Esthesioneuroblastoma

HISTOLOGY

  • Esthesioneuroblastoma is a tumor of neural crest origin.
  • other names include olfactory neuroblastoma and esthesioneurocytoma
  • may be mistaken for "small round-cell tumor"
  • route of spread is similar to ethmoid carcinomas with distant metastasis at diagnosis 11% and 1% respectively.

STAGE

Kadish stage
Stage A:

  • confined to nasal cavity
  • 4% died of disease (Elkon et al. Esthesionneuroblastoma. Cancer 44: 1087-1094, 1979).

Stage B:

  • involves the nasal cavity and one or more paranasal sinuses
  • 30% died of disease

Stage C:

  • beyond the nasal cavity and paranasal sinuses including
    • orbit
    • base of skull
    • intracranial
    • cervical LN
    • distant metastasis
  • 60% died of disease

PRIMARY THERAPY

Stage A:

  • Single modality (surgery or radiation) yield > 90% loco-regional control

Stage B:

  • controversial, combined surgery and radiotherapy may have slight advantage

Stage C:

  • combination surgery with post-operative radiation
  • no routine elective nodal treatment since the incidence of isolated nodal relapse is < 15%.
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