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Indian Journal of Otolaryngology and... Oct 2022Esthesioneuroblastoma (ENB) is a rare, aggressive and malignant sinonasal tumour. This tumour makes for a very interesting study because ever since its mention in...
Esthesioneuroblastoma (ENB) is a rare, aggressive and malignant sinonasal tumour. This tumour makes for a very interesting study because ever since its mention in medical literature, although quite a lot of research has been put into it, a lot is yet to be known about it. To summarize the available literature & document an article on the contemporary practises in the management of ENB. A comprehensive literature review was done using Google Scholar and PubMed database with the key words: "esthesioneuroblastoma", "olfactory neuroblastoma", "Kadish" and "Hyams". Articles published in English were used. Articles published till April 2021 were reviewed. ENB has a diverse histological appearance. This tumour has a bimodal age distribution without any gender predilection. Prompt identification and treatment is required for a favourable outcome and requires close collaboration of radiology, pathology, and rhinology. Staging, management and prognosis are determined by regional involvement in non-metastatic cases due to its proximity to neurological structures and locally destructive nature. Immunohistochemistry is a must for accurate diagnosis. Uni-modality of treatment has high chances of local recurrence. Multi-modality provides good results even in advanced stage. A combination of surgery and radiotherapy seems to be the optimum approach to treatment. The exact role of chemotherapy in treatment protocols is still unclear. Cervical lymph node metastases are commonly studied, however its management in patients who present without apparent nodal involvement is not yet clear. An extended follow-up period is recommended in all patients of ENB patients.
PubMed: 36452592
DOI: 10.1007/s12070-021-02726-2 -
Frontiers in Oncology 2023Olfactory neuroblastoma (ONB) is a rare neoplasm originating from the olfactory neuroepithelium representing 3-6% of tumors of the sinonasal tract. ONB require... (Review)
Review
Olfactory neuroblastoma (ONB) is a rare neoplasm originating from the olfactory neuroepithelium representing 3-6% of tumors of the sinonasal tract. ONB require multi-disciplinary care. Historically, the gold standard surgical procedure for ONB has been open craniofacial resection. In the last years, endoscopic endonasal approaches have been largely introduced with lower complication rates, shorter hospital stay, and similar clinical outcome. Radiotherapy plays an important role in the management of ONB, however there are not generally accepted recommendations for its application. Although there is agreement that multimodal therapy is needed, the optimal use of chemotherapy is still unknown. The rarity of the disease, makes difficult to draw definitive conclusions about the role of systemic treatment in induction and concomitant setting.
PubMed: 37909011
DOI: 10.3389/fonc.2023.1242453 -
Current Opinion in Otolaryngology &... Feb 2022Sinonasal malignancies are rare and understudied, often diagnosed at late stages, and may behave aggressively. This review explores investigative diagnostic,... (Review)
Review
PURPOSE OF REVIEW
Sinonasal malignancies are rare and understudied, often diagnosed at late stages, and may behave aggressively. This review explores investigative diagnostic, therapeutic, and scientific advances specific to sinonasal undifferentiated carcinoma (SNUC), intestinal-type adenocarcinoma (ITAC), and olfactory neuroblastoma (ONB).
RECENT FINDINGS
A number of studies have recently contributed more robust knowledge of the genetic and molecular landscapes of SNUC, ITAC, and ONB. These analyses have identified SMARCB1 and IDH2 mutations in SNUC, potentially allowing for the tumor's subdivision. Recent studies have also defined a role for induction chemotherapy in SNUC. Somatic mutations for ITAC have been identified and may be potentially targetable with FDA approved therapies. Studies defining the tumor microenvironment for ITAC and ONB have introduced the possibility of immune checkpoint inhibition for these tumor types.
SUMMARY
Studies reviewed here detail promising results of the most current and novel characterization of SNUC, ITAC, and ONB genetic and molecular landscapes, which have informed ongoing therapeutic discovery. With continued multi-institutional efforts, the field of sinonasal tumor research will achieve higher disease control and improved treatment outcomes for patients afflicted with these rare cancers.
Topics: Biomarkers, Tumor; Carcinoma; Esthesioneuroblastoma, Olfactory; Humans; Maxillary Sinus Neoplasms; Nasal Cavity; Nose Neoplasms; Paranasal Sinus Neoplasms; Tumor Microenvironment
PubMed: 34958321
DOI: 10.1097/MOO.0000000000000776 -
Frontiers in Oncology 2024Olfactory neuroblastoma (ONB) is a rare malignant tumor arising from the olfactory neuroepithelium. The standard of care for ONB is surgical resection; however, detailed...
BACKGROUND
Olfactory neuroblastoma (ONB) is a rare malignant tumor arising from the olfactory neuroepithelium. The standard of care for ONB is surgical resection; however, detailed treatment protocols vary by institution. Our treatment protocol consists of endoscopic skull base surgery (ESBS) for endoscopically resectable cases and induction chemotherapy followed by craniotomy combined with ESBS for locally advanced cases, with postoperative radiotherapy performed for all cases. Chemoradiotherapy (CRT) is performed in unresectable cases. In this study, we evaluate our treatment protocol and outcomes for ONB.
METHODS
A retrospective review of patients with ONB was conducted. Outcomes included survival outcomes and perioperative data.
