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Annals of Diagnostic Pathology Oct 2019Beyond squamous carcinoma, a variety of diagnostically challenging neoplasms arise within various head and neck sites. This is particularly the situation with neoplasms... (Review)
Review
CONTEXT
Beyond squamous carcinoma, a variety of diagnostically challenging neoplasms arise within various head and neck sites. This is particularly the situation with neoplasms where little cellular cytoplasm is present to assist the pathologist in categorizing such lesions.
OBJECTIVE
To highlight diagnostic pitfalls that accompanying neoplasms composed primarily of 'cytoplasmically-poor' cells. These pitfalls include morphologic and immunohistochemical traps that emerge from this class of neoplasms.
DATA SOURCES
Selection of pathologic specimens from the author's personal files, and literature review.
CONCLUSIONS
Interpretative pitfalls regarding the histopathology and immunophenotype of small 'cytoplasmically-poor' neoplasms are a diagnostic hazard in head and neck surgical pathology practice, and require knowledge of histomorphologic plasticity and aberrant immunophenotyping.
Topics: Head and Neck Neoplasms; Humans; Pathologists; Pathology, Clinical
PubMed: 31382078
DOI: 10.1016/j.anndiagpath.2019.07.005 -
Der Pathologe Feb 2018Common to all neuroendocrine neoplasms (NENs), irrespective of their site of origin, is the expression of synaptophysin and chromogranin A. NENs of the head and neck... (Review)
Review
Common to all neuroendocrine neoplasms (NENs), irrespective of their site of origin, is the expression of synaptophysin and chromogranin A. NENs of the head and neck region derive either from epithelial or neural/neuroectodermal tissues. The epithelial-type NENs express cytokeratins and include the well-differentiated typical and atypical carcinoids (also called low- and intermediate-grade neuroendocrine carcinomas by WHO), the poorly differentiated high-grade neuroendocrine carcinomas of small and large cell type and the mixed neuroendocrine-nonneuroendocrine neoplasms. The neural-neuroectodermal-type NENs comprise olfactory neuroblastoma and paraganglioma, each of them with distinct clinicopathological characteristics. Olfactory neuroblastomas show a spectrum of histologic differentiation and are prognostically classified by Hyams grading. Paragangliomas often occur multiple and show a familial background. Most head and neck NENs occur in the upper respiratory system. Their diagnosis follows the general guidelines for NENs, focusing on immunohistochemical profiling. Molecular examinations are so far only required in individual cases.
Topics: Carcinoid Tumor; Chromogranin A; Head and Neck Neoplasms; Humans; Lung Neoplasms; Neuroendocrine Tumors
PubMed: 29313083
DOI: 10.1007/s00292-017-0404-8 -
Veterinary Clinical Pathology Sep 2023Nasal tumors account for less than 10% of all feline neoplasms, with lymphoma, followed by adenocarcinoma, and squamous cell carcinoma, the most commonly reported. Nasal... (Review)
Review
Nasal tumors account for less than 10% of all feline neoplasms, with lymphoma, followed by adenocarcinoma, and squamous cell carcinoma, the most commonly reported. Nasal neuroectodermal tumors, including olfactory neuroblastoma (ONB), are scarcely described, and their tumorigenesis is largely unknown. Here we report the cytological, histological, and immunohistochemical features of a feline ONB. We also provide a pathological review of nasal neuroendocrine neoplasms in cats. A 7-year-old Burmese cat was evaluated for sneezing, occasional epistaxis, and upper respiratory noise for 8 months. Computed tomography (CT) imaging revealed a 7 × 5 × 3 mm irregular mass effacing and expanding the nasal cavity, which extended to the nasopharynx. Cytologically, neoplastic cells were round to polygonal and had a round nucleus with finely stippled chromatin, a single small nucleolus, and abundant pale blue cytoplasm, which contained abundant fine pale pink granules. They exhibited mild cellular atypia, anisocytosis, and mild to occasionally moderate anisokaryosis. Rhinoscopic biopsies revealed a densely cellular, malignant neuroepithelial neoplasm. Cells were arranged in densely packed trabeculae and formed Homer Wright and Flexner-Wintersteiner-like rosettes, with rare mitotic figures and scant supportive fibrovascular stroma. Immunohistochemically, neoplastic cells were positive for vimentin, cytokeratin AE1/AE3, COX-2, and beta-tubulin and negative for S-100, chromogranin A, CD117, and epithelial membrane antigen (EMA). An ONB was diagnosed based on histological and immunohistochemical findings. Interestingly, and similar to nasal carcinomas, neoplastic cells diffusely neo-expressed COX-2. To the authors' knowledge, there is no previous evidence of COX-2 in feline ONB. Histopathology and immunohistochemistry are required for a definitive diagnosis of ONB.
