Uma neoplasia maligna rara da cavidade sinonasal, com origem nas camadas basais das células neuroepiteliais olfatórias na abóbada nasal superior, que geralmente ocorre na 5ª à 6ª décadas de vida e é caracterizada clinicamente por sintomas inespecíficos, como bloqueio nasal ipsilateral progressivo, sinusite, dor facial, dores de cabeça intermitentes, hiposmia/disosmia, rinorreia e epistaxe, bem como proptose, diplopia e lacrimejamento excessivo devido à extensão orbital. Com o tratamento precoce e na ausência de metástases à distância, o estesioneuroblastoma parece apresentar um bom prognóstico (em comparação com outras neoplasias malignas nasais superiores), apesar de uma alta taxa de metástases cervicais.
Introdução
O que você precisa saber de cara
Visão geral
O estesioneuroblastoma é uma neoplasia maligna rara da cavidade sinonasal, com origem nas camadas basais das células neuroepiteliais olfatórias localizadas na abóbada nasal superior. Apresenta uma prevalência estimada em menos de 1 caso por 1.000.000 de pessoas (<1 / 1 000 000), afetando tipicamente indivíduos adultos e idosos, com ocorrência frequente entre a 5ª e a 6ª décadas de vida.[1]
Sinais e sintomas
O quadro clínico caracteriza-se inicialmente por sintomas inespecíficos, tais como bloqueio nasal ipsilateral progressivo, sinusite, dor facial, dores de cabeça intermitentes, alterações do olfato (hiposmia ou disosmia), rinorreia e epistaxe. Caso haja extensão para a órbita ocular, podem surgir manifestações adicionais como proptose, diplopia e lacrimejamento excessivo.[1]
Causas genéticas
Tratamentos citados na literatura
A literatura científica biomédica indexada no PubTator3 registra associações de termos terapêuticos e agentes químicos investigados no contexto do estesioneuroblastoma. Entre os compostos citados e suas respectivas contagens de publicações encontram-se: Cisplatin (22), Etoposide (16), Cyclophosphamide (8), Platinum (8), Vincristine (8), Fluorodeoxyglucose F18 (6), Carboplatin (5), Doxorubicin (4), Fluorouracil (4) e Catecholamines (3). Ressalta-se que esses dados refletem unicamente menções encontradas em publicações científicas e não constituem diretrizes, protocolos ou recomendações de prescrição médica.[3]
Prognóstico e qualidade de vida
Quando identificado e tratado precocemente, e na ausência de metástases à distância, o estesioneuroblastoma parece apresentar um bom prognóstico em comparação com outras neoplasias malignas nasais superiores, a despeito de apresentar uma taxa considerável de metástases cervicais.[1]
Conteúdo informativo gerado e mantido automaticamente a partir de fontes oficiais (Orphanet, HPO, OMIM, SUS). Não substitui avaliação médica.
O estesioneuroblastoma é um tipo de tumor maligno muito raro que começa no alto do nariz, na área das células responsáveis por identificar os cheiros. Ele costuma surgir em pessoas adultas ou mais velhas e causa sinais como nariz entupido de um lado só, sangramentos pelo nariz, sinusite, dor facial e redução do olfato. Caso o tumor cresça em direção aos olhos, podem surgir alterações visuais, olhos saltados e lacrimejamento contínuo. No Brasil, a doença não conta com um protocolo de tratamento (PCDT) próprio no Ministério da Saúde, devendo a pessoa ser acompanhada em hospitais especializados de referência.
Uma neoplasia maligna rara da cavidade sinonasal, com origem nas camadas basais das células neuroepiteliais olfatórias na abóbada nasal superior, que geralmente ocorre na 5ª à 6ª décadas de vida e é caracterizada clinicamente por sintomas inespecíficos, como bloqueio nasal ipsilateral progressivo, sinusite, dor facial, dores de cabeça intermitentes, hiposmia/disosmia, rinorreia e epistaxe, bem como proptose, diplopia e lacrimejamento excessivo devido à extensão orbital. Com o tratamento precoce e na ausência de metástases à distância, o estesioneuroblastoma parece apresentar um bom prognóstico (em comparação com outras neoplasias malignas nasais superiores), apesar de uma alta taxa de metástases cervicais.
Escala de raridade
<1/50kMuito rara
1/20kRara
1/10kPouco freq.