RESULTS
Fifteen patients (53.6%) underwent ESBS, 12 (42.9%) underwent craniotomy combined with ESBS, and 1 (3.6%) received CRT. The 5- and 10-year overall survival rates for all patients were 92.9% and 82.5%, respectively, with a median follow-up period of 81 months. The 5- and 10-year disease-free survival rates were 77.3% and 70.3%, respectively, and the 5- and 10-year local control rates were 88.2% and 80.2%, respectively. Patients undergoing ESBS demonstrated a significantly shorter operating time, period from operation to ambulation, hospitalization period, and less blood loss than those undergoing craniotomy combined with ESBS.
CONCLUSION
Our treatment protocol was found to afford favorable outcomes. Patients who underwent endoscopic resection showed lower complication rates and better perioperative data than those who underwent craniotomy combined with ESBS. With appropriate case selection, ESBS is considered a useful approach for ONB.
PubMed: 38756668
DOI: 10.3389/fonc.2024.1329572 -
Cancer Cell Jun 2024The olfactory epithelium undergoes neuronal regeneration from basal stem cells and is susceptible to olfactory neuroblastoma (ONB), a rare tumor of unclear origins....
The olfactory epithelium undergoes neuronal regeneration from basal stem cells and is susceptible to olfactory neuroblastoma (ONB), a rare tumor of unclear origins. Employing alterations in Rb1/Trp53/Myc (RPM), we establish a genetically engineered mouse model of high-grade metastatic ONB exhibiting a NEUROD1 immature neuronal phenotype. We demonstrate that globose basal cells (GBCs) are a permissive cell of origin for ONB and that ONBs exhibit cell fate heterogeneity that mimics normal GBC developmental trajectories. ASCL1 loss in RPM ONB leads to emergence of non-neuronal histopathologies, including a POU2F3 microvillar-like state. Similar to small-cell lung cancer (SCLC), mouse and human ONBs exhibit mutually exclusive NEUROD1 and POU2F3-like states, an immune-cold tumor microenvironment, intratumoral cell fate heterogeneity comprising neuronal and non-neuronal lineages, and cell fate plasticity-evidenced by barcode-based lineage tracing and single-cell transcriptomics. Collectively, our findings highlight conserved similarities between ONB and neuroendocrine tumors with significant implications for ONB classification and treatment.
Topics: Animals; Mice; Small Cell Lung Carcinoma; Humans; Esthesioneuroblastoma, Olfactory; Cell Lineage; Lung Neoplasms; Basic Helix-Loop-Helix Transcription Factors; Tumor Microenvironment; Nose Neoplasms; Olfactory Mucosa; Disease Models, Animal; Tumor Suppressor Protein p53
PubMed: 38788720
DOI: 10.1016/j.ccell.2024.05.003 -
Laryngoscope Investigative... Aug 2021Olfactory neuroblastoma (ONB) or esthesioneuroblastoma (ENB) is a rare malignancy of the nasal cavity believed to arise from the olfactory epithelium. The goal of this... (Review)
Review
BACKGROUND
Olfactory neuroblastoma (ONB) or esthesioneuroblastoma (ENB) is a rare malignancy of the nasal cavity believed to arise from the olfactory epithelium. The goal of this study was to systematically review the genomics, epigenetics, and cytogenetics of ONB and to understand the potential clinical implications of these studies.
METHODS
A systematic literature review was performed for articles published before May 2020 using Cochrane, Embase, Pubmed, and Scopus databases. Inclusion criteria included genomics, cytogenetics, and epigenetics studies on ONB. Exclusion criteria included studies not in English or systematic reviews. Articles and abstracts were reviewed by two independent reviewers to reduce bias during article selection and synthesis of results. Of the 36 studies included in this review, 24 were research articles and 12 were abstracts.
RESULTS
Although recurrent mutations among ONB tumors are uncommon, alterations in , , , , , , , , , , , , , , , and genes have been reported in several recent studies. In addition, cytogenetic studies revealed that the landscape of chromosomal aberrations varies widely amongst ONB tumors.
CONCLUSIONS
The rare character of ONB has limited the sample size available for cytogenetic, genomic, and epigenetic studies and contributes to the limitations of this systematic review. Comprehensive genomic and epigenomic studies with larger cohorts are warranted to validate the initial reports summarized in this review and to identify potential therapeutic targets for ONB.
PubMed: 34401496
DOI: 10.1002/lio2.597 -
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 -
Journal of Personalized Medicine Apr 2024Olfactory neuroblastoma (ONB) is an uncommon neuroendocrine malignancy arising from the olfactory neuroepithelium. ONB frequently presents with nonspecific sinonasal... (Review)
Review
Olfactory neuroblastoma (ONB) is an uncommon neuroendocrine malignancy arising from the olfactory neuroepithelium. ONB frequently presents with nonspecific sinonasal complaints, including nasal obstruction and epistaxis, and diagnosis can be obtained through a combination of physical examination, nasal endoscopy, and computed tomography and magnetic resonance imaging. Endoscopic resection with negative margins, with or without craniotomy, as necessary, is the standard of care for definitive treatment of ONB. Regional metastasis to the neck is often detected at presentation or may occur in a delayed fashion and should be addressed through elective neck dissection or radiation. Adjuvant radiotherapy should be considered, particularly in the case of high grade or tumor stage, as well as positive surgical margins. Systemic therapy is an area of active investigation in both the neoadjuvant and adjuvant setting, with many advocating in favor of induction chemotherapy for significant orbital or intracranial involvement prior to surgical resection. Various targeted immunotherapies are currently being studied for the treatment of recurrent or metastatic ONB. Prolonged locoregional and distant surveillance are indicated following definitive treatment, given the tendency for delayed recurrence and metastasis.
PubMed: 38673050
DOI: 10.3390/jpm14040423