-
Neuro-ophthalmology (Aeolus Press) 2022We report a case of 51-year-old medically healthy male who presented with gradual painless diminution of vision in both eyes for 3 months. His visual acuity was hand...
We report a case of 51-year-old medically healthy male who presented with gradual painless diminution of vision in both eyes for 3 months. His visual acuity was hand movement perception in the right eye, and no light perception in the left eye. The intraocular pressure, external eye examination, ocular motility and anterior segment examinations were normal. Fundus examination revealed severe bilateral papilloedema and engorged tortuous veins in both eyes. Imaging exhibited a large intracranial tumour causing raised intracranial pressure. This was debulked by neurosurgery and histological examination revealed that the tumour was an olfactory neuroblastoma (ONB). This case is noteworthy since ONBs rarely present with isolated papilloedema without any accompanying ophthalmoplegia, proptosis, epistaxis, nasal, or neurological symptoms.
PubMed: 35574165
DOI: 10.1080/01658107.2021.1958872 -
CMAJ : Canadian Medical Association... Aug 2021
Topics: Esthesioneuroblastoma, Olfactory; Female; Humans; Intensive Care Units; Middle Aged; Rhinorrhea; Terminal Care
PubMed: 34344779
DOI: 10.1503/cmaj.210463-f -
Journal of Clinical Medicine Apr 2022The aim of this study was to investigate the long-term oncologic outcome and review the state of the art in the management of olfactory neuroblastomas.
OBJECTIVE
The aim of this study was to investigate the long-term oncologic outcome and review the state of the art in the management of olfactory neuroblastomas.
MATERIAL AND METHODS
The records of all patients treated for olfactory neuroblastomas in two academic departments between 1975 and 2012 were evaluated retrospectively. Data on epidemiological parameters were collected (age, gender), along with staging (Kadish, Morita), histologic grading (Hyams), time and form of treatment, locoregional control, and disease-specific and overall survival. Patients with other malignant diseases, distant metastases of olfactory neuroblastomas at the time of initial diagnosis, a follow-up time of less than 5 years, or insufficient clinical-pathological data were excluded from further analysis.
RESULTS
In total, 53 cases made up our final study sample (26 men, 27 women; male-female ratio 0.96:1). Their mean age was 48.6 years (range: 10-84 years). The mean follow-up time was 137.5 months (4-336 months, SD: 85.0). A total of 5 out of 53 study cases (9.4%) showed metastatic involvement of the neck at the time of initial presentation. Local recurrence was detected in 8/53 (15.1%) and regional recurrence in 7/53 of our study cases (13.2%). Three patients (42.8%) from the group of cases with surgery as the sole form of management (7/53, 13.2%) died due to the disease. The cumulative disease-specific survival and overall survivalfor the whole group of patients were 88.6% and 63.6%, respectively. The cumulative disease-specific survival stratified by Kadish A/B vs. Kadish C/D as well as Hyams I/II vs. Hyams III/IV showed superior results for limited tumors, albeit without significance, and low-grade tumors (highly significant difference).
CONCLUSION
Craniofacial or sometimes solely endoscopically controlled resection can warrant resection of the olfactory neuroblastoma with wide margins. However, locoregional failures and distant metastases can occur after a long period of time. The non-negligible incidence of regional recurrences, partly in unusual localizations, leads us to consider the need to identify the "recurrence-friendly" cases and to perform individualized elective irradiation of the neck in cases with high-risk features.
PubMed: 35566413
DOI: 10.3390/jcm11092288 -
Journal of the Belgian Society of... 2024To evaluate the imaging characteristics of the tumor, emphasizing its location, and to determine the frequency of typical and atypical locations of olfactory...
OBJECTIVES
To evaluate the imaging characteristics of the tumor, emphasizing its location, and to determine the frequency of typical and atypical locations of olfactory neuroblastoma (ONB).
MATERIALS AND METHODS
We retrospectively reviewed the computed tomography and magnetic resonance imaging findings of patients with pathologically proven ONB between April 2000 and April 2023. Demographic information, chief complaints, tumor location, and tumor extension were extracted.
