Olfactory neuroblastoma is a rare sinonasal malignancy arising from the olfactory epithelium that is characterized by skull base involvement and a modest natural history. Because of its rarity and long course, identification of independent prognostic factors is dependent on multivariate analysis of large, long-term data. In this review, we outline evidence for the evaluation and treatment of olfactory neuroblastoma obtained from recent large-scale population-based studies, meta-analyses and multicenter studies. Hyams grade is currently the only pathological grade system for olfactory neuroblastoma. The modified Kadish staging and Dulguerov classification are available for clinical staging. The results of large-scale studies have confirmed Hyams, the modified Kadish and Dulguerov as independent prognostic factors. Surgery followed by radiotherapy provides the best overall survival and recurrence-free survival for resectable disease. The question of whether postoperative radiotherapy should be administered for all cases or only for those at risk of recurrence remains unanswered. Exclusively endoscopic resection is indicated for modified Kadish A/B cases without any increase in the risk of death or recurrence, and is also indicated for modified Kadish C cases if a negative surgical margin is ensured. For more advanced cases, such as those with extensive brain infiltration, the open approach is indicated. Elective nodal irradiation prevents late nodal recurrence of N0 patients. Chemotherapy has failed to show a benefit in survival or disease control. Current needs for olfactory neuroblastoma include the development and validation of refined staging systems suitable for current practice; expansion of indications for endoscopic surgery; less invasive surgery; definitive radiotherapy and novel systemic therapy.

Fish maintained in managed care may have longer lifespans as a result of advances in veterinary medicine and husbandry and reduced risk of predation. Neoplasia is of increasing interest in managed aquarium populations. However, few studies have systematically evaluated neoplasia in managed fish populations. Our objective in this retrospective study was to review and describe neoplasia diagnosed in fish at a large public display aquarium between 2005 and 2021. Any fish diagnosed with neoplasia on either antemortem or postmortem evaluation during the study period was included, and all medical records, biopsy, and autopsy reports were reviewed. Sixty-two fish met the inclusion criteria; 37 species were included in the study population, most of which were tropical freshwater fish (n = 34 fish). Thirty-two types of neoplasia were identified. Ten fish had benign neoplasms, and 53 fish had malignant neoplasms. The most common neoplasms were of epithelial and neuroectodermal origin. The most common site of tumor origin was the skin. Our data suggest that mesenchymal neoplasms may be more common in cold saltwater fish than in tropical freshwater and saltwater fish. Malignant neoplasms were most commonly diagnosed in the study population and should be a top differential when neoplasms are identified in fish managed under human care. Our study contributes to the overall knowledge of the health of aquarium fish and may aid clinicians in characterizing neoplasia that may be present in fish under human care.

Esthesioneuroblastoma is a rare malignant tumor developing from the olfactory neuroepithelium. It represents less than 5% of all cancers of the nasal cavity. We are going to report the observation of a patient followed at the regional oncology center of Oujda in Morocco who presented a locally advanced esthesioneuroblastoma. Treatment consisted of surgical resection followed by adjuvant radiotherapy on the tumor bed. Currently, the patient is in good control of his disease.

Objectives  Ectopic olfactory neuroblastoma is an uncommon manifestation of an already rare neoplasm. We aimed to systematically review the literature for cases of ectopic olfactory neuroblastoma to better characterize this rare disease entity and to present two new case reports. Methods  A search of the PubMed and Embase databases was conducted according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines to identify English-language articles reporting cases of ectopic olfactory neuroblastoma, published from 1955 through November 2021. Results  Sixty-six cases of ectopic olfactory neuroblastoma were identified in 62 articles including the current review. Ectopic olfactory neuroblastoma arose in a wide age range (2-89 years) without significant sex predilection. It occurred most commonly in the ethmoid (25%), maxillary (25%), and sphenoid (16%) sinuses. Seventy-three percent of cases presented with low Hyams grade (I and II). The most common symptoms were nasal obstruction (32%) and epistaxis (32%). Paraneoplastic syndromes were observed in 27% of patients. The most common treatment was surgical resection followed by adjuvant radiotherapy. Overall, 76% of all patients were disease-free at the time of last follow-up. Locoregional recurrences and distant metastases were found in 19 and 5% of cases, respectively. Conclusion  This systematic review describes previously reported cases of ectopic olfactory neuroblastoma, a disease entity with poorly understood characteristics. Physicians should consider olfactory neuroblastoma in the differential diagnosis for sinonasal masses, as their ectopic presentation may present considerable diagnostic and therapeutic difficulties. Patients with olfactory neuroblastoma may benefit from long-term follow-up and routine endoscopic examinations for surveillance of ectopic recurrences.

Ectopic olfactory neuroblastoma (ONB) is a rare neuroendocrine sinonasal malignancy which arises from sinonasal regions where olfactory neuroepithelium does not exist. Presentation of syndrome of inappropriate antidiuretic hormone release (SIADH) in patients with ectopic ONB is extremely rare. We report a case of a 22-year-old patient with ectopic ONB and paraneoplastic SIADH that was managed at our center. The ONB was arising from the left lamina papyracea and extending into the maxillary and ethmoid sinuses and filling the nasal cavity. Correction of sodium levels occurred within 24 hours of endoscopic resection via a combined trans-nasal transorbital approach. To avoid negative sequela secondary to rapid sodium correction, the patient was managed by desmopressin to obtain gradual sodium correction, which was achieved on the fourth postoperative day. Ectopic presentation of ONB with paraneoplastic SIADH is extremely rare with only 7 cases reported in the literature to date. Management via surgical resection and/or radiotherapy for these patients requires the managing clinician to be vigilant of serum sodium levels. A multidisciplinary approach is essential for optimal outcomes.

