-
Cureus Sep 2023Background The aim of this study was to determine the histological diagnosis of unilateral nasal polyps and to determine the prevalence of neoplastic pathologies. This...
Background The aim of this study was to determine the histological diagnosis of unilateral nasal polyps and to determine the prevalence of neoplastic pathologies. This study also assessed difference in pathologies whether patients presented symptomatically or were asymptomatic (if they had a mass found incidentally for unrelated throat symptoms). Method This was a 10-year retrospective study of patients undergoing unilateral nasal mass surgery between 2004 and 2014 at a UK district general hospital. We recorded patient demographics, laterality, histology, symptoms, clinical suspicion, and imaging findings. Results 123 patients were included who underwent unilateral surgery between 2004 and 2014 (male n=83, female n=40; mean age 56 years ± 19.5). The majority were of inflammatory origin (n=92; 74.8%). The most common benign neoplastic cause was inverted papilloma (n=19; 15.4%). A number of malignant neoplastic causes were also found, including: melanoma (n=3; 2.44%), olfactory neuroblastoma (n=2; 1.63%), and other non-inflammatory masses (n=7; 5.69%). 15 of these masses were found incidentally, with 14 being inflammatory, and one an olfactory neuroblastoma; therefore, 6.67% of our incidental unilateral nasal masses were found to be of neoplastic pathology. Conclusion This study's findings support the continued practice of routine biopsy of unilateral nasal masses for histological diagnosis, irrespective of whether they are symptomatic or found incidentally. The accuracy of both clinical suspicion and radiological suspicion on CT scans is not adequate to alter this practice.
PubMed: 37790007
DOI: 10.7759/cureus.44526 -
Ear, Nose, & Throat Journal Sep 2023Ectopic olfactory neuroblastoma (ONB) is a rare neuroendocrine sinonasal malignancy which arises from sinonasal regions where olfactory neuroepithelium does not exist....
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.
PubMed: 37776288
DOI: 10.1177/01455613231199696 -
Exploring the Impact of Using Patient-Specific 3D Prints during Consent for Skull Base Neurosurgery.Journal of Neurological Surgery. Part... Oct 2023Informed consent is fundamental to good practice. We hypothesized that a personalized three-dimensional (3D)-printed model of skull base pathology would enhance...
Informed consent is fundamental to good practice. We hypothesized that a personalized three-dimensional (3D)-printed model of skull base pathology would enhance informed consent and reduce patient anxiety. Digital images and communication in medicine (DICOM) files were 3D printed. After a standard pre-surgery consent clinic, patients completed part one of a two-part structured questionnaire. They then interacted with their personalized 3D printed model and completed part two. This explored their perceived involvement in decision-making, anxiety, concerns and also their understanding of lesion location and surgical risks. Descriptive statistics were used to report responses and text classification tools were used to analyze free text responses. In total,14 patients undergoing elective skull base surgery (with pathologies including skull base meningioma, craniopharyngioma, pituitary adenoma, Rathke cleft cyst, and olfactory neuroblastoma) were prospectively identified at a single unit. After 3D model exposure, there was a net trend toward reduced patient-reported anxiety and enhanced patient-perceived involvement in treatment. Thirteen of 14 patients (93%) felt better about their operation and 13/14 patients (93%) thought all patients should have access to personalized 3D models. After exposure, there was a net trend toward improved patient-reported understanding of surgical risks, lesion location, and extent of feeling informed. Thirteen of 14 patients (93%) felt the model helped them understand the surgical anatomy better. Analysis of free text responses to the model found mixed sentiment: 47% positive, 35% neutral, and 18% negative. In the context of skull base neurosurgery, personalized 3D-printed models of skull base pathology can inform the surgical consent process, impacting the levels of patient understanding and anxiety.
PubMed: 37671293
DOI: 10.1055/a-1885-1111 -
The Indian Journal of Medical Research Jan 2023
Topics: Humans; Clinical Relevance; Esthesioneuroblastoma, Olfactory; Hyponatremia; Nasal Cavity; Nose Neoplasms
PubMed: 37602591
DOI: 10.4103/ijmr.ijmr_2064_21 -
Journal of Neurological Surgery Reports Jul 2023Esthesioneuroblastoma is a rare sinonasal malignancy that arises from the olfactory epithelium. The overall incidence of lymph node metastases is 25%. However, neck...
Esthesioneuroblastoma is a rare sinonasal malignancy that arises from the olfactory epithelium. The overall incidence of lymph node metastases is 25%. However, neck disease can present in a delayed fashion. As such, management of the clinically negative neck is controversial, with some advocating for elective neck treatment and others recommending observation with salvage treatment if necessary. At this time, no prospective head-to-head comparisons of elective versus salvage treatment have been performed.
