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Background: Despite advances in techniques for olfactory neuroblastoma (ONB), such as unilateral cranial resection, preserving the patient’s sense of smell remains a challenge. This study aimed to examine the effectiveness of post-operative olfactory training in patients who underwent unilateral resection of ONB., Methods: This retrospective cohort study assessed the effect of post-operative olfactory training on olfactory preservation in patients with ONB undergoing unilateral cranial resection. Patients were divided into training intervention (n = 5) and non-intervention (n = 6) groups. Olfactory tests were conducted pre-operatively and at multiple post-operative intervals., Results: Partial olfactory function was preserved in all cases in the training intervention group, whereas only 17% of cases in the non-intervention group maintained partial olfactory function. Significant improvements in olfactory test scores were observed in the training intervention group compared with the non-intervention group., Conclusions: Our findings suggest that post-operative olfactory training could aid in olfactory preservation for patients with ONB after unilateral cranial resection. However, these results should be interpreted with caution, and further research with larger cohorts and extended follow-up periods is needed to confirm these observations.

Objective: Confocal laser endomicroscopy (CLE) is an optical imaging technique that allows in vivo, real-time, microscope-like assessment of superficial lesions. Although there is substantial data on CLE use in the upper GI tract, there is limited information regarding its application in the nasal cavity and paranasal sinuses. This study aims to assess the feasibility and diagnostic metrics of CLE in the nasal cavity and paranasal sinuses regarding differentiation between healthy/benign and malignant tissue. These structures show, however, a wider variety of frequent and concomitant benign and malignant pathologies, which could pose an increased challenge for optical biopsy by CLE., Methods: We performed CLE on a case series of six patients with various findings in the nose (three chronic rhinosinusitis, adenocarcinoma, meningoenzephalozele, esthesionneuroblastoma). Forty-two sequences (3792 images) from various structures in the nasal cavity and/or paranasal sinuses were acquired. Biopsies were taken at corresponding locations and analyzed in hematoxylin and eosin staining as a standard of reference. Three independent examiners blinded to the histopathology assessed the sequences., Results: Healthy and inflamed mucosa could be distinguished from malignant lesions with an accuracy, sensitivity, specificity, positive predictive value, and negative predictive value of 84.1%, 85.4%, 83.1%, 72.5%, and 92.1%, respectively, with a substantial agreement between raters (Fleiss κ = 0.62)., Conclusion: This technique shows, despite its limitations, potential as an adjunctive imaging technique during sinus surgery; however, the creation of a scoring system based on reproducible and defined characteristics in a larger more diverse population should be the focus of further research to improve its diagnostic value and clinical utility., Level of Evidence: NA Laryngoscope, 134:4217-4224, 2024., (© 2024 The American Laryngological, Rhinological and Otological Society, Inc.)

Abstract: Olfactory neuroblastoma (ONB) is an uncommon malignant tumor typically located in the upper nasal cavity. Olfactory neuroblastoma originating in the nasopharynx is extremely rare and tends to be misdiagnosed. The authors describe a rare case of ONB arising ectopically in the nasopharynx. The patient was a 65-year-old woman with recurrent epistaxis and a feeling of fullness in the right ear. After evaluation, endoscopic surgery was performed. The pathological result proved to be ONB. Postoperative magnetic resonance imaging showed that the tumor was completely resected. The patient proceeded to have 66 Gy of postoperative intensity-modulated radiotherapy and was followed for 36 months without tumor recurrence. Olfactory neuroblastoma originating from the nasopharynx is more rare condition compared with ONB located in other areas in the literature. The symptoms of ONB ectopic to the nasopharynx are similar to those of other nasopharyngeal tumors, which were likely to be misdiagnosed. The treatment principle is the same as that of nonectopic ONB, which is surgery combined with radiotherapy. Surgery can be performed using an endoscopic transnasal approach., Competing Interests: The authors report no conflicts of interest., (Copyright © 2024 by Mutaz B. Habal, MD.)

Background: Endoscopic endonasal surgical resection is an effective therapeutic approach for olfactory neuroblastoma (ONB). Unilateral excision of ONBs with limited extension has been reported with the purpose of preserving olfactory function. We aimed to review implications of surgical management, olfactory preservation feasibility, and survival outcomes in patients who underwent endoscopic unilateral resection of ONB., Methods: A systematic literature review was conducted using the search terms [("Olfactory neuroblastoma") OR ("Esthesioneuroblastoma")] AND [("Unilateral resection") OR ("Olfaction preservation")]. Studies reporting cases of unilateral ONB endoscopic resection with postoperative olfaction assessment were included. Concurrently, records of patients who met inclusion criteria at our institution were reviewed retrospectively. The survival and olfactory outcomes were analyzed in both cohorts., Results: Thirty-three patients were identified in the published literature. Twenty-three (69.7%) reported postoperative olfaction preservation. Olfactory function after surgery did not show an association with Kadish stage (P = 0.128). No evidence of disease was observed at the latest follow-up in this group of patients. Nine patients who met inclusion criteria were identified at our institution. The extent of resection influenced the level of olfaction preservation when cribriform plate and nasal septum resection coexisted (P = 0.05). A single patient at our institution developed recurrence after being lost to follow-up for 22 months., Conclusions: Olfaction preservation can be achieved in patients who undergo endoscopic unilateral resection and adjuvant radiotherapy. The extent of resection should aim for negative margins, particularly in the midline. Larger studies are required to assess the risk of contralateral microscopic disease, and, hence, close follow-up is advised., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Sinonasal malignant tumors are a group of uncommon malignancies that account for less than 1% of all tumors. These tumors often involve the maxillary sinus and nasal cavity, with less cumulative incidence in the ethmoidal sinus, sphenoidal sinus, and frontal sinus. The lack of consensus on the management of sinonasal malignancies is due to their rarity, diagnostic challenges, and the heterogeneity of treatments. In this paper, we present a case of endoscopic-assisted medial canthus incision combined with radiotherapy in the treatment of sinonasal malignant tumors, with the aim of providing valuable insights to clinicians on the management of these tumors., (© 2024. The Author(s).)

Olfactory neuroblastoma (ONB) is an uncommon malignant tumor and is usually treated by a multidisciplinary approach includes surgery, radiotherapy, and chemotherapy. A 62 years-old male had a tumor in the nasal cavity and diagnosed as ONB with Kadish A stage. Anterior skull base surgery was performed as radical treatment. Since the surgical margin was negative, no postoperative radiotherapy was administered. 14 years after the surgery, bilateral otitis media with effusion (OME) was occurred, we found the recurrence tumor at bilateral retropharyngeal lymph node (RPLN) which surrounded the internal carotid arteries. Since these were unresectable, we planned chemoradiotherapy which was 70Gy of intensity modulated radiotherapy combined with two courses of carboplatin and etoposide. The tumor volume was reduced and bilateral OME were improved. He has been alive for 3 years after salvage treatment. Although ONB has a relatively good prognosis, it is known to often cause cervical lymph node metastasis. Grades III and IV of Hyams classification are considered high risk. This case, initial tumor was limited in the nasal cavity and its clinical classification was early stage, but Hyams classification was grade III. In reference to this case, considering that RPLN metastasis are difficult to radically resect at the salvage surgery, including this area in postoperative radiotherapy was considered an option.