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.
A systematic review and proportion meta-analysis were conducted following PRISMA guidelines based on PubMed, Web of Science, and Scopus.
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%).
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.

OBJECTIVE: Schwannomas are common nerve sheath tumors and may occur anywhere in the body. 4% of head and neck schwannomas occur in the sinonasal cavity, and fewer yet have an intracranial component, making these presentations extremely rare. Furthermore, schwannomas present with nonspecific imaging signs and can only be definitively differentiated via histopathologic review, leading to misdiagnosis as various nasal tumors. We aim to conduct a review of published literature on sinonasal schwannomas with and without intracranial extension and provide additional case representations.
METHODS: A literature review was conducted using the PubMed Database with the terms \"sinonasal schwannoma,\" \"intracranial,\" \"anterior skull base,\" and \"schwannoma.\" Results were reviewed, and additional cases identified were referenced and included in the study. Inclusion criteria were any case with intracranial extension of the schwannoma. There were no exclusion criteria. Review data was compiled into Excel and used for data analysis and comparison. Additionally, a search was done within our institution to identify additional cases of sinonasal schwannoma.
RESULTS: We identified 17 cases of sinonasal schwannoma with intracranial extension, five from our institution and twelve from literature. Analysis revealed: 8 females (47%), 9 males (53%), 9 patients presented with headaches (53%), 6 patients presented with anosmia (35%), 4 patients presented with nasal obstruction (24%), and 2 patients with no symptoms (12%). Mean age and median were 39.4 ± 10.1 and 40, respectively. For treatment, 4 patients underwent endoscopic resection (24%), 11 underwent craniotomy (65%), and data was unavailable for 2 patients. Post-treatment complications occurred in 6 patients, 5 had CSF leaks (29%) and 1 had a hematoma (6%).
CONCLUSIONS: We identified and discussed 17 cases of sinonasal schwannoma with intracranial extension. We hope our review provides insight for clinicians to maintain schwannoma as a potential differential when evaluating nasal and anterior skull base masses.

Background Esthesioneuroblastoma (ENB) is a rare, aggressive and malignant sinonasal tumour. This tumour makes for a very interesting study because ever since its mention in medical literature, although quite a lot of research has been put into it, a lot is yet to be known about it. Aim To summarize the available literature & document an article on the contemporary practises in the management of ENB. Methods A comprehensive literature review was done using Google Scholar and PubMed database with the key words: \"esthesioneuroblastoma\", \"olfactory neuroblastoma\", \"Kadish\" and \"Hyams\". Articles published in English were used. Articles published till April 2021 were reviewed. Conclusion ENB has a diverse histological appearance. This tumour has a bimodal age distribution without any gender predilection. Prompt identification and treatment is required for a favourable outcome and requires close collaboration of radiology, pathology, and rhinology. Staging, management and prognosis are determined by regional involvement in non-metastatic cases due to its proximity to neurological structures and locally destructive nature. Immunohistochemistry is a must for accurate diagnosis. Uni-modality of treatment has high chances of local recurrence. Multi-modality provides good results even in advanced stage. A combination of surgery and radiotherapy seems to be the optimum approach to treatment. The exact role of chemotherapy in treatment protocols is still unclear. Cervical lymph node metastases are commonly studied, however its management in patients who present without apparent nodal involvement is not yet clear. An extended follow-up period is recommended in all patients of ENB patients.

Esthesioneuroblastoma (ENB) is a rare sinonasal malignant neoplasm with 40% 5-year survival. Because of the rarity of the tumor, the optimal treatment and subsequent prediction of prognosis are unclear. We studied a modern series of patients with ENB to evaluate the association of immunohistochemical (IHC) markers and clinical stages/grades with outcomes.
A single-center retrospective review of patients with ENB treated during a 25-year period was performed. A systematic literature review evaluating the prognostic benefits of current staging systems in evaluating survival outcomes in ENB was undertaken.
Among 29 included patients, 25 (85%) were treated surgically at our institution, with 76% of those endoscopically resected; 7 (24.1%) received chemotherapy, and 18 (62.1%) received radiation therapy. The 5-year overall survival (OS) was 91.3%, and 10-year OS was 78.3%. Progression-free survival at 5 and 10 years was 85.6% and 68.2%, respectively. A total of 36 distinct IHC markers were used to diagnose ENB but were inconsistent in predicting survival. A systematic literature review revealed predictive accuracy for OS using the Kadish, TNM, and Hyams staging/grading systems was 68%, 42%, and 50%, respectively.
This study reports the 5- and 10-year OS and progression-free survival in a modern series of patients with ENB. No traditional IHC marker consistently predicted outcome. Some novel reviewed markers show promise but have yet to enter clinical mainstream use. Our systematic review of accepted staging/grading systems also demonstrated a need for further investigation due to limited prognostic accuracy.

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.
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.
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.
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.