RESULTS
Of the 58 patients, 50 (86.2%) had the epicenter of the mass at the superior part of the nasal cavity, while eight patients (13.8%) had the epicenter at other atypical locations: seven patients (12.1%) at the middle part of the nasal cavity and one patient (1.7%) within both sphenoid sinuses.
CONCLUSION
ONB is not always present in the upper part or the roof of the nasal cavity, and a significant number of ONBs are occasionally found in the rest of the nasal cavity and other atypical locations.
PubMed: 38826683
DOI: 10.5334/jbsr.3562 -
Frontiers in Oncology 2020Tumors originating from the craniofacial region usually present in a locally advanced stage with frequent involvement of adjacent sites and have a strong tendency for... (Review)
Review
Tumors originating from the craniofacial region usually present in a locally advanced stage with frequent involvement of adjacent sites and have a strong tendency for local recurrence in the absence of adjuvant therapy, even when the original surgical resection was presumed to be radical. In the past decades, several advances in the radiological diagnosis and treatment of craniofacial malignancies have been introduced. There are, however, no randomized trials that define the optimal multimodal treatment of these tumors because of their rarity as well as heterogeneity in both histology and site of origin. The aim of this study was to conduct a critical review of the role of adjuvant therapy in the treatment of craniofacial malignancy. We conducted a critical review of the past and contemporary literature available, focusing on adjuvant oncological treatments of the most common craniofacial malignancies. Preoperative radiotherapy can have a documented role in the treatment of olfactory neuroblastoma and soft tissue sarcoma, while preoperative chemotherapy can be advocated in the treatment of sinonasal undifferentiated carcinoma, neuroendocrine carcinoma, olfactory neuroblastoma, and craniofacial sarcoma (both soft-tissue and high-grade osteosarcoma). Postoperative radiotherapy has a well-established role in the treatment of most craniofacial malignancies. The role of postoperative chemotherapy is unclear in most histologies, but is commonly used during the treatment of well-selected cases of paranasal sinus carcinoma, olfactory neuroblastoma, mucosal melanoma, soft tissue sarcoma and high-grade craniofacial osteosarcoma. Alongside developments in surgery, there have also been improvements in diagnostics, radiotherapy, and chemotherapy. Implementation of novel radiation techniques allows delivery of higher radiation doses while minimizing irradiation-related morbidity. Better understanding of tumor biology allows the construction of more complex treatment strategies, incorporating adjuvant chemotherapy either pre- or postoperatively. In the era of personalized targeted therapy, rapid strides are being made to identify specific tumor-targets for use of novel biologic agents, with the potential to change current management paradigms.
PubMed: 32850452
DOI: 10.3389/fonc.2020.01402 -
Ear, Nose, & Throat Journal Oct 2023Olfactory neuroblastoma (ONB) is a rare malignancy of the sinonasal cavity, originating from neuroepithelial olfactory cells. Olfactory neuroblastoma can be difficult to...
Olfactory neuroblastoma (ONB) is a rare malignancy of the sinonasal cavity, originating from neuroepithelial olfactory cells. Olfactory neuroblastoma can be difficult to diagnose due to its anatomic position and variable symptomatic presentation, leading to diagnosis at a more advanced stage. Here, we present the case of a 35-year-old man with no previous medical history who had a bicycle accident secondary to syncope. He was found to be hyponatremic and suspected to have syndrome of inappropriate antidiuretic hormone secretion (SIADH). In the workup of SIADH, a brain magnetic resonance imaging revealed a mass in the left middle meatus. The lesion secreted inappropriate amounts of ADH, resulting in symptomatic paraneoplastic SIADH. This ultimately led to the early recognition and successful resection of this rare ONB. The patient has remained disease-free for over 5 years. This case is a fortunate example of a functional malignancy of the sinonasal tract that was discovered early and successfully treated as a result of symptomatic SIADH.