PubMed: 37564630
DOI: 10.1055/s-0043-1770965 -
Ecancermedicalscience 2023Esthesioneuroblastoma (ENB) or olfactory neuroblastoma is a rare malignant neoplasm arising from the neural crest cells of the olfactory epithelium. The optimum...
Esthesioneuroblastoma (ENB) or olfactory neuroblastoma is a rare malignant neoplasm arising from the neural crest cells of the olfactory epithelium. The optimum treatment for this rare disease is still unclear. Most of the available literature on this rare head and neck tumour is limited to small retrospective series and single institutional reports. We conducted a retrospective study to investigate the clinical profile, treatment outcomes and prognostic factors of patients with ENB treated at a tertiary cancer centre in south India. Patients with a histopathological diagnosis of ENB treated from 2000 to 2019 were included. Patient demographics, tumour characteristics, stage, treatment details and outcome data were identified from medical records. Overall survival (OS) and disease-free survival (DFS) were estimated using the Kaplan-Meier method and the log-rank test was used for comparison. The prognostic factors were identified using Cox regression analysis. Forty-two patients underwent treatment for ENB from 2000 to 2019. Twenty-six patients underwent surgery. Twelve patients received radical radiotherapy (RT) while 24 patients underwent adjuvant radiation. After a median follow-up of 71 months, the estimated OS and DFS at 4 years were 64.4% and 54%, respectively. The estimated 4-year OS for modified Kadish A, B, C and D stages was 75.0%, 90.9%, 56.4% and 0%, respectively. Modified Kadish stage, nodal involvement, orbital invasion, intracranial extension, surgery, RT treatment and use of chemotherapy were significant predictors of OS and DFS in univariate Cox regression analysis. Orbital invasion and RT treatment were significant predictors of DFS in the multivariate analysis as well. However, only RT treatment came out to be a significant predictor for OS in multivariate Cox regression analysis. Surgery is the mainstay of treatment. Adjuvant RT may improve local control and survival in advanced cases. Advanced modified Kadish stage, lymph node involvement and orbital invasion are associated with poor outcomes.
PubMed: 37533955
DOI: 10.3332/ecancer.2023.1584 -
Oncogenesis Jul 2023The tumor suppressor and chromatin modifier cAMP response element-binding protein binding protein (CREBBP) and v-myc avian myelocytomatosis viral oncogene neuroblastoma...
The tumor suppressor and chromatin modifier cAMP response element-binding protein binding protein (CREBBP) and v-myc avian myelocytomatosis viral oncogene neuroblastoma derived homolog (MYCN), a member of the MYC oncogene family, are critically involved in brain development. Both genes are frequently mutated in the same tumor entities, including high-grade glioma and medulloblastoma. Therefore, we hypothesized that alterations in both genes cooperate to induce brain tumor formation. For further investigation, hGFAP-cre::Crebbp::lsl-MYCN mice were generated, which combine Crebbp deletion with overexpression of MYCN in neural stem cells (NSCs). Within eight months, these animals developed aggressive forebrain tumors. The first tumors were detectable in the olfactory bulbs of seven-day-old mice. This location raises the possibility that presumptive founder cells are derived from the ventricular-subventricular zone (V-SVZ). To examine the cellular biology of these tumors, single-cell RNA sequencing was performed, which revealed high intratumoral heterogeneity. Data comparison with reference CNS cell types indicated the highest similarity of tumor cells with transit-amplifying NSCs or activated NSCs of the V-SVZ. Consequently, we analyzed V-SVZ NSCs of our mouse model aiming to confirm that the tumors originate from this stem cell niche. Mutant V-SVZ NSCs showed significantly increased cell viability and proliferation as well as reduced glial and neural differentiation in vitro compared to control cells. In summary, we demonstrate the oncogenic potential of a combined loss of function of CREBBP and overexpression of MYCN in this cell population. hGFAP-cre::Crebbp::lsl-MYCN mice thus provide a valuable tool to study tumor-driving mechanisms in a key neural stem/ progenitor cell niche.
PubMed: 37407554
DOI: 10.1038/s41389-023-00481-3 -
Cureus May 2023Esthesioneuroblastoma (ENB), also known as olfactory neuroblastoma, is a rare malignant tumor of neuroectodermal origin that arises from the olfactory epithelium. We...