Objective: To investigate the clinical characteristics of esthesioneuroblastoma and the efficacy of endonasal endoscopic surgery combined with radiotherapy/chemotherapy. Methods: The clinical and surgical data of 17 patients with esthesioneuroblastoma who underwent endonasal endoscopic surgery in our department from September 2009 to June 2023 were retrospectively analyzed. Results: Among all patients, the modified Kadish stage B was identified in 4 patients, C in 10 patients, and D in 3 patients. Ten of them underwent endonasal endoscopic surgery without neck dissection in one day, whose average operation time is （5.2±2.5） hours and average blood loss is （192±162）mL. Skull base reconstructions were performed in 15 patients, postoperative complications were observed in 3 patients, and negative margins were obtained in 13 patients. All 17 patients were followed up for an average of （49.7±40.2） months. Three patients died and 6 had recurrence and/or metastasis. The 1-year, 2-year and 5-year overall survival rates were 88.2%, 80.2%, and 80.2%, respectively, and the 1-year, 2-year and 5-year disease-free survival rates were 82.4%, 82.4%, and 50.8%, respectively. The 2-year overall survival rates of patients with negative and positive margins were 100% and 25%, respectively, while the 2-year disease-free survival rates were 61.5% and 25.0%, respectively. Conclusion: Endonasal endoscopic surgery combined with radiotherapy/chemotherapy can achieve satisfactory effect in esthesioneuroblastoma, and the prognosis of patients with positive margins is poor., Competing Interests: The authors of this article and the planning committee members and staff have no relevant financial relationships with commercial interests to disclose., (Copyright© by the Editorial Department of Journal of Clinical Otorhinolaryngology Head and Neck Surgery.)

Historically, comprehensive surgical resection for olfactory neuroblastoma has included the bilateral olfactory epithelium, cribriform plate, overlying dura, olfactory bulbs and tracts. This results in postoperative anosmia that may significantly impact a patient's quality of life without definitive added benefit in survival. The prevalence of occult intracranial disease is low, especially for Hyams grade I and II tumors. A unilateral approach sparing the contralateral cribriform plate and olfactory system can be considered for select cases of early stage, low-grade tumors when the disease does not cross midline to involve the contralateral olfactory cleft or septal mucosa and when midline dural margins can be cleared with frozen pathology. Approximately half of patients who undergo unilateral resection may have residual olfaction even with adjuvant unilateral radiation. Early data suggest favorable disease-free survival and overall survival for patients who underwent the unilateral approach; however, larger sample studies are needed to confirm comparability to bilateral resections regarding oncologic outcomes., (© 2024 ARS‐AAOA, LLC.)

Objective: Olfactory neuroblastoma (ONB) is a rare, malignant tumor of the sinonasal tract that arises from olfactory epithelium. Although surgery is the preferred first-line treatment, tumor involvement of adjacent structures may preclude the ability to achieve negative margins during initial resection. Herein, the authors examine the oncological outcomes of patients with positive margins after primary resection of ONB, with the aim of determining predictors of disease progression and patterns of recurrence., Methods: The authors performed an institutional review of 25 patients with positive-margin ONB after resection. Cox survival analyses were used to determine any statistically significant predictors of worse progression-free survival (PFS) and overall survival (OS)., Results: A total of 93 patients who were diagnosed with ONB were identified, of whom 25 patients had positive margins following their primary resection. Eleven (44%) had a delayed finding of positive margins that were initially negative in the operating room but returned as positive on final pathology. Four patients had subtotal resection (STR), whereas the remaining patients underwent gross-total resection. Twenty-four patients received adjuvant radiotherapy (96%), and 15 additionally received adjuvant chemotherapy (60%). Fourteen patients (56%) experienced recurrence/progression at a median time of 35 months following resection (IQR 19-70 months). Local recurrence occurred in 10 patients (40%), regional in 9 (36%), and distant metastasis in 2 (8%). In Cox survival analyses, the 5-year PFS and OS were 55.1% and 79.2%, respectively. Kadish stage D was predictive of worse PFS in univariate (hazard ratio [HR] 15.67, 95% CI 3.38-72.61, p < 0.001) and multivariate (HR 15.46, 95% CI 1.45-164.91, p = 0.023) analyses. Hyams grade, adjuvant chemotherapy, and primary radiotherapy were not associated with PFS. Furthermore, Kadish stage D and STR were predictive of worse OS in univariate analysis (HR 12.64, 95% CI 2.03-78.86, p = 0.007; HR 7.31, 95% CI 1.45-36.84, p = 0.016; respectively). However, local and regional recurrence was not associated with worse OS., Conclusions: Approximately half of patients with positive-margin ONB may experience disease recurrence. Patients with an advanced disease stage (Kadish D) may have a higher likelihood of developing recurrence/progression. Furthermore, patients with tumor burden following resection (STR and Kadish D) may have worse OS. However, in positive-margin ONB with no gross disease following initial resection, the presence of disease recurrence does not significantly alter survival when receiving salvage therapy.

Background: We investigated the outcomes of postoperative radiation therapy for olfactory neuroblastoma (ONB) and our cross-departmental collaboration to enhance the effectiveness of cancer treatment., Methods: We retrospectively evaluated 22 patients with ONB who underwent postoperative radiotherapy after tumor resection. En bloc resection was performed; pathology specimens were prepared in coronal sections; and irradiation fields were determined after discussion with radiation oncologists, head and neck surgeons, and pathologists., Results: The overall survival and local control rates were 95.5% and 100%, respectively, at a median 37-month follow-up. The 3- and 5-year disease-free survival (DFS) rates were 64.4% and 56.3%, respectively. Of the 22 patients, 9 (8 Kadish C and 1 Kadish B) had disease recurrence. Of the nine patients, five had positive margins and two had closed margins; cervical lymph node recurrence occurred in six, and distant metastasis with or without cervical lymph node recurrence occurred in three. DFS analysis of risk factors showed no statistically significant differences, but positive margins were a significant recurrence factor in multivariate analysis., Conclusions: The local control rate of ONB treated with postoperative radiation therapy was 100%. This may be attributed to cross-departmental cooperation between head and neck surgeons, pathologists, and radiation oncologists, which resulted in accurate matching of CT images for treatment planning with the location of the tumor and positive margins. Longer follow-up periods are required to evaluate the effectiveness of our strategy., (© 2024 The Authors. Cancer Medicine published by John Wiley & Sons Ltd.)

