Search

Background: Aim of this study is to investigate the prevalence of cervical nodal metastasis at presentation and as disease relapse in primary, treatment-naive olfactory neuroblastoma (ONB), and to review treatment modalities, risk factors for regional failure and survival outcomes according to nodal status., Methods: A systematic review and proportion meta-analysis were conducted following PRISMA guidelines based on PubMed, Web of Science, and Scopus., Results: Eighteen articles were examined. The pooled proportion of patients with nodal metastasis at presentation (11.5%) was comparable to that of cN0 patients not receiving elective neck treatment developing nodal metastasis during follow-up (12.3%). Of the latter, most were Kadish stage C tumors (85.5%)., Conclusions: Cervical involvement is common both at presentation and during follow-up of cN0 ONB. The highest risk of developing late nodal metastasis is seen in cN0 patients with Kadish stage C tumors not receiving elective neck treatment. Elective cN0 neck treatment should be encouraged in selected patients to increase regional control., (© 2023 Wiley Periodicals LLC.)

Background: Non-squamous cell carcinoma sinonasal malignancies (NSCCSM) are relatively rare. Neoadjuvant radiotherapy and/or chemotherapy (NTx) have been proposed to improve outcomes compared to surgery alone. In this study, we aim to examine the prevalence of NTx utilization and associated outcomes., Methods: A retrospective study utilizing the National Cancer Database, 2004 to 2015. The study population included adult patients diagnosed with primary NSCCSM., Results: A total of 574 patients were included. The mean age of the study population was 61.7 ± 16.5 years. The median follow-up time was 40.4 months (interquartile range: 15.3-81.3 months). The histopathological diagnoses identified included: (i) 37.0% adenocarcinoma, (ii) 22.8% adenoid cystic carcinoma, (iii) 20.0% mucosal melanoma, (iv) 11.9% esthesioneuroblastoma, and (v) 8.2% sinonasal undifferentiated carcinoma (SNUC). NTx was utilized in 70 (12.20%) of the study population. Patients who received NTx were more likely to have SNUC or esthesioneuroblastoma ( P  < .01 each) and to have stage III or IV disease ( P  < .01 each). NTx was most likely to be administrated in a high-volume center [OR: 3.94, 95%CI: (1.47, 10.53), P  = .006]. Patients who received NTx had a significantly lower prevalence of positive margin postoperatively [OR: 0.48, 95%CI: (0.26, 0.87), P  = .016]. In patients with NSCCSM, negative margin was associated with improved overall survival [HR: 0.55, 95%CI: (0.36, 0.82), P  = .004]., Conclusions: This study provides an epidemiological perspective regarding NSCCSM and related practice patterns and survival outcomes. Neoadjuvant radiotherapy and/or chemotherapy is likely to decrease the risk of positive margin which ultimately could improve survival in this population.

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.

Background: Long-term follow-up in clinical reviews often presents significant difficulty with conventional medical records alone. Publicly accessible genealogical databases such as Ancestry.com provide another avenue for obtaining extended follow-up and added outcome information. No previous studies have described the use of genealogical databases in the follow-up of individual patients., Methods: Ancestry.com, the largest genealogical database in the United States, houses extensive demographic data on an increasing number of Americans. In a recent retrospective review of esthesioneuroblastoma patients treated at our institution, we used this resource to ascertain the outcomes of patients otherwise lost to follow-up. Additional information such as quality of life and supplemental treatments the patient may have received at home was obtained through direct contact with living relatives., Results: The use of Ancestry.com resulted in a 25% increase (20 months) in follow-up duration as well as incorporation of an additional 7 patients in our study (18%) who would otherwise not have had adequate hospital chart data for inclusion. Many patients within this subset had more advanced disease or were remotely located from our institution. As such, exclusion of these outliers can impact the quality of subsequent outcome analysis., Conclusion: Online genealogical databases provide a unique resource of public information that is acceptable to institutional review boards for patient follow-up in clinical reviews. Utilization of Ancestry.com data led to significant improvement in follow-up duration and increased the number of patients with sufficient data that could be included in our retrospective study., (© 2016 ARS-AAOA, LLC.)