Topics: Male; Humans; Adult; Inappropriate ADH Syndrome; Esthesioneuroblastoma, Olfactory; Nose Neoplasms; Nasal Cavity
PubMed: 34134537
DOI: 10.1177/01455613211022102 -
International Forum of Allergy &... Sep 2019Esthesioneuroblastoma (ENB), also known as olfactory neuroblastoma, represents up to 3% of all sinonasal neoplasms. Hyams histologic grading is a promising tool in... (Meta-Analysis)
Meta-Analysis Review
BACKGROUND
Esthesioneuroblastoma (ENB), also known as olfactory neuroblastoma, represents up to 3% of all sinonasal neoplasms. Hyams histologic grading is a promising tool in predicting metastases and establishing prognoses for this complex tumor.
METHODS
A systematic literature search was performed in the PubMed, Ovid MEDLINE, and Cochrane databases. ENB patients with Hyams I-II or III-IV were categorized as low-grade Hyams (LGH) or high-grade Hyams (HGH), respectively. Binary and continuous random-effects models were applied to calculate odds ratios (ORs) for the incidences of neck and distal metastases as well as for 5- and 10-year overall survival rates.
RESULTS
Of the 57 screened articles published from 1993 to 2018, 16 (525 patients) and 21 (563 patients) provided data for tumor metastases and overall survival rates, respectively. Neck metastasis was observed in 18.2% of HGH vs 7.9% of LGH patients. Distant metastasis was noted in 20.7% of HGH vs 8.9% of LGH patients. LGH patients had 5- and 10-year overall survival rates of 81.2% and 64.0%, respectively, as compared with 60.9% and 40.6%, respectively, for HGH patients. In comparing HGHs vs LGHs, the collective ORs for neck and distant metastases were 2.08 (95% confidence interval [CI], 1.09-3.99; p = 0.03) and 2.37 (95% CI, 1.07-5.26; p = 0.03), respectively. Moreover, in comparing LGHs vs HGHs, collective ORs for 5- and 10-year overall survival rates were 3.39 (95% CI, 2.09-5.49; p < 0.001) and 3.03 (95% CI, 1.82-5.06; p < 0.001), respectively.
CONCLUSION
HGH ENBs, compared with LGH ENBs, are more likely to metastasize to neck or distal targets and to have lower overall survival rates.
Topics: Esthesioneuroblastoma, Olfactory; Humans; Incidence; Models, Statistical; Nasal Cavity; Neoplasm Metastasis; Neoplasm Staging; Nose Neoplasms; Prognosis; Survival Analysis
PubMed: 31251848
DOI: 10.1002/alr.22373 -
Frontiers in Neuroscience 2022Dual specificity tyrosine-phosphorylation-regulated kinases (DYRKs) are a group of conserved eukaryotic kinases phosphorylating tyrosine, serine, and threonine residues.... (Review)
Review
Dual specificity tyrosine-phosphorylation-regulated kinases (DYRKs) are a group of conserved eukaryotic kinases phosphorylating tyrosine, serine, and threonine residues. The human DYRK family comprises 5 members (DYRK1A, DYRK1B, DYRK2, DYRK3, and DYRK4). The different DYRKs have been implicated in neurological diseases, cancer, and virus infection. Specifically, DYRK2 has been mainly implicated in cancer progression. However, its role in healthy and pathological nervous system function has been overlooked. In this context, we review current available data on DYRK2 in the nervous system, where the available studies indicate that it has key roles in neuronal development and function. DYRK2 regulates neuronal morphogenesis (e.g., axon growth and branching) by phosphorylating cytoskeletal elements (e.g., doublecortin). Comparative data reveals that it is involved in the development of olfactory and visual systems, the spinal cord and possibly the cortex. DYRK2 also participates in processes such as olfaction, vision and, learning. However, DYRK2 could be involved in other brain functions since available expression data shows that it is expressed across the whole brain. High DYRK2 protein levels have been detected in basal ganglia and cerebellum. In adult nervous system, DYRK2 mRNA expression is highest in the cortex, hippocampus, and retina. Regarding nervous system disease, DYRK2 has been implicated in neuroblastoma, glioma, epilepsy, neuroinflammation, Alzheimer's disease, Parkinson's disease, spinal cord injury and virus infection. DYRK2 upregulation usually has a negative impact in cancer-related conditions and a positive impact in non-malignant conditions. Its role in axon growth makes DYRK2 as a promising target for spinal cord or brain injury and regeneration.