Objectives: To explore the prognostic factors in patients with advanced olfactory neuroblastoma (ONB) underwent endoscopic surgery., Materials and Methods: Retrospective medical records were reviewed of patients with pathologically proven ONB who underwent endoscopic surgical resection. Clinicopathological characteristics including patient demographics, treatment, complications, follow-up, and outcomes were analyzed. Kaplan-Meier overall survival (OS) and disease-free survival (DFS) curves were plotted. Univariate and multivariate Cox regression models were used to determine prognostic factors., Results: Eighty-five patients with Kadish stage C ONB were examined. According to the various staging systems used, most patients harbored modified Kadish stage C (78.8%). Twenty-six patients (30.6%) underwent bony skull base resection, 11 (12.9%) underwent dura resection, and 24 (28.2%) underwent additional intracranial resection that included the olfactory bulb and duct. Median follow-up was 39 months. Five-year OS and DFS rates were 83.7% and 74.9%, respectively. Five-year OS was 100% in patients treated with bony skull base resection and 77.5% in those who were not ( P  = .052). Dura resection did not improve OS. Multivariate Cox regression analysis identified perioperative complications ( P  = .009), gross total resection ( P  = .004), orbital invasion ( P  = .014), postoperative radiotherapy ( P  = .030), and bony skull base resection ( P  = .019) as independent prognostic predictors., Conclusion: For patients with advanced ONB, endoscopic surgery in conjunction with radiotherapy and chemotherapy is effective and safe. Dura resection should be performed with caution in selected patients to balance survival and complications. Postoperative radiotherapy is important to improve OS and DFS., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Background: Peputide receptor radionuclide therapy with 177Lu for midgut neuroendocrine metastasis has been clinically approved as a safe treatment. Unresectable metastases of olfactory neuroblastoma have shorter survival due to insufficient effective systemic treatment., Case Report: Herein, we report a patient treated with peputide receptor radionuclide therapy for unresectable recurrent olfactory neusroblastoma following a rare cranial metastasectomy infection. A 50-year-old female patient with olfactory neuroblastoma of Kadish C was initially treated by skull base surgery plus postoperative radiotherapy following chemotherapy. Recurrent disease with neck and intracranial metastases was treated by four salvage surgeries. Surgical site infection following intracranial metastasectomy was treated with debridement and delayed cranioplasty. Peputide receptor radionuclide therapy was performed for unresectable multiple metastases after cranioplasty. Successful therapy using four cycles of peputide receptor radionuclide had neither grade 3 nor grade 4 adverse events. The patient was followed at an outpatient clinic., Conclusion: Further case accrual of peputide receptor radionuclide therapy is required to develop a treatment for unresectable olfactory neuroblastoma., (Copyright © 2023 International Institute of Anticancer Research (Dr. George J. Delinasios), All rights reserved.)

Objective: Esthesioneuroblastoma (ENB), also known as olfactory neuroblastoma, is a rare, malignant tumor of neuroectodermal origin that arises from the olfactory neuroepithelium. In this study the authors present the first series in the literature on distant brain metastases (BMs) secondary to ENB that were treated with stereotactic radiosurgery (SRS), to evaluate the safety and effectiveness of SRS for this indication., Methods: A retrospective analysis of clinical and radiological outcomes of patients with ENB who underwent CyberKnife (CK) SRS at a single center was conducted. The clinical and radiological outcomes of patients, including progression-free survival, overall survival, and local tumor control (LTC) were reported., Results: Between 2003 and 2022, 32 distant BMs in 8 patients were treated with CK SRS at Stanford University. The median patient age at BM diagnosis was 62 years (range 47-75 years). Among 32 lesions, 2 (6%) had previously been treated with surgery, whereas for all other lesions (30 [94%]), CK SRS was used as their primary treatment modality. The median target volume was 1.5 cm3 (range 0.09-21.54 cm3). CK SRS was delivered by a median marginal dose of 23 Gy (range 15-30 Gy) and a median of 3 fractions (range 1-5 fractions) to a median isodose line of 77% (range 70%-88%). The median biologically effective dose was 48 Gy (range 21-99.9 Gy) and the median follow-up was 30 months (range 3-95 months). The LTC at 1-, 2-, and 3-year follow-up was 86%, 65%, and 50%, respectively. The median progression-free survival and overall survival were 29 months (range 11-79 months) and 51 months (range 15-79 months), respectively. None of the patients presented adverse radiation effects., Conclusions: In the authors' experience, SRS provided excellent LTC without any adverse radiation effects for BMs secondary to ENB.

Objectives: Olfactory neuroblastoma (ONB), also known as esthesioneuroblastoma, is a rare malignant neoplasm of the nasal vault and anterior skull base. The results of treatment for ONB are relatively good; however, regional and distant metastases can develop several years after definitive treatment. This study aimed to validate the treatment modality of ONB for oncological outcomes, especially for regional recurrence., Methods: We retrospectively reviewed the medical records of 22 patients diagnosed with ONB at Kyoto University Hospital between 2009 and 2020. Descriptive statistics were calculated, and Kaplan-Meier curves were used., Results: The median follow-up time was 58.2 months. One (4.5%) patient was clinically node positive, (cN+) and the remaining 21 (95.5%) were clinically node negative (cN0) at presentation. Eighteen patients underwent an endoscopic endonasal approach (EEA) for primary resection, and the remaining four patients underwent a combined EEA and transcranial approach. Elective neck dissection was not performed for 21 patients with cN0 ONB, whereas unilateral neck dissection with removal of ipsilateral lateral retropharyngeal node was performed for one patient with cN+ ONB. Postoperative radiotherapy without concurrent chemotherapy was performed only at the primary tumor bed for 21 patients with cN0 ONB, and at the primary tumor bed and bilateral neck for one patient with cN+ ONB. The 5-year overall, disease-specific, and disease-free survival rates were 94.1%, 100%, and 69.6%, respectively. No patients developed local recurrence, but 6 (27.2%) patients experienced recurrence with a median time to recurrence of 36.4 months, including four and two patients who initially developed regional recurrences and bone metastases, respectively. Five (22.7%) patients had delayed neck recurrence. The salvage rate was only 60.0% in the five patients who had delayed neck recurrence. Regarding the level of delayed neck recurrence, 4 (18.2%) patients had lateral retropharyngeal lymph node metastases., Conclusion: Patients with ONB have excellent survival outcomes after endoscopic surgical resection of the primary lesion with postoperative radiotherapy only to the primary tumor bed. Despite excellent survival, delayed neck recurrence, including the lateral retropharyngeal lymph node, remains high. Because salvage surgery for lateral retropharyngeal lymph node recurrence is sometimes technically difficult, it may be better to extend the field of postoperative radiotherapy from the primary tumor bed only to include bilateral lateral retropharyngeal lymph node regions in patients with clinically N0 ONB. Further prospective studies with a large number of patients are needed to determine the extent of postoperative radiotherapy., Competing Interests: Declaration of Competing Interest The authors have no funding, financial relationships, or conflicts of interest to disclose., (Copyright © 2022 Japanese Society of Otorhinolaryngology-Head and Neck Surgery, Inc. Published by Elsevier B.V. All rights reserved.)

Background and Importance: Esthesioneuroblastoma (ENB) is a rare anterior skull base tumor derived from olfactory epithelium. There are very few operative videos in the literature demonstrating the surgical resection techniques for giant ENB because of their rarity and complexity. In this technical report, we demonstrate the microsurgical resection of a very large and complex high-grade ENB, initially deemed unresectable, through a bifrontal craniotomy and extended subfrontal approach combined with an endonasal endoscopic approach., Clinical Presentation: A 34-year-old woman presented with headaches, nasal congestion, and bloody nasal drainage. MRI showed a large nasal cavity mass with extension into the anterior cranial fossa and bifrontal lobes. There was significant bifrontal edema causing headaches and abutting the optic nerves. Initial management with surgical resection was offered to the patient for local tumor control and to preserve her vision. A combined bifrontal craniotomy and endonasal transsphenoidal approach was used for resecting this giant tumor. After achieving gross total resection, we reconstructed the anterior skull base in layers. She developed several postoperative complications which were appropriately managed., Conclusion: We demonstrate the surgical resection of a giant ENB through a combined transcranial and endonasal endoscopic approach. We discuss the several postoperative complications in this complex case and the lessons learned., (Copyright © Congress of Neurological Surgeons 2023. All rights reserved.)