Objective: Olfactory neuroblastoma and the management of neck disease has posed considerable challenges to the treating physician. The aims of the study were to determine the incidence and factors influencing neck disease and to identify at-risk patients with cervical node-negative disease at presentation., Study Design: Multicenter case series with retrospective chart review., Setting and Subjects: In sum, 113 patients with a histopathologic diagnosis of olfactory neuroblastoma across 6 tertiary hospitals in Australia and the United States., Methods: Treatment modalities for the primary site and neck included surgery, radiotherapy, and combined therapy. Treatment outcomes were measured in relation to date of primary treatment, and long-term follow-up was recorded. Disease-free survival was calculated as time for patients to develop delayed neck disease following primary treatment., Results: A total of 113 patients (46 females, 49.7 ± 13.2 years) were identified with a median follow-up of 41.5 months (interquartile range, 58.2); 7.1% of patients presented with primary neck disease, while 8.8% of patients presented with delayed neck disease. Neck disease was present in patients with Hyams grade II (22.2%), III (55.6%), and IV (22.2%) lesions (χ(2) = 5.66, P = .13). Histologic grade was higher in patients with primary neck disease (χ(2) = 16.22, P = .001). Positive surgical margins were associated with a higher risk of delayed neck disease as compared with clear surgical margin (17.9% vs 5%, P = .034)., Conclusion: Neck metastasis is an important clinical consideration for olfactory neuroblastoma at presentation and in surveillance. Primary treatment of the neck could be considered in select patients. Long-term surveillance of the neck and primary site is essential., (© American Academy of Otolaryngology—Head and Neck Surgery Foundation 2015.)

Objective: To determine if sex independently affects presentation and disease-specific survival (DSS) in patients with esthesioneuroblastoma (ENB)., Study Design: A case-control study from the Surveillance Epidemiology and End Results (SEER) data base., Methods: The assessment identified 611 patients in the SEER data base who were diagnosed with ENB from 1988 to 2010. Data on race, ethnicity, age at diagnosis, sex, histologic grade, radiation treatment status, and surgical treatment status of patients with ENB from 1988 to 2010 were extracted. By using tumor extension data, the modified Kadish stage of each case was determined. The modified Kadish system was able to successfully classify 547 of 611 tumors from 1988 to 2010. Histologic grade, modified Kadish stage and DSS of male patients was compared with the DSS of female patients., Results: Demographic data showed that male patients presented with a significantly higher grade (p < 0.05) and a trend toward a higher stage (p = 0.08). With unmatched data, male patients had significantly worse DSS than female patients (p < 0.05). After case-matching, the difference between the DSS for male versus female patients was no longer significant., Conclusions: Male patients with ENB seemed to have significantly worse DSS at 10 years than female patients. This disparity seems to be due to higher grade and stage in male patients at presentation. After accounting for these two factors, the prognosis of male patients was not found to be significantly different from that of female patients.

