Raras
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Estesioneuroblastoma
ORPHA:1957CID-10 · C30.0CID-11 · 2C20.3DOENÇA RARA

A estesioneuroblastoma é uma rara forma de câncer envolvendo a cavidade nasal e acredita-se que surja do epitélio olfatório. Pode causar a perda da visão e do paladar. Algumas vezes é considerado sinônimo do "neuroblastoma olfatório", mas o tecido de origem não tem está bem carecterizado.

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Introdução

O que você precisa saber de cara

📋

Tumor maligno raro originado na cavidade nasal superior (etmoide), podendo invadir órbita e base do crânio. Apresenta sintomas como obstrução nasal, epistaxe e alterações visuais.

Pesquisas ativas
6 ensaios
74 total registrados no ClinicalTrials.gov
Publicações científicas
966 artigos
Último publicado: 2026 Apr 9

Escala de raridade

CLASSIFICAÇÃO ORPHANET · BRASIL 2024
<1 / 1 000 000
Ultra-rara
<1/50k
Muito rara
1/20k
Rara
1/10k
Pouco freq.
1/5k
Incomum
1/2k
Prevalência
0.0
Europe
Início
Adult
+ elderly
🏥
SUS: Sem cobertura SUSScore: 0%
CID-10: C30.0
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Sinais e sintomas

O que aparece no corpo e com que frequência cada sintoma acontece

Linha do tempo da pesquisa

Publicações por ano — veja quando o interesse científico cresceu
Anos de pesquisa1desde 2026
Total histórico966PubMed
Últimos 10 anos200publicações
Pico202463 papers
Linha do tempo
2026Hoje · 2026🧪 1990Primeiro ensaio clínico📈 2024Ano de pico
Publicações por ano (últimos 10 anos)

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Genética e causas

O que está alterado no DNA e como passa nas famílias

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Nenhum gene associado encontrado

Os dados genéticos desta condição ainda estão sendo catalogados.

Diagnóstico

Os sinais que médicos procuram e os exames que confirmam

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Tratamento e manejo

Remédios, cuidados de apoio e o que precisa acompanhar

Pipeline de tratamentos
Pipeline regulatório — de medicamentos já aprovados a drogas em pesquisa exploratória.
Aprovado1
2Fase 26
1Fase 13
·Pré-clínico5
Medicamentos catalogadosEnsaios clínicos· 0 medicamentos · 15 ensaios
Carregando informações de tratamento...

Onde tratar no SUS

Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)

🇧🇷 Atendimento SUS — Estesioneuroblastoma

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Dados de DATASUS/CNES, SBGM, ABNeuro e Ministério da Saúde. Sempre confirme a disponibilidade diretamente com o estabelecimento.

Pesquisa ativa

Ensaios clínicos abertos e novidades científicas recentes

🟢 Recrutando agora

6 pesquisas recrutando participantes. Converse com seu médico sobre a possibilidade de participar.

Outros ensaios clínicos

74 ensaios clínicos encontrados, 6 ativos.

Distribuição por fase
Ver todos no ClinicalTrials.gov
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Publicações mais relevantes

📖Melhor nível de evidência: Revisão
Timeline de publicações
380 papers (10 anos)
#1

Nationwide analysis of olfactory neuroblastoma in Japan: evolving treatment approaches and prognostic outcomes.

Japanese journal of clinical oncology2026 Mar 13

Olfactory neuroblastoma (ONB) is a rare malignant tumor of the nasal cavity and paranasal sinuses. In this study, we aimed to analyze ONB cases registered in the nationwide Head and Neck Cancer Registry of Japan. Among 90 885 head and neck cancer registrations (2011-19), we identified 346 patients with ONB. We summarized demographics, tumor-node-metastasis (TNM) classification, and treatment modalities (surgery, radiotherapy, chemotherapy) and compared patterns between an early (2011-15) and a late (2016-19) period. Survival was analyzed in 95 patients with standardized 5-year outcomes available. T4 lesions were frequent, and 234 patients (67.6%) received surgery-based treatment, typically combined with postoperative radiotherapy. Over time, endoscopic approaches increased markedly and became predominant over open skull base surgery. Among the 95 patients with evaluable follow-up, the 5-year overall survival (OS) and 5-year recurrence-free survival (RFS) were 85.1% and 62.7%, respectively. Patients <60 years old and female patients exhibited better OS compared to younger patients and males. Postoperative radiotherapy was associated with improved RFS but not OS. Chemotherapy was used more often with open skull base surgery than with other surgical approaches. Endoscopic surgery for ONB rose substantially, while younger age and female sex were associated with better OS, and postoperative radiotherapy was correlated with improved RFS.

#2

Proton Beam vs Intensity-Modulated Radiotherapy in Olfactory Neuroblastoma.