Topics: Cats; Animals; Esthesioneuroblastoma, Olfactory; Cyclooxygenase 2; Nose Neoplasms; Nasal Cavity; Carcinoma; Cat Diseases
PubMed: 37468966
DOI: 10.1111/vcp.13255 -
Head and Neck Pathology Dec 2021Somatostatin receptor 2 (SSTR2) expression has previously been documented in olfactory neuroblastoma (ONB). Here, we fully characterize SSTR2 expression in ONB and...
Somatostatin receptor 2 (SSTR2) expression has previously been documented in olfactory neuroblastoma (ONB). Here, we fully characterize SSTR2 expression in ONB and correlate staining results with clinicopathologic parameters including Hyams grade. We also assess SSTR2 immunohistochemistry expression in various histologic mimics of ONB to assess its diagnostic functionality. 78 ONBs (51 primary biopsies/excisions and 27 recurrences/metastases) from 58 patients were stained for SSTR2. H-scores based on intensity (0-3 +) and percentage of tumor cells staining were assigned to all cases. 51 histologic mimics were stained and scored in an identical fashion. 77/78 (99%) ONB cases demonstrated SSTR2 staining (mean H-score: 189, range: 0-290). There were no significant differences in staining between primary tumors and recurrences/metastases (mean H-score: 185 vs 198). Primary low-grade ONB had somewhat stronger staining than high-grade tumors (mean H-score: 200 vs 174). SSTR2 expression had no prognostic value when considering disease-free or disease-specific survival. SSTR2 staining is significantly higher in ONB than its histologic mimics (mean H-score: 189 vs 12.9, p < 0.001) suggesting a potential use of the marker in diagnosis of ONB. In conclusion, SSTR2 is consistently expressed in ONB suggesting a role for somatostatin-analog based imaging and therapy in this disease. More generally, SSTR2 may be another marker of neuroendocrine differentiation in ONB.
Topics: Biomarkers, Tumor; Esthesioneuroblastoma, Olfactory; Humans; Immunohistochemistry; Nose Neoplasms; Receptors, Somatostatin
PubMed: 33929681
DOI: 10.1007/s12105-021-01329-1 -
Diagnostics (Basel, Switzerland) Mar 2022The purpose of the current study was to assess the prevalence of cyst formation at the brain-tumor interface in olfactory neuroblastoma. We used the UCLA patient-based...
The purpose of the current study was to assess the prevalence of cyst formation at the brain-tumor interface in olfactory neuroblastoma. We used the UCLA patient-based Pathology and Radiology Head and Neck Database (UPP&R HAND) to identify the largest patient cohort reported to date with imaging and pathology data. Eighteen of thirty-one patients (58.1%) had evidence of intracranial extension on MRI, while four (22.0%) demonstrated cyst formation at the brain-tumor interface. The extent of intracranial extension was by far the strongest predictor for intracranial cyst formation, regardless of Hyams tumor grade, using a binary logistics regression model ( = 0.002) and ROC curve analysis (AUC 94.6%). Cyst formation at the brain-tumor interface was an uncommon imaging finding, and tends to occur with a larger component of intracranial tumor extension.