Esthesioneuroblastoma (EN) is a rare sinonasal tumor with varied aggressiveness and potential for intracranial invasion. EN is staged anatomically with radiographic evaluation using the Kadish staging system (stages A, B, and C) and histologically by using Hyam's criteria (grades 1-4). Here we show that despite radiographic evidence of aggressive features, the prognosis of patients with Kadish stage C EN is best predicted by tumor histology using Hyam's criteria. We retrospectively analyzed patients with EN with Kadish stage C who were evaluated and treated at our institution between 1995 and 2009. Clinical information was collected using patient medical records, imaging, and review of pathological specimens. Twenty patients with Kadish stage C EN were identified with mean age of 51 years (31-70 years) with a median follow-up of 41.4 months (1.3-175 months). Upon pathological review, 44.4% of patients had low-grade (1/2) and 55.6% had high-grade (3/4) histology. About 37.5% of patients with low-grade EN had undergone gross total resection (GTR) and the remaining 62.5% had GTR and adjuvant radiation, whereas 50% of patients with high-grade ER had undergone GTR, 20% had undergone GTR and adjuvant radiation, and 30% had been treated with a subtotal resection (STR) and adjuvant radiation. The 5-year and 10-year survival in patients with low-grade EN was 86% in comparison to 56% and 28% with high-grade EN, respectively. In patients with low-grade EN, the 2-year progression free survival (PFS) was 86% and the 5-year PFS was 65% in comparison to 73% and 49% in patients with high-grade EN, respectively. The patient's tumor histology (Hyam's criteria) appeared to be the best way of predicting the prognosis and for selecting patients for adjuvant radiotherapy., (Copyright © 2012 Elsevier Ltd. All rights reserved.)

Esthesioneuroblastoma is a rare malignant neoplasm in the olfactory region of the nasal cavity and anterior skull base. Diagnosis and staging require anatomic imaging and careful pathologic assessment. Standard treatment is anterior craniofacial resection with postoperative irradiation. The role for chemotherapy is not defined, but is generally for the most advanced cases and used in the neoadjuvant setting and/or postoperatively with irradiation. Prognosis is favorable; however, metastasis rates remain relatively high. Regional and distant metastasis portends a poor outcome. Intensity-modulated radiation treatment and endoscopic surgery have reduced morbidity, but outcomes with these techniques must be fully evaluated., (Copyright © 2013 Elsevier Inc. All rights reserved.)

Esthesioneuroblastoma (ENB) is a rare malignancy, representing only 3% to 6% of all sinonasal malignancies. A wide array of treatment options for ENB have been described in the literature, but prospective clinical trials are absent given the tumor's rarity and natural history. Delay in diagnosis leading to an initial advanced stage of presentation is common secondary to the clinically hidden primary site at the anterior skull base. This article presents data from the current body of literature and reviews the advocated roles for surgery, radiation therapy and chemotherapy., (Copyright © 2012 Elsevier Inc. All rights reserved.)

Several naphthalene Unit Risk Factors (URFs) were proposed by the US Environmental Protection Agency in 2004 using data on the development of olfactory epithelial neuroblastomas and nasal respiratory epithelial adenomas in rats, but these URFs may be inappropriate and unnecessarily conservative for estimating human cancer risks. The purpose of the present exercise was to perform a screening-level population risk assessment of the US population to compare the observed number of naphthalene-induced nasal tumors in the US to the number that would be predicted if the URFs for naphthalene were as proposed. Nine scenarios were evaluated to represent the range of exposures individuals have typically experienced. Results indicate that the total predicted burden of naphthalene-induced nasal tumors per year in the US (65,905 rare nasal tumors, of which 29,121 are olfactory epithelial neuroblastomas) is much greater than the number of these tumors actually observed per year (910 total nasal tumors, of which 66 are olfactory neuroblastomas) over the period 1973-2006. This suggests that using rat nasal tumor data to derive a naphthalene URF for humans should be re-evaluated., (Copyright 2010 Elsevier Inc. All rights reserved.)