JAMA otolaryngology-- head &amp; neck surgery2026 Jan 01

Adjuvant radiotherapy can improve locoregional control and survival in patients with olfactory neuroblastoma (ONB), particularly with advanced-stage and histologic-grade disease. Standard radiotherapy treatment is with intensity-modulated radiotherapy (IMRT). Proton beam radiotherapy (PBRT) provides theoretical advantages in greater sparing of dose to uninvolved organs at risk. To investigate if there are differences in the effectiveness and radiation treatment-related adverse events (RTAEs) between adjuvant IMRT and PBRT for patients with ONB. This propensity score-matched cohort study included patients with ONB treated between February 2005 and April 2021 with either IMRT or PBRT at 9 academic tertiary care centers in North America. Patients were matched 1:2 based on age, modified Kadish stage, and Hyams grade. Data were analyzed from July 2024 to January 2025. Adjuvant IMRT or adjuvant PBRT. Local recurrence-free survival (RFS), any RFS, and overall survival (OS). RTAEs, ie, grade 2 events or higher based on Common Terminology Criteria for Adverse Events, were recorded for both modalities. Of 54 included patients, 27 (50%) were female, and the mean (SD) age was 46.2 (15.4) years. A total of 18 were treated with PBRT and 36 were treated with IMRT. Most patients had modified Kadish stage C disease (33 of 54 [61%]), and 24 patients (44%) had Hyams grade III or IV disease. The RTAE rate was 20% (8 of 40); IMRT had a rate of 21% (6 of 29), and PBRT had a rate of 18% (2 of 11). The difference in the point estimates for 10-year RFS showed a potential clinical benefit favoring IMRT, although the wide confidence interval indicates uncertainty (10-year RFS: IMRT, 63.3%; 95% CI, 44.6-89.8; PBRT, 37.8%; 95% CI, 14.2-100; difference, 25.5 percentage points; 95% CI, -17.6 to 68.6). There were no clinically meaningful differences in 10-year local RFS (IMRT, 75.6%; 95% CI, 59.8-95.4; PBRT, 72.7%; 95% CI, 45.2-100; difference, 2.9 percentage points; 95% CI, -35.9 to 41.7) or 10-year OS (IMRT, 61.8%; 95% CI, 42.8-89.1; PBRT, 57.1%; 95% CI, 24.3-100; difference, 4.7 percentage points; 95% CI, -49.2 to 58.6), although wide confidence intervals indicate considerable uncertainty. Due to the imprecision of estimates, no definitive conclusions can be made regarding the comparative effectiveness of IMRT vs PBRT for patients with ONB. These preliminary data may inform the design of appropriately powered prospective studies evaluating the efficacy of PBRT vs IMRT in this population.

#3

DNA Virus Detection in Olfactory Neuroblastomas Using Targeted Enrichment NGS.

Neuropathology : official journal of the Japanese Society of Neuropathology2026 Apr

A variety of malignancies can be found in the sinonasal tract including the nasal cavity, paranasal sinuses, and skull base. Many of these are attributed to viruses, for example, carcinomas with the presence of transcriptionally active high-risk human papillomavirus, as well as Epstein-Barr virus associated malignant lymphoepithelial carcinoma and haematolymphoid neoplasias. Olfactory neuroblastoma (ONB) is a rare malignant tumor of the olfactory fossa with an unknown etiology. We present the first comprehensive virus study of ONB. By targeted next-generation sequencing for 41 DNA viruses, we investigated the presence of papillomaviruses, herpesviruses, polyomaviruses, and parvoviruses, among others, in 12 ONBs. In addition, herpesvirus presence was evaluated by qPCR. Low-risk HPV6 was detected in one sample. Human endogenous retroviruses were positive in all samples, supporting successful library preparation and sequencing, considered as an internal quality control. No other virus findings were observed. The present broad-scale virus study did not reveal abundant presence of viral DNA in ONB suggesting a limited viral role in its etiology. Nevertheless, our cohort exhibited a finding of low-risk HPV, which has been associated with increased risk of cancer progression of inverted sinonasal papilloma in the same anatomic site advocating for further research. Trial Registration: §31/07.03.2019.

#4

Surgical Management of Esthesioneuroblastoma at a Single Tertiary Care Center.

Journal of neurological surgery. Part B, Skull base2026 Apr

To analyze management strategies and outcomes for patients with esthesioneuroblastoma (ENB) undergoing surgical resection at a single institution. Retrospective review from 1971 to 2022 from a single, high-volume tertiary academic center of all patients with ENB. A total of 60 patients received their primary treatment for ENB at our institution. The average age at diagnosis was 52 years (range 13-91), and most were male (66.7%) with Kadish C (61.7%) stage at presentation. Most patients were treated with an open approach (68.3%) compared with a purely endoscopic approach (31.7%). Median follow-up was 96.9 months (mean 118.8 months, interquartile range 28.4-183.8 months). There was one patient with local recurrence in the endoscopic cohort (5.3%) compared with 22.0% (9 patients) among those who underwent an open resection ( p  = 0.21). The 5-year survival was 87.2% (95% CI, 71.9-100.0%) in the endoscopic cohort compared with 80.2% (95% CI, 68.8-93.5%) in the open group ( p  = 0.60). The rate of death or recurrence within 5 years of treatment was comparable between open and endoscopic (32.5% vs. 15.0%, p  = 0.26). Among Kadish C patients treated in the endoscopic era (after 2006), 5-year survival was 76.2% (95% CI, 52.1-100.0%) in the endoscopic cohort and 64.8% (95% CI, 39.3-100.0%) in the open group ( p  = 0.70). The surgical approach for ENB resection is dictated by tumor extension. Endoscopic resection offers a less invasive approach with comparable postoperative outcomes in appropriately selected patients.

#5

Orbital rhabdomyosarcoma in an adult: a misleading presentation.

Radiology case reports2026 May

Rhabdomyosarcoma is the most common malignant mesenchymal tumor in children, whereas its occurrence in adults is exceptionally rare. When it occurs in this population, initial presentation with nonspecific rhinologic symptoms is unusual and may delay recognition of the underlying malignancy. We report the case of a 29-year-old woman who presented with progressive right-sided nasal obstruction and hyposmia, subsequently complicated by the rapid onset of unilateral proptosis. Cross-sectional imaging revealed a poorly circumscribed ethmoido-orbito-nasal mass with skull base erosion and orbital invasion, exhibiting aggressive radiologic features that initially raised suspicion for esthesioneuroblastoma. Histopathological examination ultimately confirmed alveolar rhabdomyosarcoma, staged as T2BN1M0 (IRS Group III). The patient underwent multimodal treatment combining VAC chemotherapy and conformal radiotherapy (45 Gy), resulting in an excellent clinical and radiologic response, with complete resolution of proptosis and significant visual recovery at 1-year follow-up. This case emphasizes the diagnostic challenges of adult orbital rhabdomyosarcoma with sinonasal extension and highlights the pivotal role of advanced imaging and histopathology in guiding accurate diagnosis and appropriate management. The rarity and unusual clinical presentation of this case underscore its value for publication.

