Interesting case

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Interesting case

• 40 yo female
• Cc r-sided facial edma
• HPI started in April 2003, increasing since
  frequent epistaxis complete nasal obstruction
  anosmic
• PMH none
• PE telecanthus, slight R proptosis, no diplopia
  flattend nasal bridge, mass completely filling
  nasal cavities

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Biopsy

• Malignant tumor of neuroectodermal origin
• olfactory neuroblastoma?

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Olfactory neuroblastoma

• Olfactory neuroblastoma (ONB) is a rare malignant
  tumor of neuroectodermal origin. This lesion has
  been referred to by a variety of terms
  esthesioneuroblastoma, esthesioneuroepithelioma,
  and esthesioneurocytoma. It is believed to arise
  from a mitotically active basal or reserve cell
  in the olfactory epithelium that gives rise to
  neuronal and sustentacular cells.

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Olfactory neuroblastoma

• Olfactory neuroblastomas occur in men and women,
  with a slight female dominance. Some studies find
  a bimodal peak of occurrence between 15 and 50
  years of age however, the majority of cases are
  seen in the third and fourth decades of life. The
  most common symptoms are nasal obstruction,
  epistaxis, and, less frequently, anosmia,
  headache, pain, excessive lacrimation, and ocular
  disturbances.

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Histology

• The microscopic features of ONBs include highly
  vascularized stroma that is infiltrated by nests
  and lobular growths of cells. These cells have
  indistinct cytoplasmic borders, round, mildly
  vesicular nuclei, infrequent mitoses, and rare
  nucleoli. A fibrillary background and glandular
  structure called a pseudorosettes is present.

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Kadish staging of olfactory neuroblastomas

• Stage Description 5-Year Survival Rate
• A confined to NC 57-88
• B confined to NC and one or more paranasal
  sinuses 58-60
• C extending beyond the NC or paranasal
  sinuses 0-50

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Treatment

• A treatment for olfactory neuroblastoma includes
  complete surgical resection, if possible, with
  adjunctive radiotherapy. Preliminary data have
  been accumulated using chemotherapy with
  accompanying irradiation in advanced or recurrent
  disease. Prognosis for olfactory neuroblastoma
  depends to a certain extent on clinical stage
  however, clinical behavior is often unpredictable.