1/5kIncomum
1/2k
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Sinais e sintomas
O que aparece no corpo e com que frequência cada sintoma acontece
Linha do tempo da pesquisa
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Genética e causas
O que está alterado no DNA e como passa nas famílias
Nenhum gene associado encontrado
Os dados genéticos desta condição ainda estão sendo catalogados.
Diagnóstico
Os sinais que médicos procuram e os exames que confirmam
Tratamento e manejo
Remédios, cuidados de apoio e o que precisa acompanhar
Onde tratar no SUS
Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)
🇧🇷 Atendimento SUS — Estesioneuroblastoma
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Publicações mais relevantes
Nationwide analysis of olfactory neuroblastoma in Japan: evolving treatment approaches and prognostic outcomes.
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.
Proton Beam vs Intensity-Modulated Radiotherapy in Olfactory Neuroblastoma.
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.
DNA Virus Detection in Olfactory Neuroblastomas Using Targeted Enrichment NGS.
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.
Surgical Management of Esthesioneuroblastoma at a Single Tertiary Care Center.
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.
Orbital rhabdomyosarcoma in an adult: a misleading presentation.
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
Outcomes Following en Bloc Resection of Olfactory Neuroblastoma.
🥉 Relato de casoDiagnosis and management of esthesioneuroblastoma.
🥉 Relato de casoDNA Virus Detection in Olfactory Neuroblastomas Using Targeted Enrichment NGS.
📖 RevisãoSurgical Management of Esthesioneuroblastoma at a Single Tertiary Care Center.
Orbital rhabdomyosarcoma in an adult: a misleading presentation.
🥉 Relato de caso📚 EuropePMC602 artigos no totalmostrando 200
Diagnosis and management of esthesioneuroblastoma.
La Tunisie medicaleDNA Virus Detection in Olfactory Neuroblastomas Using Targeted Enrichment NGS.
Neuropathology : official journal of the Japanese Society of NeuropathologySurgical Management of Esthesioneuroblastoma at a Single Tertiary Care Center.
Journal of neurological surgery. Part B, Skull baseOrbital rhabdomyosarcoma in an adult: a misleading presentation.
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Journal of neurological surgery. Part B, Skull baseNeoadjuvant 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.
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CureusRefinement of Hyams Grading Criteria for Olfactory Neuroblastoma in an Endoscopic Sinus Surgery Predominant Cohort with Extended Follow-up: Worth the Effort?
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Journal of personalized medicineRevisiting 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[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 surgeryEnhancing Diagnostic Accuracy in Sinonasal Neuroendocrine Tumors: The Case for DOTATATE PET Over FDG PET.
International forum of allergy & rhinologySIADH as an uncommon presentation of olfactory neuroblastoma: a case-based overview.
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International forum of allergy & rhinologyHypoosmolar Hyponatremia as Presenting Symptom of a Rare Case of Olfactory Neuroblastoma.
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Frontiers in oncologyHyams Grade and Ki-67 as Predictive Factors for Primary Treatment Failure in Olfactory Neuroblastoma.
The LaryngoscopeFrontal Lobe Herniation After Endoscopic Anterior Skull Base Resection: A Rare Complication.
Ear, nose, & throat journalThe 100 most-cited papers on esthesioneuroblastoma: a bibliometric analysis.
Annals of medicine and surgery (2012)Metastatic olfactory neuroblastoma with unusual solitary metastasis to femur: a case report and literature review.
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Nature cancerOlfactory Neuroblastoma With Divergent Differentiation: Contemporary Management of Unusual Pathology and Literature Review.
Ear, nose, & throat journalPathologic dural invasion is associated with regional recurrence in olfactory neuroblastoma: A multi-institutional study.
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Surgical pathology clinicsAn adolescent girl with syndrome of inappropriate antidiuretic hormone secretion preceding the diagnosis of olfactory neuroblastoma - a case report.
Frontiers in endocrinologyThe value of elective neck irradiation in management of esthesioneuroblastoma: a retrospective study based on propensity score matching.
Radiation oncology (London, England)Olfactory Carcinoma, Similar yet Distinct to Olfactory Neuroblastoma: A Case Report and Literature Review.
ORL; journal for oto-rhino-laryngology and its related specialtiesRadiologic overview of sinonasal lesions.
Frontiers in radiology[Genomic profiles and immune microenvironment of olfactory neuroblastoma].
Zhonghua bing li xue za zhi = Chinese journal of pathology[Olfactory neuroblastoma in children: clinical analysis of five cases].