Publicações recentes

Ver todas no PubMed

📚 EuropePMC602 artigos no totalmostrando 200

2025

Diagnosis and management of esthesioneuroblastoma.

La Tunisie medicale
2026

DNA Virus Detection in Olfactory Neuroblastomas Using Targeted Enrichment NGS.

Neuropathology : official journal of the Japanese Society of Neuropathology
2026

Surgical Management of Esthesioneuroblastoma at a Single Tertiary Care Center.

Journal of neurological surgery. Part B, Skull base
2026

Orbital rhabdomyosarcoma in an adult: a misleading presentation.

Radiology case reports
2026

Effect of Preoperative Superselective Embolization in the Resection of Hypervascular Tumors of the Central Nervous System, Neck, and Nasal Cavity.

Cureus
2026

Skull Base Surgery in the Pediatric Population-The 2nd International Collaborative Study (1995-2015).

Head &amp; neck
2026

Endoscopic endonasal approach with extended orbital roof removal for a giant esthesioneuroblastoma: illustrative case.

Journal of neurosurgery. Case lessons
2026

Combined Endoscopic and Open Cranial Approach for Treatment of Skull Base Lesions: A Case Series and Lessons Learned.

Journal of neurological surgery. Part B, Skull base
2025

Neoadjuvant Chemotherapy-Induced Downstaging Enables R0 Craniofacial Combined Resection in AJCC (American Joint Committee on Cancer) Stage IV Olfactory Neuroblastoma: A Case Report of 12-Year Disease-Free Survival.

Cureus
2026

Nationwide analysis of olfactory neuroblastoma in Japan: evolving treatment approaches and prognostic outcomes.

Japanese journal of clinical oncology
2025

Kadish C/Hyams II Esthesioneuroblastoma With Atypical Immunophenotype Mimicking NK/T-Cell Lymphoma: Endoscope-Assisted Transcranial Resection and Adjuvant Intensity-Modulated Radiotherapy.

Cureus
2025

Lutetium-177 DOTATATE for the Treatment of Esthesioneuroblastoma: A Case Report.

Case reports in oncological medicine
2026

Genomic and Immune Correlates of EZH2 Expression and Activity in Olfactory Neuroblastoma.

Head &amp; neck
2026

Tonsillar Olfactory Neuroblastoma With Cervical Node Involvement: A Rare Case and Literature Review.

The Laryngoscope
2026

Proton Beam vs Intensity-Modulated Radiotherapy in Olfactory Neuroblastoma.

JAMA otolaryngology-- head &amp; neck surgery
2025

Long-term Outcomes of Carbon-ion Radiotherapy for Inoperable Locally Advanced Olfactory Neuroblastoma.

Anticancer research
2025

Patterns of lymph node recurrence in sinonasal cancers: risk factors and oncological outcomes.

European archives of oto-rhino-laryngology : official journal of the European Federation of Oto-Rhino-Laryngological Societies (EUFOS) : affiliated with the German Society for Oto-Rhino-Laryngology - Head and Neck Surgery
2025

Successful Management of Advanced Olfactory Esthesioneuroblastoma: A Case Report.

Clinical case reports
2025

Characterization of Sinonasal Lesions on CT, MRI, and PET/CT.

Clinical nuclear medicine
2025

Survival Outcome for Peptide Receptor Radionuclide Therapy in Olfactory Neuroblastoma.

Anticancer research
2026

Prognostic Factors in Sinonasal Cancers: A Multicenter Pooled Analysis.

The Laryngoscope
2025

Esthesioneuroblastoma: Unraveling the Enigma and Emerging Paradigms With a Retrospective Cohort and Review of Literature.

Cureus
2025

Refinement of Hyams Grading Criteria for Olfactory Neuroblastoma in an Endoscopic Sinus Surgery Predominant Cohort with Extended Follow-up: Worth the Effort?

Head and neck pathology
2025

Updated Insights into the Molecular Pathophysiology of Olfactory Neuroblastoma Using Multi-Omics Analysis.

Journal of personalized medicine
2025

Revisiting intra-arterial chemotherapy-palliative utility in a case of recurrent esthesioneuroblastoma and literature review.

Interventional neuroradiology : journal of peritherapeutic neuroradiology, surgical procedures and related neurosciences
2025

[Comparison of the modified Kadish, AJCC T and Dulguerov T staging systems for olfactory neuroblastoma: analysis of the SEER database].

Zhonghua er bi yan hou tou jing wai ke za zhi = Chinese journal of otorhinolaryngology head and neck surgery
2025

Enhancing Diagnostic Accuracy in Sinonasal Neuroendocrine Tumors: The Case for DOTATATE PET Over FDG PET.

International forum of allergy &amp; rhinology
2025

SIADH as an uncommon presentation of olfactory neuroblastoma: a case-based overview.

Endocrine oncology (Bristol, England)
2025

Extent of Resection and Role of Adjuvant Radiotherapy in Olfactory Neuroblastoma Without Definite Radiologic Evidence of Dural Invasion.

International forum of allergy &amp; rhinology
2025

Hypoosmolar Hyponatremia as Presenting Symptom of a Rare Case of Olfactory Neuroblastoma.

JCEM case reports
2025

Cancer Stem Cell Characterization in Olfactory Neuroblastoma Tissue.

