN provides promising local control, especially for tumors <25 mL, with minimal toxicity. The optimal dose for larger tumors remains to be defined., (Copyright © 2011 Wiley Periodicals, Inc.)

Background: The present study uses population-based data from the Surveillance, Epidemiology, and End Results (SEER) database to evaluate the behavior of sinonasal tumors., Methods: Data for all sinonasal malignancies reported between 1973 and 2006 (n = 6739) were extracted from the database. Time-dependent trends in incidence and survival were analyzed by linear regression., Results: The overall incidence of sinonasal cancer was 0.556 cases per 100,000 population per year with a male:female ratio of 1.8:1. The most common histologies were squamous cell carcinoma (51.6%) and adenocarcinoma (12.6%), whereas the most common primary sites were the nasal cavity (43.9%) and maxillary sinus (35.9%). The incidence of sinonasal cancer remained relatively stable during the study period. No significant changes in overall relative survival were noted. The best relative survival was noted in patients treated with surgery or a combination of surgery and radiotherapy., Conclusions: The prognosis of patients with sinonasal cancer is generally poor, and has not changed substantially over the last 3 decades., (Copyright © 2011 Wiley Periodicals, Inc.)

Background: Olfactory neuroblastoma is a rare paranasal sinus malignancy. The traditional approach was craniofacial resection (CFR) and then postoperative radiotherapy until 1998. This review will chart development of a new protocol., Methods: This ethics-approved audit evaluated the number of new patients diagnosed with olfactory neuroblastoma, with information relating to patient, disease, and treatment factors noted., Results: There were 24 eligible patients, 16 men, 8 women, 7 Kadish stage B, 17 stage C. The planned treatment was: chemotherapy (cisplatin/etoposide) and determine treatment dependent on response in 6 patients, surgery and radiotherapy in 16 patients, and single-modality treatment only (surgery, radiotherapy 1) in 2 patients. Surgery to radiotherapy occurred in 17 patients. With salvage treatment ultimate local control was 79%., Conclusions: There was a higher local control in those patients who had surgery; abandoning this may carry a higher risk of local failure. The use of response to chemotherapy to determine local treatment remains experimental., (Copyright © 2010 Wiley Periodicals, Inc.)

Background: The purpose of our study was to assess the long-term results in the management of patients with esthesioneuroblastoma., Methods: A total of 28 patients were retrospectively reviewed to analyze their clinical features, treatment outcomes, pattern of failures, and course of the disease., Results: The patients were classified as 2 patients with T1, 10 patients with T2, 10 patients with T3, and 6 patients with T4; the results of all were included in this study. Twenty-six patients underwent surgery and 23 had postoperative radiotherapy over the tumor bed, only 9 had radiotherapy over the nodes. The average follow-up was 99.11 months. Fifteen-year and 20-year disease-specific and disease-free survival rates were 88.6% to 66.4% and 59.6% to 29.8%, respectively. Six patients had recurrences and 60% of them occurred more than 10 years after treatment. In the first decade, nodal recurrences appeared earlier than local ones when patients with N0 did not undergo neck irradiation., Conclusion: Surgery on patients with T presentations and adjuvant radiotherapy on T and N0 neck presentations seem to be essential to treat esthesioneuroblastoma. A follow-up for a minimum of 20 years is necessary to ensure complete cure., (© 2010 Wiley Periodicals, Inc. Head Neck, 2011.)

Background: The aim of the study was to characterize the distribution of anterior skull base (ASB) cancers in the pediatric population and to identify predictors of outcome., Methods: This was an international study of 1307 patients undergoing craniofacial surgery for malignant tumors; 6.4% (n = 84) among these were

Background: The relative importance of surgery within multimodality regimens commonly used to treat advanced sinonasal malignancies remains unknown., Methods: One hundred two patients with locally advanced sinonasal cancers treated with proton beam radiation therapy with or without surgery were retrospectively reviewed. Extent of surgery and outcome variables of local control, disease-free survival, and overall survival were evaluated. Patterns of failure were also assessed., Results: Extent of surgery correlated with disease-free survival and overall survival rates. Local control rate, however, was independent of the degree of surgical resection achieved. Overall, treatment failure most commonly resulted from distant metastases, which occurred in 30% of patients and also correlated with extent of surgical resection. Tumor type-specific outcomes reveal differences associated with the extent of surgery achieved., Conclusion: High-dose radiotherapy with proton beam resulted in excellent local control rates in patients with locally advanced sinonasal cancer, irrespective of the extent of surgery. Complete resection, however, was predictive of improved disease-free survival and decreased rate of distant metastasis.