Zhonghua yi xue za zhiEctopic Olfactory Neuroblastoma Arising in the Nasopharynx.
The Journal of craniofacial surgeryOlfactory neuroblastoma with orbital protrusion arising in the background of maxillary carcinoma.
Journal of surgical case reportsSurvival Analysis and Prognostic Factors After Endonasal Resection of Advanced Olfactory Neuroblastomas: A Single Institution Experience.
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Reports of practical oncology and radiotherapy : journal of Greatpoland Cancer Center in Poznan and Polish Society of Radiation OncologyOlfaction Preservation and Long-Term Outcomes in Patients with Unilateral Endoscopic Resection of Olfactory Neuroblastoma: A Systematic Review and Institutional Experience.
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CureusOlfactory neuroblastoma: A rare sinonasal malignancy.
JPMA. The Journal of the Pakistan Medical AssociationOlfactory dysfunction management following unilateral cranial resection for olfactory neuroblastoma.
RhinologyEctopic adrenocorticotropic hormone syndrome in patients with olfactory neuroblastoma.
Endocrine-related cancerA case of olfactory ganglioneuroblastoma in a dog: immunohistochemical comparison with olfactory neuroepithelia and olfactory neuroblastomas.
The Journal of veterinary medical scienceA Case of Olfactory Neuroblastoma Developing Bilateral Retropharyngeal Lymph Node Metastasis 14-years After Skull Base Surgery.
The Kobe journal of medical sciencesNasal Cavity and Paranasal Sinus Cancer: Diagnosis and Treatment.
Current oncology reportsEndoscopy-assisted medial canthus incision for olfactory neuroblastoma: a case report.
World journal of surgical oncologyThe 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 SurgeryLong non-coding RNA MALAT 1 and PHOX2B expression in olfactory neuroblastomas and sympathetic neuroblastomas.
Annals of diagnostic pathology[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 surgeryOlfactory Neuroblastoma Is Not Always Located at the Roof of the Nasal Cavity.
Journal of the Belgian Society of RadiologyAugmentation of tumor expression of HLA-DR, CXCL9, and CXCL10 may improve olfactory neuroblastoma immunotherapeutic responses.
Journal of translational medicineOlfactory neuroblastoma mimics molecular heterogeneity and lineage trajectories of small-cell lung cancer.
Cancer cellDevelopment 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 oncologyFeasibility of Optical Biopsy During Endoscopic Sinus Surgery With Confocal Laser Endomicroscopy: A Pilot Study.
The LaryngoscopeClinical outcomes for olfactory neuroblastoma.
Frontiers in oncologyExophthalmos revealing an olfactory esthesioneuroblastoma: A case report.
International journal of surgery case reportsRecurrence morbidity of olfactory neuroblastoma.
International forum of allergy & rhinologyTreatment of unilateral olfactory neuroblastoma: Appropriate extent of surgical resection and potential for olfactory preservation.
International forum of allergy & rhinologyReview 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, IncPatterns of recurrence and disease progression in patients with positive-margin olfactory neuroblastoma following primary resection.
Journal of neurosurgeryNeuroendocrine and undifferentiated sinonasal and skull base tumors: An up-to-date narrative review.
Oral and maxillofacial surgeryThe impact of multidisciplinary approaches on the outcomes of olfactory neuroblastoma treated with postoperative radiotherapy.
Cancer medicineA rare presentation of esthesioneuroblastoma: a case report and review of the literature.
The Pan African medical journalEsthesioneuroblastoma presenting as syndrome of inappropriate antidiuretic hormone secretion.
La Tunisie medicaleTo 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 IndiaTreatment Outcomes of Olfactory Neuroblastoma: A Multicenter Study by the Korean Sinonasal Tumor and Skull Base Surgery Study Group.
Clinical and experimental otorhinolaryngologyUpdate on olfactory neuroblastoma.
Virchows Archiv : an international journal of pathologySNOT-22 subdomain outcomes following treatment for sinonasal malignancy: A prospective, multicenter study.
International forum of allergy & rhinologyRecurrent Wnt Pathway and ARID1A Alterations in Sinonasal Olfactory Carcinoma.
Modern pathology : an official journal of the United States and Canadian Academy of Pathology, IncUpright proton therapy for esthesioneuroblastoma: a single-institution experience.
Frontiers in oncologyCharacterizing Immune Infiltration in Esthesioneuroblastoma Subtypes Through Gene Expression Deconvolution.