International forum of allergy &amp; rhinology
2025

Ectopic adrenocorticotropic hormone syndrome due to olfactory neuroblastoma: A case report and literature review.

Endocrinologia, diabetes y nutricion
2025

Endoscopic surgery versus various open approaches in esthesioneuroblastoma: a systematic review of the literature.

Frontiers in oncology
2025

Hyams Grade and Ki-67 as Predictive Factors for Primary Treatment Failure in Olfactory Neuroblastoma.

The Laryngoscope
2025

Frontal Lobe Herniation After Endoscopic Anterior Skull Base Resection: A Rare Complication.

Ear, nose, &amp; throat journal
2025

The 100 most-cited papers on esthesioneuroblastoma: a bibliometric analysis.

Annals of medicine and surgery (2012)
2025

Metastatic olfactory neuroblastoma with unusual solitary metastasis to femur: a case report and literature review.

World journal of surgical oncology
2025

Isocitrate Dehydrogenase 2 (IDH2)-Mutant Metastatic Esthesioneuroblastoma: A Case Report and Review of Current Therapeutic Options.

Cureus
2025

Somatostatin Receptor-Directed Theranostics in Esthesioneuroblastoma.

Clinical nuclear medicine
2025

Deep learning models for differentiating three sinonasal malignancies using multi-sequence MRI.

BMC medical imaging
2025

Forty-Year Long-Term Outcome After Endoscopic and Open Surgery for Esthesioneuroblastoma in Consideration of Prognostic Factors.

Cancers
2025

Endovascular management of carotid blowout syndrome.

Neurological research
2025

Unilateral Versus Bilateral Endoscopic Resection of Olfactory Neuroblastoma: Pooled Analysis From Prospective and Retrospective Multicenter Data.

International forum of allergy &amp; rhinology
2024

Uncommon Nasal Mass Presentation: A Radiological Case Series.

Journal of personalized medicine
2025

Olfactory neuroblastoma: literature review and presentation of two clinical cases with different tumour infiltrations and different approaches.

Neurocirugia
2025

Ectopic olfactory neuroblastoma is associated with increased frequency of syndrome of inappropriate antidiuretic hormone secretion and reduced disease control: Case series with systematic review and pooled analysis.

International forum of allergy &amp; rhinology
2025

The added value of narrow band imaging in Sinonasal tumour resection and surveillance: Our experience.

American journal of otolaryngology
2024

An Uncommon Encounter: Metastatic Olfactory Neuroblastoma in an Adult Male.

Cureus
2025

Analysis of ASCL1/NEUROD1/POU2F3/YAP1 Yields Novel Insights for the Diagnosis of Olfactory Neuroblastoma and Identifies Sinonasal Tuft Cell-Like Carcinoma.

Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc
2025

Recent developments in olfactory neuroblastoma research.

Current opinion in otolaryngology &amp; head and neck surgery
2025

Morphological variability and its impact on survival in sinonasal malignancies: a 13-year analysis.

European archives of oto-rhino-laryngology : official journal of the European Federation of Oto-Rhino-Laryngological Societies (EUFOS) : affiliated with the German Society for Oto-Rhino-Laryngology - Head and Neck Surgery
2024

Single-cell transcriptomic landscape deciphers olfactory neuroblastoma subtypes and intra-tumoral heterogeneity.

Nature cancer
2024

Olfactory Neuroblastoma With Divergent Differentiation: Contemporary Management of Unusual Pathology and Literature Review.

Ear, nose, &amp; throat journal
2025

Pathologic dural invasion is associated with regional recurrence in olfactory neuroblastoma: A multi-institutional study.

International forum of allergy &amp; rhinology
2024

Olfactory Neuroblastoma and Olfactory Carcinoma: A Practical Review.

Surgical pathology clinics
2024

An adolescent girl with syndrome of inappropriate antidiuretic hormone secretion preceding the diagnosis of olfactory neuroblastoma - a case report.

Frontiers in endocrinology
2024

The value of elective neck irradiation in management of esthesioneuroblastoma: a retrospective study based on propensity score matching.

Radiation oncology (London, England)
2024

Olfactory Carcinoma, Similar yet Distinct to Olfactory Neuroblastoma: A Case Report and Literature Review.

ORL; journal for oto-rhino-laryngology and its related specialties
2024

Radiologic overview of sinonasal lesions.

Frontiers in radiology
2024

[Genomic profiles and immune microenvironment of olfactory neuroblastoma].

Zhonghua bing li xue za zhi = Chinese journal of pathology
2024

[Olfactory neuroblastoma in children: clinical analysis of five cases].

Zhonghua yi xue za zhi
2024

Ectopic Olfactory Neuroblastoma Arising in the Nasopharynx.

The Journal of craniofacial surgery
2024

Olfactory neuroblastoma with orbital protrusion arising in the background of maxillary carcinoma.

Journal of surgical case reports
2024

Survival Analysis and Prognostic Factors After Endonasal Resection of Advanced Olfactory Neuroblastomas: A Single Institution Experience.

Journal of otolaryngology - head &amp; neck surgery = Le Journal d'oto-rhino-laryngologie et de chirurgie cervico-faciale
2024

Role of induction chemotherapy in advanced-stage olfactory neuroblastoma.

International forum of allergy &amp; rhinology
2024

Esthesioneuroblastoma: 38 years of experience in an oncological center.

Reports of practical oncology and radiotherapy : journal of Greatpoland Cancer Center in Poznan and Polish Society of Radiation Oncology
2024

Olfaction Preservation and Long-Term Outcomes in Patients with Unilateral Endoscopic Resection of Olfactory Neuroblastoma: A Systematic Review and Institutional Experience.

World neurosurgery
2025

A prospective cohort study on stereotactic radiotherapy in the management of dural recurrence of olfactory neuroblastoma.