Background: Craniofacial resection (CFR) for patients over 70 years of age is uncommon. This study examines a cohort of 36 patients who had CFR at a single institution with the aim of reporting mortality, complications, and outcome., Methods: Thirty-six patients 70 years of age and older were identified from a prospective database of 234 patients who had CFR at a single institution. The median age was 72 years (range, 70-87). Seventeen (47%) patients had had prior single-modality or combined treatment, which included surgery in 14 (40%), radiation in 13 (36%), and chemotherapy in 2 (6%). Thirty-five patients had a malignant tumor and 1 patient a benign tumor; 15 (42%) had high-grade, 17 (47%) intermediate-grade, and 4 (11%) low-grade pathology. The margins of resection were close or microscopically positive in 18 (50%). Adjuvant radiotherapy was given in 15 (42%) and chemotherapy in 1 (3%). Complications were classified into overall, local, central nervous system (CNS), systemic, and orbital. Overall survival (OS) and disease-specific survival (DSS) were determined using the Kaplan-Meier method. Outcomes were compared with patients less than 70 years of age., Results: Postoperative mortality occurred in 6 (17%) patients and postoperative complications occurred in 23 (64%) patients. Local wound complications occurred in 11 (30%), CNS in 12 (33%), systemic in 6 (17%), and orbital in 1 (3%). Postoperative mortality and complications were significantly higher in patients 70 years of age and older compared with patients less than 70 years of age (17% versus 1.5%, p = .0005; 64% versus 36%, p = .003, respectively). With a median follow-up of 27 months (range, 1-237), the 3 year OS and DSS were significantly poorer than patients less than 70 years of age (OS: 53% versus 69%, p = .0004; DSS: 61% versus 70%, p = .01) due to increased medical comorbidity (53% versus 24%, p = .001) and poorer histology (high-, intermediate-, low-grade histology: 42%, 47%, 11% versus 26%, 47%, 27%, p = .05, respectively) in patients over 70 years of age., Conclusion: CFR in patients 70 years of age and older is associated with increased mortality, increased incidence of complications, and a poorer overall and disease-specific 3-year survival, compared with patients less than 70 years of age. The survival was likely due to increased medical comorbidity and adverse histology. These factors must be taken into account when considering an elderly patient for craniofacial resection.

Background: Olfactory neuroblastoma (ONB) is a rare tumor arising from the olfactory neuroepithelium. There is no universally accepted staging system, and treatment approaches lack uniformity. We present one institution's experience with this tumor and the results of therapy., Methods: Thirty patients treated for ONB at The University of Texas M. D. Anderson Cancer Center between 1979 and 2002 were retrospectively reviewed. The diagnosis of ONB was histologically confirmed for each patient., Results: The mean follow-up was 7.32 years. In 77% of cases, patients received treatment with surgery followed by postoperative radiation therapy. Sixteen percent received chemotherapy as part of their initial treatment. Overall 5-year and 10-year survival rates were 89% and 81%, respectively. Nine patients whose disease was initially stage C had a recurrence. The mean time for recurrence was 4.67 years., Conclusions: The M. D. Anderson Cancer Center approach to ONB is complete surgical resection, usually involving a craniofacial approach, with postoperative radiation therapy. This approach seems to be curative in early-stage disease. Late recurrence warrants long-term follow-up., (2004 Wiley Periodicals, Inc.)

Background: Esthesioneuroblastoma (ENB) is an uncommon malignant neoplasm of the upper nasal cavity. Therapeutic management approaches for this neoplasm lack uniformity and there is no universally accepted staging system., Methods: A retrospective review of 27 patients with histologically confirmed ENB managed at The Johns Hopkins Hospital., Results: Eighty-five percent of patients had surgical resection as part of their disease management. Complete surgical resection was achieved in 62% of patients who had a craniofacial resection. Eighty percent of patients with negative surgical margins remain with no evidence of disease, with a median follow-up of 5.6 years. Adjuvant radiation therapy was beneficial to 62% of patients with positive surgical margins. Clinical responses were observed with cisplatin- and etoposide-containing chemotherapy regimens in patients with advanced disease. A revised staging system based on our experience is proposed., Conclusions: ENB is best managed by craniofacial resection with complete tumor resection. Adjuvant radiation therapy is warranted in patients that remain with positive histologic margins of resection. Chemotherapy with cisplatin- and etoposide-containing regimens may be useful for palliation of advanced disease.

Background: The purpose of this study is to evaluate the survival of patients treated at the University of Virginia Health Sciences Center with an anterior craniofacial resection in conjunction with radiotherapy and/or chemotherapy for malignancies of the superior sinonasal cavity. In addition, the impact of aggressive salvage therapy for patients with recurrent disease is considered., Methods: Between June 1976 and December 1992, a total of 45 patients underwent a craniofacial resection by the Departments of Otolaryngology-Head and Neck Surgery and Neurological Surgery at the University of Virginia. One patient was excluded from the analysis because his neoplasm was benign. Another patient died 2 days postoperatively from multiple strokes. The remaining 43 patients were divided into two subgroups: (1) patients with esthesioneuroblastoma (24 patients) and patients with non-esthesioneuroblastoma malignancies (19). Their survival curves were estimated for the percent survival free of disease by month of follow-up using the product limit of Kaplan and Meier. In addition, the salvage treatment for recurrences was examined for both groups., Results: The 5-year disease-free survival rate for the entire group was 77%, with a 2.3% postoperative mortality. The 5-year disease-free survival for the esthesioneuroblastoma patients was 90%, and that for the non-esthesioneuroblastoma group was 59.1% (p = 0.028). Four of 8 esthesioneuroblastoma patients who recurred and were treated with aggressive salvage therapy were without evidence of disease 5 years after completion of therapy, and 3 of the 10 non-esthesioneuroblastoma patients salvaged were without evidence of disease 57.3 months after therapy (39% surgical salvage)., Conclusions: There is a statistically significant difference between the 5-year disease-free survival for the esthesioneuroblastoma patients and the non-esthesioneuroblastoma patients (90% vs 59.1%; p = 0.028), and aggressive salvage therapy appears to be a more successful option in the esthesioneuroblastoma group of patients.