World neurosurgeryThe role of insulinoma-associated protein 1 in predicting the progression and prognosis of human olfactory neuroblastoma in China.
Pathology, research and practiceThe 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 SurgeryThe medial transorbital approach in cranioendoscopic skull base tumor resections for locally advanced tumors.
Journal of clinical neuroscience : official journal of the Neurosurgical Society of AustralasiaOncology for the rhinologist.
Current opinion in otolaryngology & head and neck surgeryRare case of mortality after hemorrhagic property of esthesioneuroblastoma.
Archive of clinical casesGenomic characterization of an esthesioneuroblastoma with spinal metastases: illustrative case.
Journal of neurosurgery. Case lessonsSimple and safe resection of the crista galli.
Head & neckPeptide Receptor Radionuclide Therapy for Recurrent Olfactory Neuroblastoma After Cranioplasty for Surgical Infection: A Case Report.
Anticancer researchOutcomes following proton therapy for pediatric esthesioneuroblastoma.
Pediatric blood & cancerPredictability of Olfactory Neuroblastoma Staging Systems-Reply.
JAMA otolaryngology-- head & neck surgeryPredictability of Olfactory Neuroblastoma Staging Systems.
JAMA otolaryngology-- head & neck surgeryFailure Patterns of Recurrence in Patients With Localized Esthesioneuroblastoma Following Surgery and Adjuvant Radiotherapy Without Elective Nodal Irradiation.
CureusOlfactory neuroblastoma: diagnosis, management, and current treatment options.
Frontiers in oncologyPrimary Sellar Neuroblastoma Masquerading as a Pituitary Macroadenoma.
JCEM case reports[Chinese expert consensus on pathological diagnosis of olfactory neuroblastoma(2023 version)].
Zhonghua bing li xue za zhi = Chinese journal of pathologyOlfactory schwannoma masquerading as esthesioneuroblastoma.
BMJ case reportsSyndrome of Inappropriate Antidiuretic Hormone Release Secondary to Ectopic Olfactory Neuroblastoma: Case Report and Review of Literature.
Ear, nose, & throat journalAnterior Skull Base Osteoradionecrosis in the Age of Intensity-Modulated Radiation Therapy: A Case Series.
Journal of neurological surgery reportsInduction Chemotherapy for Sinonasal Tumors in Patients Presenting with Brain Invasion and/or Neurological Deficits.
Journal of neurological surgery. Part B, Skull baseSolid and Vascular Neck Masses in Children.
Neuroimaging clinics of North AmericaEndoscopic 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 SurgeryA Rare Case of Sinonasal Ewing Sarcoma With Radiologic-Pathologic Correlation.
CureusInternational Consensus Statement on Allergy and Rhinology: Sinonasal Tumors.
International forum of allergy & rhinologyUnderstanding frozen section histopathology in Sinonasal and anterior skull base malignancy and proposed reporting guidelines.
Journal of surgical oncologySmall 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 IndiaClinical relevance of hyponatraemia in olfactory neuroblastoma.
The Indian journal of medical researchEsthesioneuroblastoma: Management of the N0 neck.
Journal of neurological surgery reportsSetting the Stage for Olfactory Neuroblastoma.
JAMA otolaryngology-- head & neck surgeryMulticenter Survival Analysis and Application of an Olfactory Neuroblastoma Staging Modification Incorporating Hyams Grade.
JAMA otolaryngology-- head & neck surgeryTreatment outcomes and prognostic factors of esthesioneuroblastoma-a retrospective study from South India.
EcancermedicalscienceStereotactic radiosurgery for distant brain metastases secondary to esthesioneuroblastoma: a single-institution series.
Neurosurgical focusOlfactory neuroblastoma in a domestic cat and review of the literature.
Veterinary clinical pathologyReal 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 oncologyCyberKnife Radiosurgery for Spinal Leptomeningeal Metastases Secondary to Esthesioneuroblastoma: A Clinical Case Report.
CureusDeconstructing Olfactory Epithelium Developmental Pathways in Olfactory Neuroblastoma.
Cancer research communicationsDOTATATE PET Imaging in Olfactory Neuroblastoma and Association with SSTR Expression.
Journal of neurological surgery. Part B, Skull baseComparing the role of different treatment modalities in locoregionally advanced olfactory neuroblastoma: The 20-year clinical experience of a single institution.
Head & neckA multi-institutional retrospective study of 340 cases of sinonasal malignant tumor.
Auris, nasus, larynxAn atypical Esthesioneuroblastoma of the sphenoid sinus: a case report.