PubMed: 35328167
DOI: 10.3390/diagnostics12030614 -
World Neurosurgery Mar 2022Because of their rarity, it is not known whether isocitrate dehydrogenase 2 (IDH2) mutations are related to the occurrence of olfactory neuroblastoma (ONB). We...
OBJECTIVE
Because of their rarity, it is not known whether isocitrate dehydrogenase 2 (IDH2) mutations are related to the occurrence of olfactory neuroblastoma (ONB). We investigated the relationships between IDH2 mutations, clinicopathological parameters, and the prognosis for ONB to establish a molecular classification using IDH2 mutations.
METHODS
An 82-patient cohort was retrospectively screened using immunohistochemistry with a mutation-specific IDH2 antibody and real-time polymerase chain reactions for IDH2 mutations. We also immunohistochemically determined the expression of chromogranin A, synaptophysin, neuron-specific enolase, CD56, S100, and Ki-67.
RESULTS
The 2 methods used for the detection of IDH2 mutations had high consistency. Mutation of IDH2 detected by real-time polymerase chain reaction correlated with higher Kadish stage, Hyams grade, and Ki-67 proliferation index. Mutation of IDH2 correlated negatively with the expression of chromogranin A, synaptophysin, CD56, and S100. Kaplan-Meier analysis showed that an IDH2 mutation, a high Hyams grade, and high Ki-67 proliferation index were associated with poor overall survival. The Hyams grade and IDH2 mutation were independent prognostic factors on multivariable analysis.
CONCLUSIONS
Immunohistochemistry was a reliable method to assess the mutation status of IDH2. Tumors with IDH2 mutations represented a distinct subset with aggressive behavior and conferred a poor prognosis. The gene status of IDH2 could be a major molecular classification criterion in ONB.
Topics: Chromogranin A; Esthesioneuroblastoma, Olfactory; Humans; Isocitrate Dehydrogenase; Ki-67 Antigen; Mutation; Nasal Cavity; Nose Neoplasms; Prognosis; Retrospective Studies; Synaptophysin
PubMed: 34856401
DOI: 10.1016/j.wneu.2021.11.103 -
Acta Otorrinolaringologica Espanola 2018Olfactory neuroblastoma (ONB) is a rare entity that constitutes less than 5% of nasosinusal malignancies. Mainstream treatment consists in surgical resection+/-adjuvant... (Review)
Review
INTRODUCTION AND OBJECTIVES
Olfactory neuroblastoma (ONB) is a rare entity that constitutes less than 5% of nasosinusal malignancies. Mainstream treatment consists in surgical resection+/-adjuvant radiotherapy. By exposing results observed with apparition of new therapeutic options as neoadjuvant chemotherapy, the objective is to evaluate a series and a review of the current literature.
METHODS
A retrospective review was conducted including patients diagnosed and followed-up for ONB from 2008 to 2015 in our institution.
RESULTS
9 patients were included. Mean follow-up of 52.5 months (range 10-107). Kadish stage: A, 1 patient (11.1%) treated with endoscopic surgery; B, 2 patients (22.2%) treated with endoscopic surgery (one of them received adjuvant radiotherapy); C, 6 patients (66.7%), 4 patients presented intracranial extension and were treated with neoadjuvant chemotherapy followed by surgery and radiotherapy. The other 2 patients presented isolated orbital extension, treated with radical surgery (endoscopic or craniofacial resection) plus radiotherapy. The 5-year disease free and overall survival observed was 88.9%.
CONCLUSION
Neoadjuvant chemotherapy could be an effective treatment for tumor reduction, improving surgical resection and reducing its complications.
Topics: Adult; Aged; Chemotherapy, Adjuvant; Esthesioneuroblastoma, Olfactory; Female; Follow-Up Studies; Humans; Male; Middle Aged; Nasal Cavity; Neoadjuvant Therapy; Nose Neoplasms; Retrospective Studies
PubMed: 29061289
DOI: 10.1016/j.otorri.2017.07.001 -
Auris, Nasus, Larynx Jun 2023Olfactory neuroblastoma (ONB) is often difficult to pathologically distinguish from other small round cell tumors (SRCTs) arising in the nasal cavities. Although there...