Olfactory neuroblastoma (ONB) is a rare tumour of the skull base, typically originating from the nasal cavity and around the cribriform plate. We present the rare case of ONB originating from and limited to the sphenoid sinus in a 42-year old lady. Pre-operatively the lesion was thought to be a sinonasal polyp and underwent functional endoscopic sinus surgery (FESS) and total excision of the polypoid lesion. Review of histology unexpectedly revealed ONB. She underwent further surgery to ensure wide local excision was achieved with negative margins on histology, followed by radiotherapy. This is only the third reported case of ONB limited to the sphenoid sinus and the ninth reported case of primary sphenoid ONB in the literature. We review the literature pertaining with primary sphenoidal ONB here and suggest complete resection is indicated in ectopic ONB, not unlike classical ONB. There may be a role for adjuvant oncological treatments and lifelong follow up in a multidisciplinary approach is recommended.

Objective: Esthesioneuroblastoma (ENB) is a rare malignant neoplasm arising from the olfactory epithelium of the cribriform plate. The goal of this study was to update our oncologic outcomes for this disease and explore prognostic factors associated with survival., Materials and Methods: We performed a retrospective analysis of patients with ENB treated at a single tertiary care institution from January 1, 1960, to January 1, 2020. Univariate and multivariate analysis was performed. Overall survival (OS), progression-free survival (PFS), and distant metastasis-free survival (DMFS) were reported., Results: Among 143 included patients, the 5-year OS was 82.3% and the 5-year PFS was 51.6%; 5-year OS and PFS have improved in the modern era (2005-present). Delayed regional nodal metastasis was the most common site of recurrence in 22% of patients (median, 57 months). On univariate analysis, modified Kadish staging (mKadish) had a negative effect on OS, PFS, and DMFS (p < 0.05). Higher Hyams grade had a negative effect on PFS and DMFS (p < 0.05). Positive margin status had a negative effect on PFS (p < 0.05). Orbital invasion demonstrated worsening OS (hazard ratio, 3.1; p < 0.05). On multivariable analysis, high Hyams grade (3 or 4), high mKadish stage (C+D), and increasing age were independent negative prognostic factors for OS (p < 0.05). High Hyams grade (3+4), high mKadish stage (C+D), age, and positive margin status were independent negative prognostic factors for PFS (p < 0.05). High Hyams grade (3+4) was an independent negative prognostic factor for DMFS (p < 0.05)., Conclusions: Patients with low Hyams grade and mKadish stage have favorable 5-year OS, PFS, and DMFS., (© 2022 ARS-AAOA, LLC.)

Purpose: Esthesioneuroblastoma (ENB) is a rare malignant neoplasm of the olfactory epithelium with an estimated incidence of 0.4/million. It can directly extend along the cribriform plate in order to metastasize to the central nervous system. However, non-contiguous intracranial involvement without recurrence at the primary site is extremely uncommon. In this report, the authors review the literature and present a case of non-contiguous intracranial metastasis of ENB without recurrence at the primary site. To the best of our knowledge, this case presents the longest disease-free interval reported in the literature., Methods: A systematic review of literature was conducted in accordance with the PRISMA guidelines. Additionally, the presentation, surgical management, and post-operative outcomes of an 82-year-old female with non-contiguous intracranial metastasis of ENB after 19 years of remission are described., Results: A total of 137 deduplicated works were identified after the search. Of these, 6 papers satisfied our inclusion criteria for our systematic review. Average age at presentation was 50.8 years (range: 26-66) and 52.6% of patients were female. A majority of cases achieved gross-total resection and received adjuvant radiotherapy for initial treatment. The median interval to intracranial metastasis was 6 years from the time of primary tumor presentation. The median overall survival from ENB recurrence with non-contiguous intracranial metastasis was 11.5 months., Conclusions: ENB is a highly recurrent tumor and harbors the potential to involve the intracranial space even years after remission. Intracranial involvement entails poor overall survival. Lifetime radiographic follow-up should be considered in all patients with ENB., Competing Interests: Declaration of Competing Interest All authors declare that they have no conflict of interest., (Copyright © 2022 The Authors. Published by Elsevier B.V. All rights reserved.)

Pupose: To evaluate the impact of postoperative radiotherapy (PORT) on survival in olfactory neuroblastoma (ONB) patients with different tumor staging., Material and Methods: Patients with ONB were selected in the Surveillance, Epidemiology and End Results (SEER) database from 2004-2016. Survival analyses were performed using Kaplan-Meier (K-M) method, Cox regression analysis, and competing risk model., Results: A total of 513 patients were included in the study. Univariate and multivariate analysis results demonstrated that PORT was not an independent prognostic factor for overall survival (OS) of modified Kadish stage A and B patients (P=0.699 and P=0.248, respectively). Kadish stage C and D patients who underwent PORT had significantly better OS than those who did not undergo PORT (P=0.03 and P<0.0001). K-M curves revealed that the 5- and 10-year OS rates of patients who underwent PORT vs. non-PORT were 85.3% vs. 70.4% and 68.2% vs. 56.8% in stage C patients, respectively. For stage D patients, the 5-year OS rates were 70.7% and 42.6%, and 10-year OS rates were 53.4% and 29.5% in the PORT and non-PORT groups, respectively. The competitive risk model revealed that the 5-year cancer-specific cumulative mortality incidence decreased by 26.6% while the 10-year mortality incidence decreased by 41.4% in Kadish stage C patients who were treated using PORT; meanwhile, for Kadish stage D patients who were treated with PORT, the 5- and 10-year mortality incidences were reduced by 35.3% and 42.6%, respectively. Furthermore, we found that chemotherapy was not related to the prognosis of ONB patients (all P>0.05)., Conclusion: Our results indicate that PORT improved survival outcomes of modified Kadish stage C and D ONB patients. However, PORT may not affect survival for modified Kadish stage A and B individuals. Chemotherapy was not recommended for ONB; therefore, further studies are warranted to determine its therapeutic significance., (Copyright © 2022 The Author(s). Published by Elsevier Masson SAS.. All rights reserved.)

Background: Olfactory neuroblastoma, also known as esthesioneuroblastoma, accounts for only 3-6% of sinonasal malignancies but confers a 40% 5-year overall survival., Method: The authors describe techniques for the endonasal, minimally invasive resection of an esthesioneuroblastoma in a 69-year-old man who presented with headaches and anosmia and describe surgical nuances and their effect on adjuvant therapy planning., Conclusion: This approach, along with microsurgical techniques, helped increase tumor visualization, improved marginal resection, and reduced surgical risk, which may improve patient outcomes. Multilayered reconstruction with a synthetic dural substitute and creation of a nasoseptal flap were performed to reduce postoperative cerebrospinal fluid leak., (© 2022. The Author(s), under exclusive licence to Springer-Verlag GmbH Austria, part of Springer Nature.)

Objective: Olfactory neuroblastoma (ONB) is a rare malignant tumor arising in the upper nasal cleft. Rarely, ONB may originate in ectopic sites and the impact of this on prognosis and treatment strategies continues to be debated., Methods: A retrospective analysis was undertaken of patients with ectopic ONB treated between 2000 and 2020 in a tertiary-care referral center for skull base tumors. Three patients were included in this analysis: a 37-year-old woman with ONB arising from the bulla ethmoidalis; a 28-year-old man with inappropriate secretion of antidiuretic hormone caused by a maxillary sinus ONB; and a 41-year-old man with lacrimal sac ONB. Preoperative workup, surgical approach, adjuvant treatments and postoperative surveillance were analyzed. Relevant literature published between 2000 and January 2021 was fully reviewed to investigate oncologic outcomes and delineate the standard of care for such rare tumors., Results: All patients were treated via endoscopic endonasal resection with radical intent, followed by adjuvant treatments when required. No recurrences of disease were observed after a mean follow-up time of 32 months (range, 12-60 months). Data emerging from the literature suggest that a multidisciplinary treatment approach, including free-margins surgical resection followed by adjuvant radiotherapy or radiochemotherapy, is recommended. Olfactory bulb and dura preservation should be attempted whenever feasible., Conclusions: Endoscopic endonasal surgery should be preferred, when possible, to achieve complete excision to minimize patients' morbidity. The ectopic site of origin affects prognosis and should be considered when selecting the appropriate multimodal treatment strategy., (Copyright © 2021 Elsevier Inc. All rights reserved.)