PubMed: 36161154
DOI: 10.3389/fnins.2022.994256 -
Indian Journal of Otolaryngology and... Oct 2022Olfactory neuroblastoma (ONB) or esthesioneuroblastoma is a rare malignant intranasal tumor, commonly originated from upper part of nasal cavity. Majority of cases...
Olfactory neuroblastoma (ONB) or esthesioneuroblastoma is a rare malignant intranasal tumor, commonly originated from upper part of nasal cavity. Majority of cases presented with nasal obstruction or epistaxis. ONB is rarely reported in ectopic locations. Here we present the first-ever documented case of an olfactory neuroblastoma situated anterior to body of maxilla, presented as left sided facial swelling. This case report is aimed at achieving the consideration of this rare tumour as a differential diagnosis in the lesions of the anatomical region surrounding the commonly known site of origin i.e. the sinonasal cavity.
PubMed: 36452651
DOI: 10.1007/s12070-021-02595-9 -
Cancers Feb 2023Esthesioneuroblastoma (ENB) is a rare malignant neoplasm arising from the olfactory epithelium of the cribriform plate. Although survival is excellent with a reported...
INTRODUCTION
Esthesioneuroblastoma (ENB) is a rare malignant neoplasm arising from the olfactory epithelium of the cribriform plate. Although survival is excellent with a reported 5-year overall survival (OS) of 82%, recurrence is frequent and occurs in 40-50% of cases. This study investigates the characteristics of ENB recurrence and the subsequent prognosis of patients with recurrence.
METHODS
The clinical records of all patients diagnosed as having ENB with subsequent recurrence at a tertiary hospital from 1 January 1960 to 1 January 2020 were retrospectively reviewed. Overall survival (OS) and progression-free survival (PFS) were reported.
RESULTS
A total of 64 out of 143 ENB patients had recurrences. In total, 45 out of 64 recurrences met the inclusion criteria and were included in this study. From these, 10 (22%) had a sinonasal recurrence, 14 (31%) had an intracranial recurrence, 15 (33%) had a regional recurrence, and 6 (13%) had a distal recurrence. The average interval from initial treatment to recurrence was 4.74 years. There were no differences in rates of recurrence with respect to age, sex, or types of surgery (endoscopic, transcranial, lateral rhinotomy, and combined). The time to recurrence was shorter for Hyams grades 3 and 4 compared to Hyams grades 1 and 2 (3.75 years vs. 5.70 years, < 0.05). Patients with recurrence limited to the sinonasal region had a lower overall primary Kadish stage compared to recurrences beyond the sinonasal region (2.60 vs. 3.03, < 0.05). A total of 9 (20%) out of 45 patients developed secondary recurrence. Following recurrence, the subsequent 5-year OS and PFS were 63 and 56%, respectively. The mean time to secondary recurrence after treatment of the primary recurrence was 32 months, which was significantly shorter than the time to primary recurrence (32 months vs. 57 months, = 0.048). The mean age of the secondary recurrence group is significantly older than the primary recurrence group (59.78 years vs. 50.31 years, = 0.02). No statistically significant differences were observed between the secondary recurrence group and the recurrence group in terms of their overall Kadish stages or Hyams grades.
CONCLUSIONS
Following an ENB recurrence, salvage therapy appears to be an effective therapeutic option with a subsequent 5-year OS of 63%. However, subsequent recurrences are not infrequent and may require additional therapy.
PubMed: 36900297
DOI: 10.3390/cancers15051506