Objective: The purpose of this work was to investigate the clinical manifestations and diagnostic range of malignant entities presenting as a nasal mass in the pediatric population., Patients and Methods: A retrospective cohort analysis was conducted at a specialty hospital and a tertiary care university hospital. Patients aged between birth and 18 years and diagnosed with a malignancy that arose within the nasal cavity between the years 1991 and 2006 were included. This institution-specific patient group was compared with a similar cohort of patients extracted from the national Surveillance Epidemiology and End Results database. The main outcome measures were the incidence, presentation, and diagnoses of nasal cancer presenting in this population., Results: Sixteen patients with nasal malignancies presented institutionally in the defined pediatric age group. Patient age at the time of diagnosis ranged from 7 months to 17 years, with a slight male predominance. The main presenting symptoms were unilateral nasal congestion and ophthalmologic complaints. The median time from presentation to diagnosis was 7 weeks; patients who presented with nonspecific complaints, such as nasal obstruction, headache, and fatigue, were given a diagnosis, on average, later than those who presented with focal manifestations. Nationwide, 47 patients were identified from the Surveillance Epidemiology and End Results database. In both subject groups, the most common diagnoses were rhabdomyosarcoma (37.5% institutionally and 23% in the Surveillance Epidemiology and End Results group) and esthesioneuroblastoma (25% institutionally and 28% Surveillance Epidemiology and End Results). In the Surveillance Epidemiology and End Results cohort, the overall mean survival rate was 188 months., Conclusions: Nasal cancer in the pediatric population often presents with nonspecific signs and symptoms, and a high index of suspicion is necessary for a timely diagnosis. Soft tissue sarcomas are expectedly common. The relative high frequency of esthesioneuroblastoma is particularly noteworthy.

Conclusions: The progression of olfactory neuroblastoma showed a biphasic pattern. As well as Hyams' histopathological grading and neck metastasis at presentation, early phase recurrence should be regarded as an important prognosticator. A high local failure rate suggests that craniofacial resection followed by postoperative radiotherapy should still be the standard treatment for olfactory neuroblastoma., Objective: The aim of this study was to evaluate factors associated with survival and local control of olfactory neuroblastoma in the long run and to estimate treatment strategies., Patients and Methods: Twelve patients (seven men and five women) who had undergone initial curative treatment for olfactory neuroblastoma were retrospectively analyzed., Results: Cause-specific 10-year survival was 64.8%, while disease-free 10-year survival remained 28.6%. Local failure was found in half of the patients. All of the three patients who did not receive radiotherapy developed local failure. A biphasic pattern of recurrence was observed. The early phase recurrence group showed a significantly poorer survival than the late phase recurrence group. Hyams' histopathological grading and neck metastasis at presentation were also correlated with survival.

In the present study we review ENT tumor pathology in childhood. Only the most salient aspects are emphasized and the variety of entities reviewed was restricted. Molecular biology techniques reveal infection by human papilloma virus (types 6 and 11) in 50 % of papillomas, while immunohistochemical techniques are less effective in papilloma virus detection. The myofibroblastic nature of nasal angiofibroma has been demonstrated and its incidence is 25 times more frequent in patients with familial polyposis of the colon. Overexpression of p53 occurs in the initial stages of nasopharyngeal carcinoma, while overexpression of c-myc is correlated with an unfavorable prognosis. Recently, olfactory neuroblastoma has been shown not to express the protein product of the MIC-2 gene (antibody 12E7), thus the hypothesis that it could be a member of the Ewing tumor family (neuroectodermal peripheral tumors) has not been confirmed, although it is a primitive neural tumor. The head and neck rhabdomyosarcoma with the best prognosis is that located in the orbit, and cytogenetic studies have shown chromosomic translocation t(2;13) in 50 % of these childhood tumors when they are of the alveolar-type, while trisomy of chromosome 2 or 20 is more characteristic of the embryonic-type. Currently, any classifying features of ENT lymphomas must be based on the Revised European-American Classification of Lymphoid Neoplasms (REAL). Papillary and medullary carcinomas are the most common histological types of thyroid carcinoma in childhood. Alterations in ret/PTC play a significant role in the pathogenesis of both.

