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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 -
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 -
Ectopic Olfactory Neuroblastoma: Systematic Review of a Rare Clinical Entity among Sinonasal Tumors.Journal of Neurological Surgery. Part... Apr 2024Ectopic olfactory neuroblastoma is an uncommon manifestation of an already rare neoplasm. We aimed to systematically review the literature for cases of ectopic...
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. 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. 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. 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.
PubMed: 38463937
DOI: 10.1055/a-1993-7790 -
Journal of Microscopy and Ultrastructure 2024One of the most challenging diagnostic categories in the sinonasal tract includes small-blue-round-cell tumors. These are malignant tumors which show many overlapping...
BACKGROUND
One of the most challenging diagnostic categories in the sinonasal tract includes small-blue-round-cell tumors. These are malignant tumors which show many overlapping histomorphology and immunohistochemistry (IHC) findings. Limited, small biopsy of these not completely excisable tumors adds to the diagnostic confusion.
MATERIALS AND METHODS
A cross-sectional study was done for 2 years (January 2018-December 2020) in a tertiary care institute, which included 70 cases of tumors of which 49 cases were malignant. All paraffin-embedded blocks were subjected to hematoxylin and eosin stain and IHC followed by molecular study wherever needed.
RESULTS
Of the total cases, small-blue-round-cell tumor constituted the major category comprising 20 rare and interesting cases which included sinonasal undifferentiated carcinoma (4 cases), malignant lymphoma (2 cases of diffuse large B-cell lymphoma and 2 cases of extranodal natural killer/T-cell lymphoma), rhabdomyosarcoma (2 cases), olfactory neuroblastoma (2 cases), malignant melanoma (2 cases), plasmacytoma (2 cases), atypical Ewing's sarcoma (EWS) (1 case), EWS (1 case), nuclear protein in testis (NUT) carcinoma (1 case), and small-cell neuroendocrine carcinoma (1 case).
CONCLUSION
Tumors of the sinonasal tract are very diverse, more so in small-round-cell tumor which present with a undifferentiated morphology. Thus, accurate diagnosis needs clinicoradiological parameters and special ancillary techniques such as IHC and molecular study in addition to histopathology for early diagnosis and therapy to prevent significant morbidity and mortality caused in these tumors.
PubMed: 38633570
DOI: 10.4103/jmau.jmau_70_21 -
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