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World Neurosurgery Jun 2019Many controversies exist in the diagnosis and management of this aggressively malignant condition, mainly because of limited literature and lack of randomized control... (Review)
Review
INTRODUCTION
Many controversies exist in the diagnosis and management of this aggressively malignant condition, mainly because of limited literature and lack of randomized control trials, resulting in nonstandardized treatment methods. We performed a comprehensive review of the literature to identify management approach and treatment options for esthesioneuroblastoma.
METHODS
An extensive review of the published literature was conducted in PubMed, OVID Medline, and EMBASE journals for studies of esthesioneuroblastoma. Terms for search included esthesioneuroblastoma (ENB) and olfactory neuroblastoma (ONB). No date restrictions were used.
RESULTS
The search yielded 3876 related articles. Cross-checking of articles led to exclusion of duplicate articles. The remaining 1170 articles were screened for their full text and English language availability. Of 609 full-text articles available, animal studies, irrelevant articles, and studies with mixed/confusing data were excluded. We finalized 149 articles pertaining to the topic, including 119 original research articles, 3 book chapters, 11 reviews, 9 case reports, and 7 case series.
CONCLUSIONS
Surgical resection followed by radiotherapy is the standard for treatment for higher-grade lesions. The endoscopic endonasal approach is gaining further recognition with more favorable outcomes and better survival than for open surgery. Postoperative radiotherapy is associated with the highest overall survival and shows benefit for patients with higher-stage disease and those who receive chemotherapy. Recurrence rates after treatment vary drastically in the literature and, therefore, prolonged follow-up with repeated imaging is recommended. Lifelong surveillance is recommended because of late recurrences associated with this tumor.
Topics: Disease Management; Esthesioneuroblastoma, Olfactory; Humans; Nasal Cavity; Neurosurgical Procedures; Nose Neoplasms; Radiotherapy, Adjuvant
PubMed: 30862589
DOI: 10.1016/j.wneu.2019.03.014 -
Annals of Diagnostic Pathology Jun 2016Olfactory neuroblastoma (ONB) is a rare malignant tumor. Although the vast majority of cases arise in the nasal cavity, ONB is rarely reported in ectopic locations. We... (Review)
Review
Olfactory neuroblastoma (ONB) is a rare malignant tumor. Although the vast majority of cases arise in the nasal cavity, ONB is rarely reported in ectopic locations. We report a case of ONB in the maxillary sinus. A 63-year-old woman presented with left-sided nasal obstruction and epistaxis. Magnetic resonance imaging showed a nonenhancing left maxillary sinus tumor. Histologic sections showed ONB, Hyams grade IV, invading bone, skeletal muscle, and adjacent fibroadipose tissue. It is essential to be accurate when diagnosing sinonasal tumors because the differential diagnosis is broad, and one must consider the possibility of ectopic ONB, although it is rare. The behavior of ONB and other neuroendocrine tumors of the sinonasal region is quite different, and there are varied approaches to treatment. Therefore, an accurate diagnosis as well as correct grade and stage must be assigned.
Topics: Diagnosis, Differential; Esthesioneuroblastoma, Olfactory; Female; Humans; Maxillary Sinus Neoplasms; Middle Aged; Nasal Cavity
PubMed: 27180059
DOI: 10.1016/j.anndiagpath.2016.04.001 -
Modern Pathology : An Official Journal... May 2023Olfactory neuroblastoma (ONB, esthesioneuroblastoma) is a sinonasal cancer with an underdeveloped diagnostic toolkit, and is the subject of many incidents of tumor...
Olfactory neuroblastoma (ONB, esthesioneuroblastoma) is a sinonasal cancer with an underdeveloped diagnostic toolkit, and is the subject of many incidents of tumor misclassification throughout the literature. Despite its name, connections between the cancer and normal cells of the olfactory epithelium have not been systematically explored and markers of olfactory epithelial cell types are not deployed in clinical practice. Here, we utilize an integrated human-mouse single-cell atlas of the nasal mucosa, including the olfactory epithelium, to identify transcriptomic programs that link ONB to a specific population of stem/progenitor cells known as olfactory epithelial globose basal cells (GBCs). Expression of a GBC transcription factor NEUROD1 distinguishes both low- and high-grade ONB from sinonasal undifferentiated carcinoma, a potential histologic mimic with a distinctly unfavorable prognosis. Furthermore, we identify a reproducible subpopulation of highly proliferative ONB cells expressing the GBC stemness marker EZH2, suggesting that EZH2 inhibition may play a role in the targeted treatment of ONB. Finally, we study the cellular states comprising ONB parenchyma using single-cell transcriptomics and identify evidence of a conserved GBC transcriptional regulatory circuit that governs divergent neuronal-versus-sustentacular differentiation. These results link ONB to a specific cell type for the first time and identify conserved developmental pathways within ONB that inform diagnostic, prognostic, and mechanistic investigation.