Head &amp; neck
2024

Exploring the Impact of Artificial Intelligence and Machine Learning in the Diagnosis and Management of Esthesioneuroblastomas: A Comprehensive Review.

Cureus
2024

Olfactory neuroblastoma: A rare sinonasal malignancy.

JPMA. The Journal of the Pakistan Medical Association
2024

Olfactory dysfunction management following unilateral cranial resection for olfactory neuroblastoma.

Rhinology
2024

Ectopic adrenocorticotropic hormone syndrome in patients with olfactory neuroblastoma.

Endocrine-related cancer
2024

A case of olfactory ganglioneuroblastoma in a dog: immunohistochemical comparison with olfactory neuroepithelia and olfactory neuroblastomas.

The Journal of veterinary medical science
2024

A Case of Olfactory Neuroblastoma Developing Bilateral Retropharyngeal Lymph Node Metastasis 14-years After Skull Base Surgery.

The Kobe journal of medical sciences
2024

Nasal Cavity and Paranasal Sinus Cancer: Diagnosis and Treatment.

Current oncology reports
2024

Endoscopy-assisted medial canthus incision for olfactory neuroblastoma: a case report.

World journal of surgical oncology
2024

The Prognostic Implications of Neutrophil-to-Lymphocyte Ratio in Olfactory Neuroblastoma.

Otolaryngology--head and neck surgery : official journal of American Academy of Otolaryngology-Head and Neck Surgery
2024

Long non-coding RNA MALAT 1 and PHOX2B expression in olfactory neuroblastomas and sympathetic neuroblastomas.

Annals of diagnostic pathology
2024

[Clinical analysis of endonasal endoscopic surgery in esthesioneuroblastoma].

Lin chuang er bi yan hou tou jing wai ke za zhi = Journal of clinical otorhinolaryngology head and neck surgery
2024

Olfactory Neuroblastoma Is Not Always Located at the Roof of the Nasal Cavity.

Journal of the Belgian Society of Radiology
2024

Augmentation of tumor expression of HLA-DR, CXCL9, and CXCL10 may improve olfactory neuroblastoma immunotherapeutic responses.

Journal of translational medicine
2024

Olfactory neuroblastoma mimics molecular heterogeneity and lineage trajectories of small-cell lung cancer.

Cancer cell
2024

Development of an evaluation and treatment strategy for olfactory neuroblastoma: a review of evidence from large-scale studies, including population-based and multicenter studies, and meta-analyses.

Japanese journal of clinical oncology
2024

Feasibility of Optical Biopsy During Endoscopic Sinus Surgery With Confocal Laser Endomicroscopy: A Pilot Study.

The Laryngoscope
2024

Clinical outcomes for olfactory neuroblastoma.

Frontiers in oncology
2024

Exophthalmos revealing an olfactory esthesioneuroblastoma: A case report.

International journal of surgery case reports
2024

Recurrence morbidity of olfactory neuroblastoma.

International forum of allergy &amp; rhinology
2024

Treatment of unilateral olfactory neuroblastoma: Appropriate extent of surgical resection and potential for olfactory preservation.

International forum of allergy &amp; rhinology
2024

Review of neoplasia in fish at a large display aquarium, 2005-2021.

Journal of veterinary diagnostic investigation : official publication of the American Association of Veterinary Laboratory Diagnosticians, Inc
2024

Patterns of recurrence and disease progression in patients with positive-margin olfactory neuroblastoma following primary resection.

Journal of neurosurgery
2024

Neuroendocrine and undifferentiated sinonasal and skull base tumors: An up-to-date narrative review.

Oral and maxillofacial surgery
2024

The impact of multidisciplinary approaches on the outcomes of olfactory neuroblastoma treated with postoperative radiotherapy.

Cancer medicine
2023

A rare presentation of esthesioneuroblastoma: a case report and review of the literature.

The Pan African medical journal
2023

Esthesioneuroblastoma presenting as syndrome of inappropriate antidiuretic hormone secretion.

La Tunisie medicale
2024

To Study the Efficacy of Coblation in the Endoscopic Surgery of Sinonasal and Skull Base Masses.

Indian journal of otolaryngology and head and neck surgery : official publication of the Association of Otolaryngologists of India
2024

Treatment Outcomes of Olfactory Neuroblastoma: A Multicenter Study by the Korean Sinonasal Tumor and Skull Base Surgery Study Group.

Clinical and experimental otorhinolaryngology
2024

Update on olfactory neuroblastoma.

Virchows Archiv : an international journal of pathology
2024

SNOT-22 subdomain outcomes following treatment for sinonasal malignancy: A prospective, multicenter study.

International forum of allergy &amp; rhinology
2024

Recurrent Wnt Pathway and ARID1A Alterations in Sinonasal Olfactory Carcinoma.

Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc
2024

Upright proton therapy for esthesioneuroblastoma: a single-institution experience.

Frontiers in oncology
2024

Characterizing Immune Infiltration in Esthesioneuroblastoma Subtypes Through Gene Expression Deconvolution.

World neurosurgery
2024

The role of insulinoma-associated protein 1 in predicting the progression and prognosis of human olfactory neuroblastoma in China.

Pathology, research and practice
2024

The syndrome of inappropriate antidiuretic hormone associated with nasal and paranasal malignant tumors.

European archives of oto-rhino-laryngology : official journal of the European Federation of Oto-Rhino-Laryngological Societies (EUFOS) : affiliated with the German Society for Oto-Rhino-Laryngology - Head and Neck Surgery
2024

The medial transorbital approach in cranioendoscopic skull base tumor resections for locally advanced tumors.

Journal of clinical neuroscience : official journal of the Neurosurgical Society of Australasia
2024

Oncology for the rhinologist.