Esthesioneuroblastoma (ENB), also known as olfactory neuroblastoma, is a rare malignant tumor of neuroectodermal origin that arises from the olfactory epithelium. We present a case of ENB metastasizing through the leptomeningeal route to the spinal dura, which was treated with CyberKnife (CK) stereotactic radiosurgery (SRS), and aim to assess the safety and effectiveness of SRS in such cases. To the best of our knowledge, this is the first case report in the literature that discusses ENB spinal leptomeningeal metastases treated with CK radiosurgery. We retrospectively review the clinical and radiological outcomes in a 70-year-old female with ENB metastasis to the spine. Progression-free survival (PFS), overall survival (OS), and local tumor control (LTC) are investigated. In our patient, ENB had been diagnosed at the age of 58 years and spinal metastases had been first noted at the age of 65 years. A total of six spinal lesions received CK SRS. Lesions were present at the level of C1, C2, C3, C6-C7, T5, and T10-11. The median target volume was 0.72 cc (range: 0.32-2.54). A median marginal dose of 24 Gy was delivered to the tumors with a median of three fractions to a median isodose line of 80% (range: 78-81). LTC at the 24-month follow-up was 100%. PFS and OS were 27 months and 40 months, respectively. No adverse radiation effects were reported. Even though the treated spinal lesions remained stable, the number of new metastatic lesions had increased with progressive osseous and dural metastatic lesions within the cervical, thoracic, and lumbar spine at the last follow-up. SRS provides relatively good LTC for patients with ENB metastasizing to the spine, with no radiation-induced adverse events.
PubMed: 37398775
DOI: 10.7759/cureus.39791 -
Cureus May 2023Olfactory neuroblastoma is a rare, undifferentiated carcinoma of the nasal cavity. It is an extremely rare malignancy, usually occurring in the sixth decade of life with...
Olfactory neuroblastoma is a rare, undifferentiated carcinoma of the nasal cavity. It is an extremely rare malignancy, usually occurring in the sixth decade of life with no known underlying cause. In this case report, we present a 71-year-old male with an enlarging facial mass near the right medial nasal bridge, initially diagnosed as undifferentiated carcinoma on biopsy and later confirmed as olfactory neuroblastoma eroding into the anterior skull base. Our patient presented with the signs and symptoms of epiphora, epistaxis, intermittent headaches, anosmia, and an enlarging facial mass. The treatment modalities include surgery, radiation therapy, and chemotherapy. The purpose of this case report is to highlight the importance of chemotherapy and adjuvant radiotherapy for treatment without the need for surgery. Further studies need to be done to divulge the risk factors for olfactory neuroblastoma and to implore new chemotherapeutic treatment modalities that minimize long-term mortality and morbidity.
PubMed: 37384073
DOI: 10.7759/cureus.39614 -
Cancer Research Communications Jun 2023Olfactory neuroblastoma is a rare tumor arising from the olfactory cleft region of the nasal cavity. Because of the low incidence of this tumor, as well as an absence of...
UNLABELLED
Olfactory neuroblastoma is a rare tumor arising from the olfactory cleft region of the nasal cavity. Because of the low incidence of this tumor, as well as an absence of established cell lines and murine models, understanding the mechanisms driving olfactory neuroblastoma pathobiology has been challenging. Here, we sought to apply advances from research on the human olfactory epithelial neurogenic niche, along with new biocomputational approaches, to better understand the cellular and molecular factors in low- and high-grade olfactory neuroblastoma and how specific transcriptomic markers may predict prognosis. We analyzed a total of 19 olfactory neuroblastoma samples with available bulk RNA-sequencing and survival data, along with 10 samples from normal olfactory epithelium. A bulk RNA-sequencing deconvolution model identified a significant increase in globose basal cell (GBC) and CD8 T-cell identities in high-grade tumors (GBC from ∼0% to 8%, CD8 T cell from 0.7% to 2.2%), and significant decreases in mature neuronal, Bowman's gland, and olfactory ensheathing programs, in high-grade tumors (mature neuronal from 3.7% to ∼0%, Bowman's gland from 18.6% to 10.5%, olfactory ensheathing from 3.4% to 1.1%). Trajectory analysis identified potential regulatory pathways in proliferative olfactory neuroblastoma cells, including PRC2, which was validated by immunofluorescence staining. Survival analysis guided by gene expression in bulk RNA-sequencing data identified favorable prognostic markers such as SOX9, S100B, and PLP1 expression.
SIGNIFICANCE
Our analyses provide a basis for additional research on olfactory neuroblastoma management, as well as identification of potential new prognostic markers.
Topics: Mice; Humans; Animals; Esthesioneuroblastoma, Olfactory; Olfactory Mucosa; Olfactory Pathways; Nose Neoplasms; RNA
PubMed: 37377616
DOI: 10.1158/2767-9764.CRC-23-0013