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Updated: Jul 15, 2026

Identification of OTX1 and OTX2 As Two Possible Molecular Markers for Sinonasal Carcinomas and Olfactory Neuroblastomas
07:00

Identification of OTX1 and OTX2 As Two Possible Molecular Markers for Sinonasal Carcinomas and Olfactory Neuroblastomas

Published on: February 28, 2019

REFCOR guidelines on the management of olfactory neuroblastoma: A formalized expert consensus

V Gorzkowski1, C Monnot2, C Castain3

  • 1Service d'ORL-Chirurgie Cervico-Faciale, Université de Lorraine, CHRU Nancy, Service ORL, 54000 Nancy, France.

European Annals of Otorhinolaryngology, Head and Neck Diseases
|July 13, 2026
PubMed
Abstract

Insights

Olfactory neuroblastoma (ONB) is a rare sinonasal cancer. New French guidelines standardize diagnosis and treatment, emphasizing surgery, radiotherapy, and long-term follow-up for this rare tumor.

Area of Science:

  • Oncology
  • Otorhinolaryngology (ENT)
  • Rare Cancers

Background:

  • Olfactory neuroblastoma (ONB) is a rare malignant tumor originating in the sinonasal tract.
  • ONB represents 6% of all sinonasal cancers, with no established risk factors.
  • Diagnosis involves CT and MRI for staging, with histopathology crucial for prognosis.

Purpose of the Study:

  • To synthesize evidence-based recommendations from the French Expert Network of Rare ENT Cancers (REFCOR) for ONB management.
  • To provide updated diagnostic and therapeutic guidelines for olfactory neuroblastoma.
  • To establish a standardized approach for a rare sinonasal malignancy.

Main Methods:

  • Multidisciplinary consensus development.
  • Critical analysis of existing literature.
  • Feedback from expert centers within a structured process.

Main Results:

  • Histopathological grading (Hyams grade) is the primary prognostic indicator.
  • Standard treatment involves surgical resection followed by adjuvant radiotherapy.
  • Chemotherapy is an option for high-grade or unresectable ONB.
  • Long-term follow-up (≥20 years) is recommended due to late recurrence risk.

Conclusions:

  • REFCOR guidelines aim to standardize ONB diagnosis and treatment.
  • Discussion in a specialized multidisciplinary tumor board is essential for optimal patient management.
  • These recommendations support improved outcomes for patients with olfactory neuroblastoma.
Keywords:
GuidelinesOlfactory neuroblastomaREFCORSinonasal cancer

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