Annals of medicine and surgery (2012)Clinical Outcome in Patients with Large Sinonasal Tumors with Intracranial Extension.
Journal of neurological surgery. Part B, Skull baseProceedings 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 pathologyDetermining the association of insurance coverage and survival outcomes in patients with olfactory neuroblastoma utilizing machine learning.
International forum of allergy & rhinologyOlfactory Neuroblastoma-A Challenging Fine Line between Metastasis and Hematology.
Medicina (Kaunas, Lithuania)[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 surgeryOlfactory neuroblastoma with rhabdomyoblasts: A rare case report and review of the literature.
Indian journal of pathology & microbiologyMetastatic esthesioneuroblastoma with widespread recurrence to the central nervous system.
Surgical neurology internationalClinical 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 IndiaLymph 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 & neckRecurrent Esthesioneuroblastoma: Long-Term Outcomes of Salvage Therapy.
CancersRadiographic predictors of occult intracranial involvement in olfactory neuroblastoma patients.
International forum of allergy & rhinologyMolecular 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, IncGa-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, IndiaOperative 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.)Sinonasal Schwannomas: Imaging Findings and Review of Literature.
Ear, nose, & throat journalPrognostic significance of PD-1, CTLA-4, CD4, and CD8 expression in olfactory neuroblastoma.
Clinical neuropathologyThe Role of Induction Therapy for Sinonasal Cancers.
Current treatment options in oncologyAn exceptionally rare case of Cushing's syndrome caused by ectopic ACTH syndrome due to olfactory neuroblastoma in childhood.
Journal of pediatric endocrinology & metabolism : JPEMEctopic Olfactory Neuroblastoma: Systematic Review of a Rare Clinical Entity among Sinonasal Tumors.
Journal of neurological surgery. Part B, Skull baseLong-term survival outcomes and recurrence patterns of olfactory neuroblastoma: A 13-year experience at a single institution.
Auris, nasus, larynxCarbon-ion radiotherapy in the treatment of radiation-induced second primary malignancies.
Annals of translational medicineOlfactory Neuroblastoma: A Novel Site of Presentation.
Indian journal of otolaryngology and head and neck surgery : official publication of the Association of Otolaryngologists of IndiaA Review on Esthesioneuroblastoma.
Indian journal of otolaryngology and head and neck surgery : official publication of the Association of Otolaryngologists of IndiaOlfactory Neuroblastoma: Morphological Reappraisal and Molecular Insights with Quantum Leap in Clinical Perspectives.
Current oncology reportsImmunohistochemical Profiling and Staging in Esthesioneuroblastoma: A Single-Center Cohort Study and Systematic Review.
World neurosurgeryCytological diagnosis of olfactory neuroblastoma at metastatic sites, with emphasis on role of insulinoma-associated protein 1 immunocytochemistry.
Diagnostic cytopathologyThe 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 radiologyDNA Methylation Profiling in Rare Sellar Tumors.
BiomedicinesIdiopathic SIADH: 4 years of diagnostic wandering.
European journal of nuclear medicine and molecular imagingMetastatic esthesioneuroblastoma recurrence after 19 years of remission: A systematic review with case illustration.
Journal of the neurological sciencesExpressions of NeuroD and GAP43 as diagnostic markers for olfactory neuroblastoma.
Auris, nasus, larynxThe contemporary management of cancers of the sinonasal tract in adults.
CA: a cancer journal for cliniciansEsthesioneuroblastoma (Olfactory Neuroblastoma): Overview and Extent of Surgical Approach and Skull Base Resection.
Journal of neurological surgery reportsThe prognostic value of S-100 protein and Ki-67 index in olfactory neuroblastoma.
Japanese journal of clinical oncologyApplication of the subdural evacuating port system for the drainage of postoperative tension pneumocephalus: A technical note.
Surgical neurology internationalFrontal lobe position after single-layer cadaveric dermal matrix repair of large anterior skull base defects.
World journal of otorhinolaryngology - head and neck surgeryExclusively endoscopic surgical resection of esthesioneuroblastoma: A systematic review.
World journal of otorhinolaryngology - head and neck surgeryOlfactory Neuroblastomas: What Actually Happens in the Long-Term?
Journal of clinical medicineMinimally invasive pericranial flap harvest through a supraorbital eyebrow craniotomy: Technical note in salvage skull base reconstruction.