Current opinion in otolaryngology &amp; head and neck surgery
2023

Rare case of mortality after hemorrhagic property of esthesioneuroblastoma.

Archive of clinical cases
2023

Genomic characterization of an esthesioneuroblastoma with spinal metastases: illustrative case.

Journal of neurosurgery. Case lessons
2024

Simple and safe resection of the crista galli.

Head &amp; neck
2023

Peptide Receptor Radionuclide Therapy for Recurrent Olfactory Neuroblastoma After Cranioplasty for Surgical Infection: A Case Report.

Anticancer research
2024

Outcomes following proton therapy for pediatric esthesioneuroblastoma.

Pediatric blood &amp; cancer
2024

Predictability of Olfactory Neuroblastoma Staging Systems-Reply.

JAMA otolaryngology-- head &amp; neck surgery
2024

Predictability of Olfactory Neuroblastoma Staging Systems.

JAMA otolaryngology-- head &amp; neck surgery
2023

Failure Patterns of Recurrence in Patients With Localized Esthesioneuroblastoma Following Surgery and Adjuvant Radiotherapy Without Elective Nodal Irradiation.

Cureus
2023

Olfactory neuroblastoma: diagnosis, management, and current treatment options.

Frontiers in oncology
2023

Primary Sellar Neuroblastoma Masquerading as a Pituitary Macroadenoma.

JCEM case reports
2023

[Chinese expert consensus on pathological diagnosis of olfactory neuroblastoma(2023 version)].

Zhonghua bing li xue za zhi = Chinese journal of pathology
2023

Olfactory schwannoma masquerading as esthesioneuroblastoma.

BMJ case reports
2025

Syndrome of Inappropriate Antidiuretic Hormone Release Secondary to Ectopic Olfactory Neuroblastoma: Case Report and Review of Literature.

Ear, nose, &amp; throat journal
2023

Anterior Skull Base Osteoradionecrosis in the Age of Intensity-Modulated Radiation Therapy: A Case Series.

Journal of neurological surgery reports
2024

Induction Chemotherapy for Sinonasal Tumors in Patients Presenting with Brain Invasion and/or Neurological Deficits.

Journal of neurological surgery. Part B, Skull base
2023

Solid and Vascular Neck Masses in Children.

Neuroimaging clinics of North America
2024

Endoscopic management of sinonasal tumours in the Nordic university hospitals: a survey.

European archives of oto-rhino-laryngology : official journal of the European Federation of Oto-Rhino-Laryngological Societies (EUFOS) : affiliated with the German Society for Oto-Rhino-Laryngology - Head and Neck Surgery
2023

A Rare Case of Sinonasal Ewing Sarcoma With Radiologic-Pathologic Correlation.

Cureus
2024

International Consensus Statement on Allergy and Rhinology: Sinonasal Tumors.

International forum of allergy &amp; rhinology
2023

Understanding frozen section histopathology in Sinonasal and anterior skull base malignancy and proposed reporting guidelines.

Journal of surgical oncology
2023

Small Round Blue Cell Tumours of the Sinonasal Area: Our 5 year Experience in a Tertiary Care Centre in India.

Indian journal of otolaryngology and head and neck surgery : official publication of the Association of Otolaryngologists of India
2023

Clinical relevance of hyponatraemia in olfactory neuroblastoma.

The Indian journal of medical research
2023

Esthesioneuroblastoma: Management of the N0 neck.

Journal of neurological surgery reports
2023

Setting the Stage for Olfactory Neuroblastoma.

JAMA otolaryngology-- head &amp; neck surgery
2023

Multicenter Survival Analysis and Application of an Olfactory Neuroblastoma Staging Modification Incorporating Hyams Grade.

JAMA otolaryngology-- head &amp; neck surgery
2023

Treatment outcomes and prognostic factors of esthesioneuroblastoma-a retrospective study from South India.

Ecancermedicalscience
2023

Stereotactic radiosurgery for distant brain metastases secondary to esthesioneuroblastoma: a single-institution series.

Neurosurgical focus
2023

Olfactory neuroblastoma in a domestic cat and review of the literature.

Veterinary clinical pathology
2023

Real world data on long term outcome of neoadjuvant chemotherapy in locally advanced esthesioneuroblastoma and sinonasal tumor with neuroendocrine differentiation - Results from a single centre study.

Oral oncology
2023

CyberKnife Radiosurgery for Spinal Leptomeningeal Metastases Secondary to Esthesioneuroblastoma: A Clinical Case Report.

Cureus
2023

Deconstructing Olfactory Epithelium Developmental Pathways in Olfactory Neuroblastoma.

Cancer research communications
2024

DOTATATE PET Imaging in Olfactory Neuroblastoma and Association with SSTR Expression.

Journal of neurological surgery. Part B, Skull base
2023

Comparing the role of different treatment modalities in locoregionally advanced olfactory neuroblastoma: The 20-year clinical experience of a single institution.

Head &amp; neck
2024

A multi-institutional retrospective study of 340 cases of sinonasal malignant tumor.

Auris, nasus, larynx
2023

An atypical Esthesioneuroblastoma of the sphenoid sinus: a case report.

Annals of medicine and surgery (2012)
2024

Clinical Outcome in Patients with Large Sinonasal Tumors with Intracranial Extension.

Journal of neurological surgery. Part B, Skull base
2023

Proceedings of the 2023 North American Society of Head and Neck Pathology Companion Meeting, New Orleans, LA, March 12, 2023: Navigating New Developments in High Grade Sinonasal Neuroendocrine and Neuroectodermal Neoplasms.

Head and neck pathology
2023

Determining the association of insurance coverage and survival outcomes in patients with olfactory neuroblastoma utilizing machine learning.