Objective: A retrospective review of all diagnosed cases of esthesioneuroblastoma registered in Denmark between 1978 and 2000 was carried out in order to obtain epidemiological data and optimize national treatment guidelines., Material and Methods: Forty cases were verified histologically and included in the analysis Epidemiological and histopathological data were evaluated in relation to the clinical outcome., Results: The 40 cases represent an incidence rate of 0.4 cases/million inhabitants per year. Eight (20%) patients were classified as Kadish stage A, 13 (32.5%) as stage B and 19 (47.5%) as stage C. The histopathological findings were classified according to the grading system of Hyams The median follow-up time was 2.3 years (range 0.3-11.1 years). The 5-year crude survival rate was 61%, with a median survival of 3.1 years (range 0.3-19.2 years). The 5-year disease-free survival rate was 50%, with a median survival of 1.7 years (range 0-19.2 years). Only 3 (7%) patients had positive cervical lymph nodes at presentation. A nationwide consensus regarding treatment was seen in patients classified as Kadish stages A and B. The longest duration before the first recurrence of esthesioneuroblastoma was 5(1/2) years., Conclusions: The following therapeutic guidelines are suggested: Kadish stage A patients, surgical tumour resection and radiotherapy; Kadish stage B, surgical tumour resection and radiotherapy; Kadish stage C, surgical tumour resection via a craniofacial resection and radiotherapy combined with chemotherapy. Long-term follow-up of esthesioneuroblastoma patients is mandatory.

Aim: To evaluate the radiocurability of esthesioneuroblastoma, we have retrospectively analyzed the results of radiation therapy in this tumor in our clinic., Patients and Methods: From 1985 through 1990, twelve patients with esthesioneuroblastoma have been treated at the Department of Radiotherapy at the University of Erlangen. Two had Kadish stage A, one stage B, and nine stage C. There were seven males and five females with a mean age of 43 years. Eleven patients had combined transcranial-transbasal surgery prior to radiotherapy (five R0-, four R1-, and two patients R2-resections) and one received radiotherapy only. The dose was 12 to 60 Gy (mean 54 Gy) in 1.8 Gy to 2 Gy per fraction., Results: 8/12 patients (67%) were locally controlled. One had progressive disease during radiotherapy after partial resection and died. Three had local recurrences, one in-field and two marginal. One patient with a local recurrence developed cervical lymph node metastases, and one locally controlled patient developed bone metastases but is alive eight years after chemotherapy plus radiotherapy for metastatic disease. The five-year-overall and recurrence-free survival was 72% and 55%, respectively., Conclusions: Sophisticated surgery plus radiotherapy may cure a reasonable number of patients with esthesioneuroblastoma.

This paper describes the pathobiology of some of the more common skull base tumors. In addition to clinicopathologic features, emphasis is placed upon methods of diagnosis utilizing immunoperoxidase stains and molecular markers that may or may not impact upon prognosis.

Purpose: We describe our experience with adjuvant radiation therapy in patients who underwent operation for esthesioneuroblastoma., Methods and Materials: Between January 1951 and December 1990, 49 patients with esthesioneuroblastoma received their initial treatment at the Mayo Clinic. There were 27 male and 22 female patients; their ages ranged from 3 to 79 years (median, 54 years). The tumors were Kadish Stage A in 4 patients, Stage B in 13, Stage C in 29, and modified Kadish Stage D in three (cervical nodal or distant metastasis). The tumors were graded according to Hyams' classification. Treatment included gross total resection alone in 22 patients and gross total resection and postoperative adjuvant radiation therapy in 16. The patients treated with adjuvant radiation had a greater proportion of advanced-stage and high-grade tumors., Results: The 5-year actuarial overall survival, disease-free survival, and local control rates were 69.1% + 7.0%, 54.8% + 7.6%, and 65.3% + 7.4%, respectively. The only significant predictor for overall survival, disease-free survival, and local control was Hyams' grade. Local control was improved in patients who received postoperative adjuvant radiation even though this group of patients had more advanced and higher-grade tumors (5-year rate of local control was 85.9% + 9.3%, compared with 72.7% + 9.5% for those who had operation alone, p = 0.26)., Conclusion: Adjuvant radiation therapy for esthesioneuroblastoma improves local tumor control, particularly for high-grade and high-stage tumors. We recommend additional treatment with radiation (55.5 Gy) after complete resection of esthesioneuroblastoma.