We present the update of the recommendations of the French society of radiotherapy and oncology on the indications and the technical methods of carrying out radiotherapy of sinonasal cancers. Sinonasal cancers (nasal fossae and sinus) account for 3 to 5% of all cancers of the head and neck. They include carcinomas, mucosal melanomas, sarcomas and lymphomas. The management of sinonasal cancers is multidisciplinary but less standardized than that of squamous cell carcinomas of the upper aerodigestive tract. As such, patients with sinonasal tumors can benefit from the expertise of the French expertise network for rare ENT cancers (Refcor). Knowledge of sinonasal tumour characteristics (histology, grade, risk of lymph node involvement, molecular characterization, type of surgery) is critical to the determination of target volumes. An update of multidisciplinary indications and recommendations for radiotherapy in terms of techniques, target volumes and radiotherapy fractionation of the French society of radiotherapy and oncology (SFRO) was reported in this manuscript., (Copyright © 2021 Société française de radiothérapie oncologique (SFRO). Published by Elsevier Masson SAS. All rights reserved.)

Objective: Esthesioneuroblastoma (ENB) is a rare malignancy of the sinonasal tract and its infrequency has confounded efforts at clearly describing the survival trends associated with this neoplasm over the years. In this study, we reviewed survival trends in ENB and investigated the impact of treatment extent and modality on patient outcomes., Methods: We accessed the Surveillance, Epidemiology, and End Result (SEER) program to identify ENB cases from 1998 to 2016. A χ 2 test was used to compare the categorical covariates and a t test or Mann-Whitney U test was utilized for continuous variables. The impact of prognostic factors on survival was computed using a Kaplan-Meier analysis and multivariate Cox proportional hazards model. We divided ENB patients into 4 periods including 1998-2002, 2003-2007, 2008-2012, and 2013-2016, and investigated survival trends using the Kaplan-Meier curve and log-rank test., Results: ENB patients who underwent biopsy alone were associated with older age, larger tumor diameter, increased rates of tumor extension, nodal/distant metastases, and advanced stages as compared with patients undergoing tumor resection. Our results also demonstrated that surgical resection and adjuvant radiotherapy could confer survival advantages, whereas chemotherapy was associated with reduced survival in patients with ENB. Over the past 2 decades, surprisingly, there has been no change in survival rates for patient with ENB (P = 0.793)., Conclusions: Despite advanced diagnostic studies and modernized treatment approaches, ENB survival has remained unchanged over the years, calling for improved efforts to develop appropriate individualized interventions for this rare tumor entity. Our results also confirmed that surgery and adjuvant radiotherapy is associated with improved patient survival whereas the use of chemotherapy should be considered carefully., (Copyright © 2021 Elsevier Inc. All rights reserved.)

Case Description: A 4-year-old sexually intact male leucistic axolotl (Ambystoma mexicanum) was presented with a 2-week history of dysrexia and difficulty swallowing., Clinical Findings: Physical examination revealed a 1-cm-diameter intraoral mass on the rostral aspect of the palate and swelling of the left nasal fossa. Local invasion into the left nasal fossa was suspected during oral examination. The lesion was marginally excised, and an incompletely excised olfactory neuroblastoma was diagnosed histologically. Five weeks later, physical examination revealed persistent erythema, delayed healing of the rostral portion of the palate, and a mild facial deformity associated with a white mass in the nasal cavity., Treatment and Outcome: 6 weeks after excision, adjuvant electron (6-MeV) beam radiotherapy was initiated for treatment of the incompletely excised olfactory neuroblastoma and likely presence of a recurrent mass. The protocol consisted of 4 weekly fractions of 8 Gy each (total, 32 Gy) with the axolotl under anesthesia. No acute adverse radiation effects were noted following radiotherapy. The oral erythema resolved after the third session. No recurrence was observed 2 months after treatment, and the owners reported no abnormal signs at home. The axolotl died 3.5 months after radiotherapy was completed (8 months after marginal excision of the tumor) secondary to an environmental management failure. Postmortem histologic evaluation showed no evidence of neoplasia., Clinical Relevance: In axolotls, olfactory neuroblastoma should be considered in the differential diagnosis of intraoral palatal masses. This report describes the first application of radiotherapy for treatment of an olfactory neuroblastoma in an axolotl.

Abstract: Esthesioneuroblastoma is a rare neoplasm originating from the olfactory neuroepithelium at the cribriform plate. The superior nasal cavity is primarily affected. Morbidity and mortality are related to locally destructive growth as well as metastatic potential. Orbital involvement is associated with decreased survival. The authors describe a case of advanced esthesioneuroblastoma with bilateral orbital involvement, presenting with a rare constellation of orbital hypertelorism and Foster-Kennedy Syndrome., Competing Interests: The authors report no conflicts of interest., (Copyright © 2021 by Mutaz B. Habal, MD.)

Introduction: The short-term adverse events and predictors of morbidity in surgical resection of esthesioneuroblastoma (ENB) are largely unknown, and investigating these variables can help direct planning for at-risk patients., Methods: The 2005-2017 National Surgical Quality Improvement Program database was queried to identify patients with a diagnosis of ENB undergoing skull base surgery for tumor resection. Information regarding demographics, patient morbidity score, pre-operative and intra-operative data, and post-operative outcomes were extracted. Cox proportional hazard analysis was utilized to assess complication and readmission/reoperation rates., Results: A total of 95 patients undergoing skull base surgery for resection of ENB were included. Mean age, BMI, operation time, and post-operative length of stay (LOS) of the cohort were 53.6 ± 16.2 years, 29.1 ± 6.5, 392.0 ± 204.6 minutes, and 5.8 ± 4.6 days, respectively. In total, 31 patients (32.6%) experienced at least one 30-day adverse event, which included blood transfusion intra-operatively or within 72 hours from the operation (22.1%), readmission (10.7%), intubation >48 hours (7.4%), reintubation (4.2%), organ or space infection (4.2%), reoperation (4.0%), superficial or deep surgical site infection (2.1%), sepsis (2.1%), pulmonary embolism (1.1%), and myocardial infarction (1.1%). Patients who experienced at least one adverse event had significantly higher operation time (486.8 ± 230.4 vs. 347.5 ± 176.2 minutes, p  = 0.002), LOS (9.2 ± 5.6 days vs. 4.2 ± 3.0, p  < 0.001), and lower hematocrit (37.3 ± 5.9 vs. 41.2 ± 3.8, p  < 0.001) and albumin levels (3.8 ± 0.6 vs. 4.2 ± 0.3, p  = 0.009). Patients with a higher American Society of Anesthesiologists (ASA) score (HR = 2.39; p  = 0.047) or longer operation time (HR = 1.004; p  = 0.001) had a significantly higher risk for experiencing adverse events. Obesity was not associated with different intra- or post-operative outcomes, but older patients had shorter operations ( p  = 0.002) and LOS ( p  = 0.0014)., Conclusion: Longer operation time and lower pre-operative hematocrit and albumin levels may all increase complication rates in ENB resection. Patients with high ASA score or more advanced age may have different short-term outcomes.

