Olfactory neuroblastoma

Olfactory neuroblastoma 

Updated: 10/12/2020

© Jun Wang, MD, PhD

General features
  • AKA esthesioneuroblastoma
  • Rare, malignant neuroectodermal tumor
  • Probably arise from olfactory membrane or olfactory placode
  • NOT related to neuroblastomas elsewhere in body
  • Mean age 53 years, range 3-79 years
  • Slightly more common in male
  • Late recurrence common
  • Metastasis: Cervical lymph nodes, lungs
Clinical presentations
  • Usually upper nasal vault; rarely in nasopharynx, maxillary or ethmoid sinus
  • Nasal obstruction, epistaxis
  • Neurologic presentations: headache, nausea, diplopia, etc
  • Rarely paraneoplastic syndromes: Hypercalcemia, ectopic adrenocorticotropic hormone syndrome, etc
  • Presentations associated with local invasion
Radiologic findings
  • Homogeneous soft tissue mass with uniform and moderate contrast enhancement

Key morphological features
Markers
  • Positive for cytokeratin, neuron-specific enolase, synaptophysin, chromogranin, neurofilament, catecholamines, S100
Genetic abnormality
Treatment
  • Surgery with combined radiation therapy


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