# Estesioneuroblastoma

> Página oficial: https://raras.org/doenca/estesioneuroblastoma
> Fonte: Raras.org — Banco de Dados de Doenças Raras em Português (CC BY-NC-SA 4.0)
> Última atualização: 2026-05-08

## Identificadores

- **ORPHA**: 1957 — https://www.orpha.net/en/disease/detail/1957
- **CID-10**: C30.0

## Descrição clínica

A rare malignant neoplasm of the sinonasal cavity, arising from the basal layers of olfactory neuroepithelial cells in the superior nasal vault, which usually occurs in the 5th to 6th decades of life and is characterized clinically by non-specific symptoms such as progressive ipsilateral nasal block, sinusitis, facial pain, intermittent headaches, hyposmia/dysosmia, rhinorrhea and epistaxis as well as proptosis, diplopia and excessive lacrimation due to orbital extension. With early treatment and in the absence of distant metastases, ENB appears to have a good prognosis (compared to other superior nasal malignancies), despite a high rate of cervical metastases.

## Epidemiologia e herança

- **Prevalência**: <1 / 1 000 000
- **Padrão de herança**: Not applicable

## Ensaios clínicos ativos (6)

- **NCT06607692** [RECRUITING]: Study in Children and Adolescents of 177Lu-DOTATATE (Lutathera®) Combined With the PARP Inhibitor Olaparib for the Treatment of Recurrent or Relapsed Solid Tumours Expressing Somatostatin Receptor (SSTR) (LuPARPed). — https://clinicaltrials.gov/study/NCT06607692
- **NCT04755205** [RECRUITING]: A Natural History Study of Children and Adults With Olfactory Neuroblastoma — https://clinicaltrials.gov/study/NCT04755205
- **NCT04081701** [RECRUITING]: 68-Ga DOTATATE PET/MRI in the Diagnosis and Management of Somatostatin Receptor Positive CNS Tumors. — https://clinicaltrials.gov/study/NCT04081701
- **NCT06814496** [RECRUITING]: Radiation Combined With BIspecific T-Cell Engager in DLL3 Expressing Tumors — https://clinicaltrials.gov/study/NCT06814496
- **NCT07072143** [RECRUITING]: An International Study on Pediatric Patients With Rare Tumors. — https://clinicaltrials.gov/study/NCT07072143
- **NCT04087902** [RECRUITING]: Long-Term Longitudinal QoL in Patients Undergoing EEA — https://clinicaltrials.gov/study/NCT04087902
- **NCT05012098** [COMPLETED]: Phase 2 Study of Bintrafusp Alfa in Recurrent/Metastatic Olfactory Neuroblastoma (BARON). — https://clinicaltrials.gov/study/NCT05012098
- **NCT01586767** [UNKNOWN]: Intensity-Modulated or Proton Radiation Therapy for Sinonasal Malignancy — https://clinicaltrials.gov/study/NCT01586767
- **NCT00470496** [COMPLETED]: Photodynamic Therapy Using HPPH in Treating Patients Undergoing Surgery for Primary or Recurrent Head and Neck Cancer — https://clinicaltrials.gov/study/NCT00470496
- **NCT02580136** [WITHDRAWN]: Esthesioneuroblastoma: Outcomes After Treatment — https://clinicaltrials.gov/study/NCT02580136

## Importante

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Pacientes devem consultar profissionais de saúde qualificados para decisões clínicas.

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**Citação sugerida**: Raras.org — Estesioneuroblastoma. Disponível em: https://raras.org/doenca/estesioneuroblastoma
**Formato HTML**: https://raras.org/doenca/estesioneuroblastoma
**Formato RDF/Turtle**: https://raras.org/api/rdf?orpha=1957