Objective: The endoscopic endonasal approach (EEA) has been applied in the treatment of olfactory neuroblastoma (ONB). However, there is a lack of research examining the impact of EEA on locally advanced ONB. This study assessed the outcomes of EEA in patients with locally advanced ONB and its impact on the quality of life (QOL)., Methods: We retrospectively reviewed patients with Kadish stage C ONB who underwent EEA between December 2004 and October 2019 and assessed demographic data, histopathologic grade, the extent of resection, postoperative complications, and outcomes. Preoperative and postoperative QOL was assessed using the Sino-Nasal Outcome Test., Results: Twenty-six patients (18 men, 8 women; aged 26-79 years) were enrolled, with 12 cases of Hyams grade II and III and 1 case of grade I and IV each. In total, 25 patients received radiotherapy and 16 patients received chemotherapy, of whom 11 received preoperative neoadjuvant chemotherapy. Postoperative nasal bleeding was observed in 2 patients. The follow-up ranged from 8 to 124 months (median, 42.3 months). The 1-year and 5-year overall survival were 96.2% and 84.8%, respectively. The 1-year and 5-year disease-free survival were 76.9% each. The analysis of the postoperative Sino-Nasal Outcome Test scores showed significant improvement in certain psychological and sleep-associated domains, compared with the preoperative scores., Conclusions: Our results showed that pure EEA followed by radiotherapy offered excellent outcomes in the management of selected patients with locally advanced ONB. The postoperative QOL was significantly improved. More research is required on neoadjuvant chemotherapy to establish its role., (Copyright © 2021. Published by Elsevier Inc.)

Background: For olfactory neuroblastoma (ONB), the combination of surgical tumor resection and radiation therapy (RT) has been considered the "gold standard" in treatment protocols intended to cure., Objective: To summarize evidence on the treatment of ONB using surgery alone., Methods: A retrospective institutional case series, a systematic review of the literature, and an individual patient data (IPD) meta-analysis on only surgically treated ONB patients., Results: At our institution, a total of 10 patients were treated through surgery alone and remained alive and free of disease at last follow-up. The IPD meta-analysis on 128 patients revealed a disease-free survival (DFS) and overall survival (OS) of 67.7% and 75.4% at 5 years and 57.1% and 71.9% at 10 years, respectively. Univariate analysis showed that Kadish stage C/D and Hyams grading III//IV significantly affected OS (P = 0.000 and P = 0.000) and DFS (P = 0.000 and P = 0.002). For low-risk patients, the DFS was 80.6% at 5 years and 67.8% at 10 years, respectively., Conclusions: Surgery alone is an equivalent alternative to combined treatment in carefully selected low-risk ONB patients with better outcome measures than previously reported.

Background: The role of surgery is not well defined in locally advanced sinonasal cancers with intracranial involvement after all medical options have been exhausted. We hypothesize that patients whose tumors are deemed unresectable and referred to palliative care may benefit from radical salvage surgery., Methods: We performed a single-center retrospective review of patients with malignant, locally advanced (stage T4b) sinonasal cancers with intracranial involvement from 2000 to 2020, inclusive. Data were collected on the patient demographics, details of chemotherapy, radiation, histology, perioperative complications, surgical approaches, and survival. We compared the survival outcomes of patients with different duration of disease before presentation., Results: We identified 17 patients who had undergone salvage surgical resection of treatment-recalcitrant, locally advanced sinonasal tumors. Almost all patients had undergone prior surgery, radiotherapy, and chemotherapy. Perioperative complications occurred in 6 of 17 patients with 1 death. Patients with clinically less aggressive disease had significantly longer progression-free and overall survival compared with the more aggressive group., Conclusions: Salvage surgery for locally advanced sinonasal cancers with intracranial invasion that is recalcitrant to all other therapies should be considered for patients who otherwise have no other treatment options., (Crown Copyright © 2021. Published by Elsevier Inc. All rights reserved.)

Background: A combined transcranial and transfacial approach has long been the gold standard for surgical management of large tumors with sinonasal and skull base involvement. The extended endoscopic endonasal approach for such pathologies has its advantages, but it has flaws as well, such as anatomic limitations and more ponderous skull base reconstruction and thus higher risk of postoperative complications. Our primary technique for surgical treatment of these pathologies has been a combination of transfacial and minimally invasive transciliary supraorbital keyhole approaches. With the aim to further minimize invasiveness, potential complications, and unsatisfactory aesthetic outcomes during surgical treatment of large tumors invading both the sinonasal area and the skull base, we abandoned the transfacial approach and simultaneously combined the transciliary supraorbital keyhole approach with the endoscopic endonasal approach., Methods: The well-known microscope-assisted minimally invasive approach via a transciliary supraorbital keyhole craniotomy was combined with the endoscopic endonasal approach., Results: Six patients with different histologic types of tumors affecting the sinonasal area and the skull base were operated on. The mean operative time was 3 hours, there were no unexpected intraoperative or postoperative complications, and total tumor removal was achieved in each patient. None of the patients experienced complications associated with the surgery during follow-up., Conclusions: Our combined simultaneous multiportal approach enables total tumor eradication with reduced operative time and is associated with minimal intraoperative and postoperative complications, low mortality rate, and excellent cosmetic results., (Copyright © 2021 The Authors. Published by Elsevier Inc. All rights reserved.)

Background: Olfactory neuroblastoma, or esthesioneuroblastoma (ENB), is an uncommon sinonasal malignancy arising from olfactory neuroepithelium that is optimally treated with surgical resection. The objective of this study is to determine preoperative predictors of undergoing open versus endoscopic approach for ENB and to investigate significant postoperative survival outcomes between the two surgical approaches., Methods: The National Cancer Database (NCDB) was queried for cases of ENB histology that were treated surgically from 2010 to 2015. Groups were stratified into open or endoscopic approach cohorts. Patient demographics, tumor characteristics, treatment modality, and 5-year overall survival were compared between the two groups using Chi-Square analysis and Kaplan-Meier survival analysis. Cases were classified as Kadish stage A, B, C, or D based on the "Collaborative Stage-Extension" codes in NCDB., Results: Of 533 patients meeting inclusion criteria, 276 (51.8%) patients underwent open, and 257 (48.2%) patients underwent endoscopic surgical approaches. Patients undergoing endoscopic surgery were more likely to be Kadish stages A and B and less likely to be stages C and D ( P  = .020). Those undergoing endoscopic approach overall had a shorter mean hospital stay postoperatively (3.8 vs. 7.0 days, P  < .001). Endoscopic cases had a greater 5-year overall survival (81.9% vs. 75.6%, P  = .030); after multivariate regression, there was a trend toward survival benefit to endoscopic surgery that did not reach clinical significance (HR 0.644, [0.392-1.058], P  = .083)., Conclusion: Although not statistically significant, there is a trend toward increased overall survival with an endoscopic approach in patients undergoing surgery for ENB as compared to an open approach, regardless of Kadish stage. An endoscopic approach is an adequate alternative to an open approach for the surgical treatment of ENB.

