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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 -
Current Oncology Reports Feb 2018To provide a comprehensive review of the literature focusing on the recent advances in the diagnosis and management of olfactory neuroblastoma. (Review)
Review
PURPOSE OF REVIEW
To provide a comprehensive review of the literature focusing on the recent advances in the diagnosis and management of olfactory neuroblastoma.
RECENT FINDINGS
Multimodality treatment is usually recommended for the majority of ONB cases. Recent advances in surgical approaches include the evolving role of endonasal endoscopic surgical resection and reconstruction. The introduction of new conformal radiation techniques has improved the outcomes and reduced treatment-related toxicity to important structures such as the eye and the brain. The role of neoadjuvant and adjuvant chemotherapy is yet to be defined. In the last two decades, there have been advances in surgical techniques with endoscopic approaches, either alone or in combination with craniotomy, gradually replacing the open traditional approaches. Prolonged surveillance is recommended for ONB due to late recurrences associated with that tumor. The role of chemotherapy and elective neck irradiation is still controversial and needs more studies to investigate their optimal role.
Topics: Animals; Combined Modality Therapy; Esthesioneuroblastoma, Olfactory; Humans; Neuroblastoma; Nose Neoplasms
PubMed: 29411144
DOI: 10.1007/s11912-018-0661-6 -
Advances in Oto-rhino-laryngology 2020Olfactory neuroblastoma is a rare tumor. Nasal endoscopy typically identifies a soft mass arising from the olfactory cleft. Computer tomography and magnetic resonance... (Review)
Review
Olfactory neuroblastoma is a rare tumor. Nasal endoscopy typically identifies a soft mass arising from the olfactory cleft. Computer tomography and magnetic resonance imaging are mandatory for staging (in association with 18F-fluorodeoxyglucose positron emission tomography) in high-grade and/or high-stage tumors. Biopsy must be representative to confirm a diagnosis and for grading purposes. Two complementary classifications are described: one (Kadish) based on clinical-radiological analysis, and the other (Hyams) on histological criteria. Based on Hyams grading, studies have pointed out that grades III-IV entail significantly different behavior and prognosis. A multimodal approach, which may combine surgery, chemotherapy, and radiotherapy, is essential to manage these tumors. Treatment schedules which include surgery seem to be superior to others. Surgery classically consisted of anterior craniofacial resection to obtain good exposure. However, the role of transnasal endoscopic surgery has expanded because of its association with fewer complications, shorter hospital stays, and comparable oncologic results to the open surgical techniques. Unilateral endoscopic craniectomy can be performed for limited lesions to avoid definitive anosmia. Treatment that includes radio- and chemotherapy is recommended for advanced and high-grade tumors. The role of neoadjuvant chemotherapy in advanced-stage lesions is emerging. The main prognostic factors associated with poor patient outcome are Hyams grade III-IV, Kadish C-D, and positive surgical margins. Lifelong follow up is recommended.
Topics: Combined Modality Therapy; Endoscopy; Esthesioneuroblastoma, Olfactory; Female; Humans; Male; Nasal Cavity; Nose Neoplasms; Prognosis
PubMed: 32731231
DOI: 10.1159/000457935 -
Virchows Archiv : An International... Apr 2024Olfactory neuroblastomas are uncommon malignancies that arise from olfactory receptor cells located high in the nasal cavity. Accurate diagnosis plays a crucial role in... (Review)
Review
Olfactory neuroblastomas are uncommon malignancies that arise from olfactory receptor cells located high in the nasal cavity. Accurate diagnosis plays a crucial role in determining clinical results and guiding treatment decisions. Diagnosis can be a major challenge for pathologists, especially when dealing with tumours with poor differentiation. The discovery of several molecular and immunohistochemical markers would help to overcome classification difficulties. Due to the paucity of large-scale studies, standardisation of diagnosis, treatment and prediction of outcome remains a challenge. Surgical resection by endoscopic techniques with the addition of postoperative irradiation is the treatment of choice. In addition, it is advisable to consider elective neck irradiation to minimise the risk of nodal recurrence. Molecular characterisation will help not only to make more accurate diagnoses but also to identify specific molecular targets that can be used to develop personalised treatment options tailored to each patient. The present review aims to summarise the current state of knowledge on histopathological diagnosis, the molecular biology and management of this disease.
Topics: Humans; Esthesioneuroblastoma, Olfactory; Nose Neoplasms; Nasal Cavity; Biomarkers, Tumor
PubMed: 38386106
DOI: 10.1007/s00428-024-03758-z -
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 -
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 -
Veterinary and Comparative Oncology Sep 2016Olfactory neuroblastoma (ONB) is a rare tumour of nasal cavity and paranasal sinuses that arises from the olfactory neuroepithelium and has unpredictable clinical... (Review)
Review
Olfactory neuroblastoma (ONB) is a rare tumour of nasal cavity and paranasal sinuses that arises from the olfactory neuroepithelium and has unpredictable clinical course. As the sense of smell is phylogenetically one of the first senses and olfactory neuroepithelium is evolutionary conserved with striking similarities among different species, we performed an extensive analysis of the literature in order to evaluate the similarities and differences between animals and humans on the clinical, morphological, immunohistochemical, ultrastructural and molecular level. Our analysis revealed that ONB was reported mainly in mammals and showed striking similarities to human ONB. These observations provide rationale for introduction of therapy modalities used in humans into the veterinary medicine. Animal models of neuroblastoma should be considered for the preclinical studies evaluating novel therapies for ONB.
Topics: Animals; Esthesioneuroblastoma, Olfactory; Humans; Nasal Cavity; Nose Neoplasms
PubMed: 25041470
DOI: 10.1111/vco.12102 -
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