Topics: Humans; Mice; Animals; Esthesioneuroblastoma, Olfactory; Paranasal Sinus Neoplasms; Neurons; Nose Neoplasms; Nasal Cavity
PubMed: 36841178
DOI: 10.1016/j.modpat.2023.100122 -
Head and Neck Pathology Mar 2016Neuroendocrine neoplasms (NENs) can occur in organs or tissues that do not contain neuroendocrine cells normally and do not necessarily imply embryologic derivation from... (Review)
Review
Neuroendocrine neoplasms (NENs) can occur in organs or tissues that do not contain neuroendocrine cells normally and do not necessarily imply embryologic derivation from the neuroectoderm; but rather reflect a shared phenotype characterized by the expression of multiple genes encoding both endocrine and neuronal features. NENs are rare in the sinonasal tract and are subdivided into epithelial and neural subtypes based on the presence of keratins or neurofilaments, respectively. Although relatively rare, neuroendocrine carcinomas (NECs) and olfactory neuroblastoma (ONB) are the most common neuroendocrine neoplasms of the sinonasal tract. The focus of this review is to highlight recent developments in the pathology of sinonasal NECs and ONB in light of the upcoming update of the World Health Organization (WHO) 2005 classification of tumors of the head and neck.
Topics: Carcinoma, Neuroendocrine; Esthesioneuroblastoma, Olfactory; Humans; Nasal Cavity; Nose Neoplasms; Paranasal Sinus Neoplasms
PubMed: 26830400
DOI: 10.1007/s12105-016-0696-7 -
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 -
European Journal of Cancer (Oxford,... Feb 2022Olfactory neuroblastoma (ONB) is a rare cancer of the sinonasal region. We provide a comprehensive analysis of this malignancy with molecular and clinical trial data on...
INTRODUCTION
Olfactory neuroblastoma (ONB) is a rare cancer of the sinonasal region. We provide a comprehensive analysis of this malignancy with molecular and clinical trial data on a subset of our cohort to report on the potential efficacy of somatostatin receptor 2 (SSTR2)-targeting imaging and therapy.
METHODS
We conducted a retrospective analysis of 404 primary, locally recurrent, and metastatic olfactory neuroblastoma (ONB) patients from 12 institutions in the United States of America, United Kingdom and Europe. Clinicopathological characteristics and treatment approach were evaluated. SSTR2 expression, SSTR2-targeted imaging and the efficacy of peptide receptor radionuclide therapy [PRRT](Lu-DOTATATE) were reported in a subset of our cohort (LUTHREE trial; NCT03454763).
RESULTS
Dural infiltration at presentation was a significant predictor of overall survival (OS) and disease-free survival (DFS) in primary cases (n = 278). Kadish-Morita staging and Dulguerov T-stage both had limitations regarding their prognostic value. Multivariable survival analysis demonstrated improved outcomes with lower stage and receipt of adjuvant radiotherapy. Prophylactic neck irradiation significantly reduces the rate of nodal recurrence. 82.4% of the cohort were positive for SSTR2; treatment of three metastatic cases with SSTR2-targeted peptide-radionuclide receptor therapy (PRRT) in the LUTHREE trial was well-tolerated and resulted in stable disease (SD).
CONCLUSIONS
This study presents pertinent clinical data from the largest dataset, to date, on ONB. We identify key prognostic markers and integrate these into an updated staging system, highlight the importance of adjuvant radiotherapy across all disease stages, the utility of prophylactic neck irradiation and the potential efficacy of targeting SSTR2 to manage disease.