Introduction: In old and frail patients, oncologic anterior skull-base surgery through an endonasal endoscopic approach avoids the morbidity incurred by transfacial and transcranial approaches, sometimes considered unreasonable, although surgery remains the gold standard treatment for sinonasal cancer., Objectives: To assess the functional and oncologic results of this surgery in over-70 year-olds., Material and Methods: A single-center retrospective study included all patients aged over 70 years at surgery, who underwent endonasal endoscopic oncologic resection and reconstruction of the anterior skull base, between October 2008 and October 2018., Results: Fifteen procedures in 13 patients met the inclusion criteria. Mean hospital stay was 7 days. All resections were considered R0, apart from one case with positive dura-mater margins (6.7%). All patients had complete radio-surgical treatment, in accordance with the REFCOR recommendations. Two cases of meningitis were reported (13.3%). At a median follow-up of 27 months, 4 patients presented local recurrence, 1 of whom also had lung metastases. Two patients died of disease-related or treatment-related causes., Conclusion: This technique is a feasible treatment in patients aged over 70 years, providing good functional results, and acceptable oncologic outcome., (Copyright © 2019 Elsevier Masson SAS. All rights reserved.)

Objective: To compare the Kadish and the modified Dulguerov staging of individual participants to determine the impact of stage and other prognostic factors on disease-free (DFS) and overall survival (OS)., Data Sources: Systematic review of EMBASE, MEDLINE, Cochrane Library, and CINAHL databases., Review Methods: The Preferred Reporting Items for Systematic Reviews and Meta-analysis (PRISMA) was followed for this study. Articles including patients with olfactory neuroblastoma (ONB) staged with both Kadish and Dulguerov staging systems were reviewed. The raw data from eligible studies were requested to perform an individual participant data (IPD) meta-analysis., Results: Pooled data from 21 studies representing 399 patients with ONB undergoing treatment with curative intent showed that increasing age, treatment with chemotherapy, and positive or unreported margin status portended worse DFS ( P < .05). Increasing stage for both Kadish and Dulguerov staging systems was prognostic for worse DFS and OS ( P < .05), with Kadish C representing a heterogeneous group with regard to outcome and corresponding Dulguerov T stage. Using the Akaike information criterion, the Dulguerov staging system had superior performance to the Kadish system for DFS (1088.72 vs 1092.54) and OS (632.71 vs 644.23)., Conclusion: This study represents the first IPD meta-analysis of ONB directly comparing the outcomes of Kadish and Dulguerov staging systems in patients treated with primary surgery. Both systems correlated with DFS and OS, with superior performance in the Dulguerov system. Furthermore, the Kadish C group represented a heterogeneous group with regard to outcomes after stratification by the Dulguerov system. Dulguerov T4 patients had the worst outcome, with most being approached with open resection.

Esthesioneuroblastomas are uncommon tumors in pediatric patients and are typically treated with multimodal therapy. Changes in gross tumor quality and character in response to adjuvant treatment have not been clearly reported. We report the case of a 15-year-old female with a diagnosis of Kadish stage C esthesioneuroblastoma who was treated with neoadjuvant chemotherapy and surgical resection. The patient's tumor demonstrated cytoreduction after chemotherapy but also was found to have calcified. A combined trans-frontal sinus craniotomy with endoscopic endonasal resection was performed and resulted in negative margins and good clinical outcome.

Tumour-to-tumour metastasis is a rare phenomenon. It occurs when a primary tumour is a recipient of a separate tumour within the same individual. We present a case of a 66-year-old woman with known breast cancer who presented with one-sided nasal symptoms. Examination and imaging revealed a unilateral polyp arising from the skull base. She underwent endoscopic polypectomy with the histology demonstrating tumour-to-tumour metastasis from a breast carcinoma to an olfactory neuroblastoma, a rare sinonasal tumour. Clinicians should be cautious of distant metastases in any patient presenting with head and neck symptoms and a known primary tumour. This is the first documented case of this type.

Background: Olfactory neuroblastoma, also called esthesioneuroblastoma (ONB), is a rare neuroectodermal neoplasm that originates from the olfactory epithelium of the nose-sinus tract. It generally occurs with epistaxis, nasal obstruction, diplopia, and anosmia., Methods: A 16-year-old female was admitted to our Unit with a complaint of nasal obstruction, recurrent epistaxis, anosmia, and intermittent headache of sixth month's duration. After the ENT consultation, physical examination, endonasal endoscopy, and multiple biopsies were performed. Instrumental images (CT, MRI) have been requested to stage the aforementioned pathology., Results: Instrumental images (CT, MRI) showed a mass filling the right nasal cavity and the maxillary bone and involving the cribriform plate without evidence of dural invasion. Craniofacial resection by means of a bifrontal craniotomy combined with a modified lateral rhinotomic transfacial route was performed. The reconstruction of the inferior and medial orbital walls with employing split-thickness calvarial grafts, pedicled galea-pericranium flap rotated downwards was performed. The patient received 56 Gy of external beam radiotherapy over a 6-week period., Conclusion: Early diagnosis and treatment coordinated by a multidisciplinary team of ENTs, neurosurgeons, oncologists, pathologists, and radiologists are a prerequisite for a good prognosis. An excellent surgical debulking, negative margins, and subsequent locoregional control of the pathology through radiotherapy is fundamental.

Objective: The aim of this study is to evaluate the efficacy of endoscopic surgery and conventional surgery combined with radiotherapy in the treatment of Neuroblastoma. Method: Forty-three patients with olfactory neuroblastoma undergoing surgery combined with radiotherapy were retrospectively analyzed. The patients were divided into endoscopic surgery and conventional surgery. All patients received postoperative radiotherapy at a dose of 60-70 Gy, the 5-year survival rate and local recurrence time of the two groups were compared, and the therapeutic effects of endoscopic surgery and traditional surgery were compared. Result: Through survival analysis, the 5-year overall survival rates of the traditional surgery group and the endoscopic surgery group were 50% and 58% （ P =0.560）, the local recurrence rates were 44% and 48% （ P =0.288）, and the mean recurrence time was 5.6 months and 12.5 months （ P =0.032）. Conclusion: There was no difference between endoscopic surgery and conventional surgery combined with radiotherapy in the treatment of Neuroblastoma, and the time of local recurrence was significantly prolonged. In early Neuroblastoma, endoscopic sinus surgery may be superior to open surgery in terms of efficacy and patient survival., Competing Interests: The authors of this article and the planning committee members and staff have no relevant financial relationships with commercial interests to disclose., (Copyright© by the Editorial Department of Journal of Clinical Otorhinolaryngology Head and Neck Surgery.)

Sinonasal malignant tumors are characterized by high histological variability and complexity of the differential diagnosis. Currently, there are classifications of these tumors, which are based on their localization and involvement of various anatomical structures. However, generally accepted algorithms for treatment of this pathology have not yet been developed. This review describes the most important algorithms for treatment of the most common histological variants of sinonasal malignant tumors: squamous cell carcinoma, adenocarcinoma, sinonasal undifferentiated carcinoma, esthesioneuroblastoma, adenoid cystic cancer, and sinonasal adenocarcinoma. The main problems in choosing the approach for treating these tumors are the lack of generally accepted resectability criteria and contradictions between oncological and neurosurgical indications for surgical treatment. Further research is needed to study the role of radiosensitizers and radioprotectors in comprehensive treatment of sinonasal malignant tumors.