OBJECTIVE
Olfactory neuroblastoma (ONB) is often difficult to pathologically distinguish from other small round cell tumors (SRCTs) arising in the nasal cavities. Although there are several diagnostic markers used for differential diagnosis of ONB, these molecules are also expressed in various neuronal derived tumors. Here, we examined the expression of NeuroD, GAP43, and olfactory marker protein (OMP) in ONB and non-ONB SRCT to determine their utility in the differential diagnosis of ONB.
METHODS
Twenty-six patients diagnosed with and treated for ONB at Kobe University Hospital between 1997 and 2017 with formalin-fixed, paraffin-embedded biopsy or surgical resection specimens were included. The expressions of NeuroD, GAP43, and OMP were immunohistochemically examined in these 26 ONB specimens and specimens from 13 SRCTs arising in the nasal cavities for reference.
RESULTS
Among the 26 ONB samples, focal, patchy, and marked staining for NeuroD was observed in 4, 3, and 9 samples, respectively. Focal, patchy, and marked GAP43 staining was observed in 5, 3, and 11 samples, respectively. Consequently, marked positive staining for either NeuroD or GAP43 was observed in 54% (14/26) of ONBs. Among the 13 SRCTs, marked staining for NeuroD was observed in two small cell carcinomas, one undifferentiated carcinoma, and one neuroendocrine carcinoma, whereas marked positive staining for GAP43 was observed only in one undifferentiated carcinoma. No specimen in this study exhibited OMP staining.
CONCLUSIONS
Our results suggest possible roles of GAP43 immunostaining in the differential diagnosis of ONB.
Topics: Humans; Carcinoma, Neuroendocrine; Esthesioneuroblastoma, Olfactory; Nasal Cavity; Nose Neoplasms; Olfactory Marker Protein; GAP-43 Protein
PubMed: 35999123
DOI: 10.1016/j.anl.2022.08.004 -
Surgical and Radiologic Anatomy : SRA Jul 2015Olfactory neuroblastoma (ONB) is a rare malignant tumor of the nose. The currently available evidence links this disease with cells of the olfactory epithelium. The...
PURPOSE
Olfactory neuroblastoma (ONB) is a rare malignant tumor of the nose. The currently available evidence links this disease with cells of the olfactory epithelium. The detailed description of tumor site and its extension is the key of treatment. The aim of the present study was to describe the way ONB develops inside and outside the olfactory cleft.
METHODS
Thirteen consecutive patients treated between 2004 and 2014 for ONB with unequivocal pathologic diagnosis, complete diagnostic imaging and endonasal endoscopy surgery were enrolled in this retrospective study. The site of origin and local extension of each tumor were studied in detail based on computed tomography/magnetic resonance imaging, surgical report, registered videotape of the surgery, and pathological reports.
RESULTS
This series shows the behavior of a tumor arising either in the olfactory clefts (11 cases) or in the ethmoidal labyrinth (2 cases). When the setting begins with a tumor located in the olfactory cleft (below or in contact with the cribriform plate), the further step can be the extension to the ethmoidal labyrinth before intracranial or intraorbital extension. When tumors originate inside the ethmoidal labyrinths, the extension can first be into frontal sinus or orbital cavity.
CONCLUSIONS
This fine anatomic and radiologic description shows the natural behavior of ONB inside and outside the olfactory cleft. As a consequence, the staging system developed by Kadish seems inadequate and Dulguerov's staging system could be improved. However, the preliminary proposed modification has to be evaluated in a prospective and large, multicenter cohort of patients.
Topics: Adolescent; Adult; Aged; Aged, 80 and over; Esthesioneuroblastoma, Olfactory; Female; Humans; Magnetic Resonance Imaging; Male; Middle Aged; Nasal Cavity; Nose Neoplasms; Retrospective Studies; Tomography, X-Ray Computed; Young Adult
PubMed: 25218517
DOI: 10.1007/s00276-014-1375-6 -
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