Clinical neurology and neurosurgeryNasoethmoidal Schwannoma as a Mimicar of Esthesioneuroblastoma: A Case Report and Literature Review.
Neurology IndiaTranscription 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 pathologyCase Report: Esthesioneuroblastoma Involving the Optic Pathways.
Frontiers in surgeryImpact of esthesioneuroblastoma treatment delays on overall patient survival.
The LaryngoscopeMagnetic resonance imaging findings in patients with idiopathic olfactory dysfunction and normal findings on nasoendoscopy.
The Journal of laryngology and otologyManagement of Esthesioneuroblastoma: A Retrospective Study of 6 Cases and Literature Review.
Case reports in oncologySinonasal 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 pathologyCombined Nasal Endoscopic and Subfrontal Craniotomy for Resection Tumors of Anterior Skull Base.
The Journal of craniofacial surgeryLong-term oncologic outcomes in esthesioneuroblastoma: An institutional experience of 143 patients.
International forum of allergy & rhinologyPercutaneous Cryoablation for Recurrent Head and Neck Tumors.
Cardiovascular and interventional radiologyOlfactory Neuroblastoma: Re-Evaluating the Paradigm of Intracranial Extension and Cyst Formation.
Diagnostics (Basel, Switzerland)How I do it: endonasal transcribriform approach for resection of esthesioneuroblastoma.
Acta neurochirurgicaEsthesioneuroblastoma presenting with orbital signs and ectopic adrenocorticotropic hormone syndrome.
Proceedings (Baylor University. Medical Center)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 oncologiqueCystic Intracranial Recurrence of Olfactory Neuroblastoma without Accumulation on Fluorine-18-fluorodeoxyglucose Positron Emission Tomography.
Acta medica OkayamaOlfactory neuroblastoma limited to sphenoid sinus.
British journal of neurosurgeryAssociation of Race, Ethnicity, and Socioeconomic Status With Esthesioneuroblastoma Presentation, Treatment, and Survival.
OTO openPrognostic value of microvessel density and its correlation with clinicopathological features in human olfactory neuroblastoma.
Clinical neuropathologyLess common extracerebral tumors.
Progress in brain researchOlfactory Neuroblastoma Managed with Endoscopic Sinus Surgery.
Journal of pharmacy & bioallied sciencesEsthesioneuroblastoma with recurrent dural metastases: Long-term multimodality treatment and considerations.
Surgical neurology internationalAssociações
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Doença com base genética
Um médico geneticista pode ajudar no diagnóstico de Estesioneuroblastoma e no aconselhamento genético da família.
Doenças relacionadas
Doenças com sintomas parecidos — ajudam quem ainda está buscando diagnóstico
Ainda não achamos doenças com sintomas parecidos o suficiente.
Perguntas frequentes
O que as famílias mais perguntam sobre esta doença — cada resposta com a fonte de onde saiu
Não, o modelo de herança genética é catalogado como não aplicável para esta condição. Não existem genes de transmissão hereditária estabelecidos nos bancos de dados oficiais.
Referências
Fontes citadas no texto, publicações do grafo e bases de dados usadas neste verbete
4 fontes citadas no texto · 7 publicações do grafo RarasNet (PubMed) · 5 bases de dados. Títulos, periódicos e PMIDs vêm direto da fonte, sem intermediação de IA.
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.
Citar este verbete
Raras. (2026). Estesioneuroblastoma. Em Raras — Enciclopédia de Doenças Raras do Brasil. https://raras.org/doenca/estesioneuroblastoma
Formato APA. Conteúdo sob CC BY 4.0 — reuso livre com atribuição.
Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar
Estesioneuroblastoma
📋 Origem dos dados
Esta página agrega dados de fontes públicas e oficiais. Dados sobre cobertura no SUS (PCDT, CEAF) são verificados ativamente por agente proativo (ver badge no infobox). Demais dados têm atribuição de fonte + data da última sincronização — clique para abrir o original.
- Doença rara (ontologia)
- fonte: Orphanet
- Identificador unificado
- fonte: MONDO
- Codificação WHO/SUS
- fonte: WHO ICD-10 / DATASUS
- CID-11 (futuro)
- fonte: WHO ICD-11
- NIH/GARD
- fonte: GARD (NIH)
- Indexação biomédica
- fonte: MeSH (NLM)
- Dado público estruturado
- fonte: Wikidata
- Reposicionamento
- fonte: Drug Repurposing Hub
- Ensaios clínicos
- fonte: ClinicalTrials.gov