Objective: The Caldwell-Luc (CL) approach with a sublabial incision is used to manipulate the anterior wall of the maxillary sinus. Paresthesia is one of the major complications associated with the CL approach. We developed a new method, "direct approach to the anterior and lateral part of the maxillary sinus with an endoscope" (DALMA), that negates the need for a sublabial incision and minimizes dental paresthesia by reducing the risk of anterior superior alveolar nerve injury. This study aimed to describe how to perform the DALMA technique, and to review its effectiveness and associated complications., Methods: We retrospectively reviewed 10 patients who had received DALMA., Results: Ten patients underwent DALMA. The anterior superior alveolar nerve was identified in all patients. Access to the lateral side beyond the infraorbital canal and anterior wall of the maxillary sinus was achieved., Conclusions: DALMA is a simple, effective, and minimally invasive technique that can be used as an alternative to CL with sublabial incision., (Copyright © 2019 Elsevier B.V. All rights reserved.)

Objective: The role of endonasal endoscopic approach for pathologies in the paediatric population is evolving and has still not been accepted as standard of care in neurosurgery. It represents a challenge in terms of narrow access, instrument manipulation and adequate reconstruction of defects. We have described our experience in 49 cases from a single neurosurgical unit in paediatric skull base surgeries through this approach over the last 12 years., Material and Methods: A case series of 59 paediatric skull base surgeries in 49 children through endoscopic endonasal route over the last 12 years is presented. The age ranges from 4 months to 18 years. Out of 49 cases, 22 cases were of craniopharyngiomas, 8 cases of pituitary adenomas, 5 cases with CSF rhinorrhea, 5 cases with meningoencephalocele, 3 cases of Rathke's cleft cysts, 2 cases of odontoidectomy and 4 miscellaneous cases viz. mucocele, hypothalamic glioma, esthesioneuroblastoma and epidermoid. CSF leaks were repaired with free graft in the initial years and by vascularized flap more recently., Results: The goal of surgery was achieved in all but two cases in whom the tumour excision was unsatisfactory due to failure of the cyst wall to collapse after decompression. Extent of tumour excision was not compromised by the choice of this approach. Revision surgery for CSF leak was required in three patients. Local vascularized nasoseptal flap has been possible even in very young patients and has now become the standard for reconstruction., Conclusion: In spite of the challenges posed by small nostrils and ill-developed sinuses in the paediatric age group, surgery from endoscopic endonasal corridor is possible to be carried out successfully in selected cases.

Secondary spinal manifestations of esthesioneuroblastoma are rare. A 67-year-old woman was presented with an extradural spinal manifestation at the vertebra Th7 within 8 weeks after resection of an esthesioneuroblastoma. Subtotal removal of the epidural tumour was achieved combined with dorsal transpedicular stabilization. Early screening for distant metastases may be considered in patients with esthesioneuroblastoma.

Background: Endoscopic endonasal approaches have been shown to be as effective as transcranial approaches for sinonasal malignancies involving the skull base. Although they are associated with less risk than an open cranial resection, cerebrospinal fluid (CSF) leaks remain a main concern. Some drugs can raise the intracranial pressure and exacerbate the risk of a postoperative CSF leak. In this case report, we present a 32-year-old woman who was treated via endoscopic endonasal approach for an olfactory neuroblastoma and later underwent fertility preservation treatment with leuprolide, which likely led to a delayed CSF leak., Case Description: A 32-year-old woman diagnosed with a large invasive olfactory neuroblastoma underwent resection via an endonasal transcribriform approach and repair of skull base defect using a pericranial flap. Forty-seven days post operation the patient returned, presenting with a clear nasal discharge. Magnetic resonance imaging raised concern for focal flap necrosis, while computed tomography cisternogram revealed a defect in the pericranial flap. The defect was debrided and repaired; a postoperative lumbar drain was placed and showed an opening pressure of 26 cm of water. Discussion with the patient revealed that she received fertility preservation treatment with leuprolide followed by oocyte retrieval 32 days before presentation of her delayed CSF leak., Conclusions: Leuprolide or any drug that can potentially increase intracranial pressure should be held for 3 months after surgery or until after a skull base defect has fully healed., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Esthesioneuroblastoma is a rare neoplasm arising from the neural crest cells of olfactory epithelium mostly in the nasal vault. We describe the Ga DOTANOC PET/CT findings of a 44-year-old woman who was operated for right nasal mass diagnosed as paraganglioma in the past and then develop a mass in the right nasal cavity after 2 years which upon surgery diagnosed to be esthesioneuroblastoma on histopathology.

Background: Endoscopic endonasal anterior skull base malignant sinonasal tumor resection and reconstruction remains a challenge. We describe our septal flip flap (SFF) reconstruction, a new surgical technique for repairing anterior skull base defects and report our outcomes., Methods: We retrospectively reviewed the clinical data of 24 patients who underwent skull base reconstruction using a SFF following endoscopic resection with transnasal craniectomy. We raise the SFF from the contralateral nasal septum based on the septal branches of the anterior and posterior ethmoidal arteries; the SFF is then rotated laterally for anterior skull base reconstruction after transnasal resection with craniectomy., Results: The SFF was used for multiple tumor types including, most commonly, intestinal-type adenocarcinoma, followed by olfactory neuroblastoma, squamous cell carcinoma, sinonasal undifferentiated carcinoma, and other types. All of the cases had either preoperative or postoperative radiation therapy. All flaps remained viable postoperatively. Postoperatively, nasal crusting was significantly reduced with faster healing of the surgical cavity., Conclusions: The SFF adds to the clinical armamentarium the opportunity to provide vascularized mucosal coverage extending from the frontal recess back to the planum sphenoidalis. The use of SFF requires careful consideration when dealing with paranasal sinus cancers so as not to infringe oncologic principles., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Introduction: The authors describe their experience of surgical closure of the anterior skull base after tumour resection, using the posterior wall of the frontal sinus., Material and Method: The authors describe their anterior skull base closure technique performed in three patients after tumour resection. Tumour resection via a transglabellar approach resulted in an anterior skull base defect. Reconstruction consisted of direct implantation of the posterior wall of the frontal sinus without using a bone substitute (except when nasofrontal duct obstruction is required)., Results: Three patients were operated by this surgical procedure with complete tumour resection in every case and no infectious complications. This technique was easy to perform, despite one case of persistent CSF leak. Follow-up imaging showed no displacement of the onlay bone graft., Conclusion: Anterior skull base reconstruction after tumour resection using autologous frontal sinus bone graft is easy to perform with a low complication rate., (Copyright © 2019 Elsevier Masson SAS. All rights reserved.)

Esthesioneuroblastoma is an uncommon tumour, and isolated primary involvement of the maxillary sinus is exceedingly rare. Esthesioneuroblastoma has infrequently been reported as a source of paraneoplastic ectopic hormone production. We report a case of isolated primary maxillary esthesioneuroblastoma, presenting as idiopathic syndrome of inappropriate antidiuretic hormone (SIADH). A 17-year-old girl presented with symptoms consistent with SIADH and no sino-nasal symptoms. MRI to exclude pituitary tumour revealed an isolated lesion of the right maxillary sinus. Biopsy demonstrated esthesioneuroblastoma. The lesion was removed endoscopically as a single en bloc specimen. Following resection, the sodium level returned to normal. This is only the third report in the literature of a primary maxillary esthesioneuroblastoma presenting as SIADH., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2019. No commercial re-use. See rights and permissions. Published by BMJ.)

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.)