Aggressive primary olfactory neuroblastoma of the sphenoclival region: a case report and literature review

Laryngoscope. 2015 Apr;125(4):822-5. doi: 10.1002/lary.24925. Epub 2014 Sep 12.

Abstract

Olfactory neuroblastoma (ONB) is an uncommon malignant tumor of neural crest origin, which commonly arises in the superior nasal cavity. Ectopic origin of an ONB is exceedingly rare. We describe a rare case of an ectopic sphenoclival ONB with extensive involvement of the central skull base and with development of systemic metastases. To our knowledge, ours is the first case that describes the imaging features of this rare entity on computed tomography (CT), magnetic resonance imaging, 18 F-fluorodeoxyglucose-positron-emission tomography/CT, and digital subtraction angiography. We also describe the histological features, imaging differentials, and treatment options for this tumor along with a brief literature review.

Keywords: Ectopic; olfactory neuroblastoma; sphenoclival.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Antineoplastic Combined Chemotherapy Protocols / administration & dosage
  • Combined Modality Therapy
  • Disease Progression
  • Esthesioneuroblastoma, Olfactory / diagnosis*
  • Esthesioneuroblastoma, Olfactory / therapy*
  • Fatal Outcome
  • Female
  • Fluorodeoxyglucose F18
  • Humans
  • Magnetic Resonance Imaging / methods
  • Middle Aged
  • Multimodal Imaging / methods*
  • Neoplasm Invasiveness / pathology
  • Neoplasm Recurrence, Local / pathology*
  • Neoplasm Recurrence, Local / therapy
  • Neoplasm Staging / methods
  • Positron-Emission Tomography / methods
  • Radiotherapy, Adjuvant
  • Rare Diseases
  • Skull Base / pathology*
  • Sphenoid Sinus / pathology*
  • Tomography, X-Ray Computed / methods

Substances

  • Fluorodeoxyglucose F18