International forum of allergy &amp; rhinology
2023

Olfactory Neuroblastoma-A Challenging Fine Line between Metastasis and Hematology.

Medicina (Kaunas, Lithuania)
2023

[Neoadjuvant chemotherapy in the treatment of locally advanced olfactory neuroblastoma in 25 cases].

Zhonghua er bi yan hou tou jing wai ke za zhi = Chinese journal of otorhinolaryngology head and neck surgery
2023

Olfactory neuroblastoma with rhabdomyoblasts: A rare case report and review of the literature.

Indian journal of pathology &amp; microbiology
2023

Metastatic esthesioneuroblastoma with widespread recurrence to the central nervous system.

Surgical neurology international
2023

Clinical Outcomes and Patterns of Failure in Esthesioneuroblatoma: A Single Institutional Experience.

Indian journal of otolaryngology and head and neck surgery : official publication of the Association of Otolaryngologists of India
2023

Lymph node metastasis from olfactory neuroblastoma at presentation and as disease relapse: A systematic review and proportion meta-analysis of prevalence data and variables influencing regional control.

Head &amp; neck
2023

Recurrent Esthesioneuroblastoma: Long-Term Outcomes of Salvage Therapy.

Cancers
2023

Radiographic predictors of occult intracranial involvement in olfactory neuroblastoma patients.

International forum of allergy &amp; rhinology
2023

Molecular Evidence for Olfactory Neuroblastoma as a Tumor of Malignant Globose Basal Cells.

Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc
2022

Ga-68 DOTATATE PET/CT in the Evaluation of Paragangliomas and Other Indeterminate Lesions in the Head and Neck.

Indian journal of nuclear medicine : IJNM : the official journal of the Society of Nuclear Medicine, India
2023

Operative Technique and Complication Management in a Case of Giant Esthesioneuroblastoma Resected by a Combined Transcranial and Endonasal Endoscopic Approach: Technical Case Report.

Operative neurosurgery (Hagerstown, Md.)
2025

Sinonasal Schwannomas: Imaging Findings and Review of Literature.

Ear, nose, &amp; throat journal
2023

Prognostic significance of PD-1, CTLA-4, CD4, and CD8 expression in olfactory neuroblastoma.

Clinical neuropathology
2023

The Role of Induction Therapy for Sinonasal Cancers.

Current treatment options in oncology
2023

An exceptionally rare case of Cushing's syndrome caused by ectopic ACTH syndrome due to olfactory neuroblastoma in childhood.

Journal of pediatric endocrinology &amp; metabolism : JPEM
2024

Ectopic Olfactory Neuroblastoma: Systematic Review of a Rare Clinical Entity among Sinonasal Tumors.

Journal of neurological surgery. Part B, Skull base
2023

Long-term survival outcomes and recurrence patterns of olfactory neuroblastoma: A 13-year experience at a single institution.

Auris, nasus, larynx
2022

Carbon-ion radiotherapy in the treatment of radiation-induced second primary malignancies.

Annals of translational medicine
2022

Olfactory Neuroblastoma: A Novel Site of Presentation.

Indian journal of otolaryngology and head and neck surgery : official publication of the Association of Otolaryngologists of India
2022

A Review on Esthesioneuroblastoma.

Indian journal of otolaryngology and head and neck surgery : official publication of the Association of Otolaryngologists of India
2023

Olfactory Neuroblastoma: Morphological Reappraisal and Molecular Insights with Quantum Leap in Clinical Perspectives.

Current oncology reports
2023

Immunohistochemical Profiling and Staging in Esthesioneuroblastoma: A Single-Center Cohort Study and Systematic Review.

World neurosurgery
2023

Cytological diagnosis of olfactory neuroblastoma at metastatic sites, with emphasis on role of insulinoma-associated protein 1 immunocytochemistry.

Diagnostic cytopathology
2022

The role of ADC value and Ki-67 index in predicting the response to neoadjuvant chemotherapy in advanced stages of olfactory neuroblastoma.

The British journal of radiology
2022

DNA Methylation Profiling in Rare Sellar Tumors.

Biomedicines
2022

Idiopathic SIADH: 4 years of diagnostic wandering.

European journal of nuclear medicine and molecular imaging
2022

Metastatic esthesioneuroblastoma recurrence after 19 years of remission: A systematic review with case illustration.

Journal of the neurological sciences
2023

Expressions of NeuroD and GAP43 as diagnostic markers for olfactory neuroblastoma.

Auris, nasus, larynx
2023

The contemporary management of cancers of the sinonasal tract in adults.

CA: a cancer journal for clinicians
2022

Esthesioneuroblastoma (Olfactory Neuroblastoma): Overview and Extent of Surgical Approach and Skull Base Resection.

Journal of neurological surgery reports
2022

The prognostic value of S-100 protein and Ki-67 index in olfactory neuroblastoma.

Japanese journal of clinical oncology
2022

Application of the subdural evacuating port system for the drainage of postoperative tension pneumocephalus: A technical note.

Surgical neurology international
2022

Frontal lobe position after single-layer cadaveric dermal matrix repair of large anterior skull base defects.

World journal of otorhinolaryngology - head and neck surgery
2022

Exclusively endoscopic surgical resection of esthesioneuroblastoma: A systematic review.

World journal of otorhinolaryngology - head and neck surgery
2022

Olfactory Neuroblastomas: What Actually Happens in the Long-Term?

Journal of clinical medicine
2022

Minimally invasive pericranial flap harvest through a supraorbital eyebrow craniotomy: Technical note in salvage skull base reconstruction.

Clinical neurology and neurosurgery
2022

Nasoethmoidal Schwannoma as a Mimicar of Esthesioneuroblastoma: A Case Report and Literature Review.