Topics: Esthesioneuroblastoma, Olfactory; Humans; Nasal Cavity; Neuroblastoma; Nose Neoplasms; Positron-Emission Tomography; Radioisotopes; Radionuclide Imaging; Receptors, Somatostatin; Retrospective Studies
PubMed: 34980502
DOI: 10.1016/j.ejca.2021.09.046 -
Auris, Nasus, Larynx May 1998A total of 17 histologically confirmed olfactory neuroblastomas treated at Helsinki University Central Hospital between 1962 and 1996 were reviewed retrospectively. The... (Comparative Study)
Comparative Study Review
A total of 17 histologically confirmed olfactory neuroblastomas treated at Helsinki University Central Hospital between 1962 and 1996 were reviewed retrospectively. The tumors displayed a variety of imaging characteristics and aggressiveness. Imaging evolved from plain X-rays at the beginning of the study period to CT and MRI during the latter part of the study. CT provided the best information about the tumor and its local invasion especially into surrounding bony structures. MRI allowed an estimate of tumor spread into surrounding soft-tissue areas, such as the anterior cranial fossa and the retromaxillary space. However, signal intensity characteristics were not specific for olfactory neuroblastomas. Bone scintigraphy and MIBG scan allowed us to detect distant metastases. Olfactory neuroblastoma is an aggressive malignancy and the prognosis is poor in most cases, as shown by the short survival rates (average 45.3 months) in our study group. The tumor can be detected, delineated and its characteristics suspected by modern radiology. Definite diagnosis is based on histopathology. This study proposes general imaging strategies for detecting this disease.
Topics: Adolescent; Adult; Aged; Aged, 80 and over; Child, Preschool; Diagnostic Imaging; Esthesioneuroblastoma, Olfactory; Female; Humans; Male; Middle Aged; Nasal Cavity; Neoplasm Invasiveness; Neoplasm Staging; Nose Neoplasms; Sensitivity and Specificity
PubMed: 9673731
DOI: 10.1016/s0385-8146(98)00003-0 -
JPMA. the Journal of the Pakistan... Dec 1997
Topics: Adult; Esthesioneuroblastoma, Olfactory; Female; Humans; Immunohistochemistry; Nasal Cavity; Nose Neoplasms
PubMed: 9510645
DOI: No ID Found -
Head and Neck Pathology Mar 2018Sinonasal tumors with neuroendocrine differentiation form a group of rare heterogeneous neoplasms of neuroectodermal and epithelial origin, consisting of olfactory... (Review)
Review
Sinonasal tumors with neuroendocrine differentiation form a group of rare heterogeneous neoplasms of neuroectodermal and epithelial origin, consisting of olfactory neuroblastomas and neuroendocrine carcinomas. Because the natural history and biological behavior of this group of tumors vary, the morphological diagnosis coupled with grading/staging is important for prognostication, and the approach to treatment and rehabilitation is multidisciplinary. The identification of molecular abnormalities underlying these tumors is critical to the development of specific targeted therapies and the design of clinical trials.
Topics: Carcinoma, Neuroendocrine; Esthesioneuroblastoma, Olfactory; Humans; Nasal Cavity; Nose Neoplasms; Paranasal Sinus Neoplasms
PubMed: 29427030
DOI: 10.1007/s12105-018-0887-5 -
Lin Chuang Er Bi Yan Hou Tou Jing Wai... Mar 2021To explore the therapeutic effect and prognostic risk factors of olfactory neuroblastoma. Retrospective analysis of clinical data of 31 patients with olfactory...
To explore the therapeutic effect and prognostic risk factors of olfactory neuroblastoma. Retrospective analysis of clinical data of 31 patients with olfactory neuroblastoma. The Kaplan-Meier method was used for survival analysis to calculate the overall survival rate and progress-free survival rate. All 31 patients underwent surgical treatment and 7 patients died, of which 4 patients died of simple intracranial invasion and 3 patients died of concurrent distant metastasis (lung and spinal cord). The average death time was 40.7 (20-57) months. Statistical analysis showed that craniocerebral invasion (=0.035), age ≥60 years (=0.042), and Ki-67≥20%(=0.018) were closely related to the poor prognosis. It is speculated that the increase of T staging and modified Kadish staging are also predictors of poor prognosis. The 1-year and 5-year overall survival rates were 100.0% and 72.5%, and the 1-year and 5-year progress-free survival rates were 87.8% and 33.6% after first surgery. Conclusion:Surgery combined with radiotherapy and chemotherapy are the main treatments for olfactory neuroblastoma, but postoperative recurrence and metastasis are common. About 22.6% of the patients died during the follow-up. Advanced age, intracranial invasion and Ki-67≥20% are closely related to poor prognosis. The tumor was completely removed by the initial surgery and restricted in nasal cavity and sinuses are the key factors for a good prognosis.
Topics: Esthesioneuroblastoma, Olfactory; Humans; Middle Aged; Nasal Cavity; Neoplasm Recurrence, Local; Neoplasm Staging; Nose Neoplasms; Prognosis; Retrospective Studies; Survival Rate
PubMed: 33794606
DOI: 10.13201/j.issn.2096-7993.2021.03.007