Neurology India
2022

Transcription Factor Expression in Sinonasal Neuroendocrine Neoplasms and Olfactory Neuroblastoma (ONB): Hyams' Grades 1-3 ONBs Expand the Spectrum of SATB2 and GATA3-Positive Neoplasms.

Endocrine pathology
2022

Case Report: Esthesioneuroblastoma Involving the Optic Pathways.

Frontiers in surgery
2023

Impact of esthesioneuroblastoma treatment delays on overall patient survival.

The Laryngoscope
2023

Magnetic resonance imaging findings in patients with idiopathic olfactory dysfunction and normal findings on nasoendoscopy.

The Journal of laryngology and otology
2022

Management of Esthesioneuroblastoma: A Retrospective Study of 6 Cases and Literature Review.

Case reports in oncology
2022

Sinonasal Tumors With Neuroepithelial Differentiation (Olfactory Carcinoma): Delineation of Their Pathologic and Clinical Features With Insights into Their Relationship to Olfactory Neuroblastoma and Sinonasal Carcinoma.

The American journal of surgical pathology
2022

Combined Nasal Endoscopic and Subfrontal Craniotomy for Resection Tumors of Anterior Skull Base.

The Journal of craniofacial surgery
2022

Long-term oncologic outcomes in esthesioneuroblastoma: An institutional experience of 143 patients.

International forum of allergy &amp; rhinology
2022

Percutaneous Cryoablation for Recurrent Head and Neck Tumors.

Cardiovascular and interventional radiology
2022

Olfactory Neuroblastoma: Re-Evaluating the Paradigm of Intracranial Extension and Cyst Formation.

Diagnostics (Basel, Switzerland)
2022

How I do it: endonasal transcribriform approach for resection of esthesioneuroblastoma.

Acta neurochirurgica
2022

Esthesioneuroblastoma presenting with orbital signs and ectopic adrenocorticotropic hormone syndrome.

Proceedings (Baylor University. Medical Center)
2022

Survival impact of postoperative radiotherapy in patients with olfactory neuroblastoma: 513 cases from the SEER database.

Cancer radiotherapie : journal de la Societe francaise de radiotherapie oncologique
2022

Cystic Intracranial Recurrence of Olfactory Neuroblastoma without Accumulation on Fluorine-18-fluorodeoxyglucose Positron Emission Tomography.

Acta medica Okayama
2023

Olfactory neuroblastoma limited to sphenoid sinus.

British journal of neurosurgery
2022

Association of Race, Ethnicity, and Socioeconomic Status With Esthesioneuroblastoma Presentation, Treatment, and Survival.

OTO open
2022

Prognostic value of microvessel density and its correlation with clinicopathological features in human olfactory neuroblastoma.

Clinical neuropathology
2022

Less common extracerebral tumors.

Progress in brain research
2021

Olfactory Neuroblastoma Managed with Endoscopic Sinus Surgery.

Journal of pharmacy &amp; bioallied sciences
2021

Esthesioneuroblastoma with recurrent dural metastases: Long-term multimodality treatment and considerations.

Surgical neurology international
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Doenças relacionadas

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Referências e fontes

Bases de dados externas citadas neste artigo

Publicações científicas

Artigos indexados no PubMed ligados a esta doença no grafo RarasNet — título, periódico e PMID direto da fonte, sem intermediação de IA.

  1. Nationwide analysis of olfactory neuroblastoma in Japan: evolving treatment approaches and prognostic outcomes.
    Japanese journal of clinical oncology· 2026· PMID 41449573mais citado
  2. Proton Beam vs Intensity-Modulated Radiotherapy in Olfactory Neuroblastoma.
    JAMA otolaryngology-- head &amp; neck surgery· 2026· PMID 41165699mais citado
  3. DNA Virus Detection in Olfactory Neuroblastomas Using Targeted Enrichment NGS.
    Neuropathology : official journal of the Japanese Society of Neuropathology· 2026· PMID 41874350mais citado
  4. Surgical Management of Esthesioneuroblastoma at a Single Tertiary Care Center.
    Journal of neurological surgery. Part B, Skull base· 2026· PMID 41808975mais citado
  5. Orbital rhabdomyosarcoma in an adult: a misleading presentation.
    Radiology case reports· 2026· PMID 41798674mais citado
  6. Outcomes Following en Bloc Resection of Olfactory Neuroblastoma.
    Head Neck· 2026· PMID 41952527recente
  7. Diagnosis and management of esthesioneuroblastoma.
    Tunis Med· 2025· PMID 41879702recente

Bases de dados e fontes oficiais

Identificadores e referências canônicas usadas para montar este verbete.

  1. ORPHA:1957(Orphanet)
  2. MONDO:0016029(MONDO)
  3. GARD:2197(GARD (NIH))
  4. Busca completa no PubMed(PubMed)
  5. Artigo Wikipedia(Wikipedia)
  6. Q256806(Wikidata)

Dados compilados pelo RarasNet a partir de fontes abertas (Orphanet, OMIM, MONDO, PubMed/EuropePMC, ClinicalTrials.gov, DATASUS, PCDT/MS). Este conteúdo é informativo e não substitui avaliação médica.

Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar

Estesioneuroblastoma
Compêndio · Raras BR

Estesioneuroblastoma

ORPHA:1957 · MONDO:0016029
Prevalência
<1 / 1 000 000
Herança
Not applicable
CID-10
C30.0 · Neoplasia maligna da cavidade nasal
CID-11
Ensaios
6 ativos
Início
Adult, Elderly
Prevalência
0.0 (Europe)
MedGen
UMLS
C0206717
Repurposing
2 candidatos
cyclophosphamideDNA alkylating agent
iobenguaneantineoplastic agent
EuropePMC
Wikidata
Wikipedia
Papers 10a
DiscussaoAtiva

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