Radiation Oncology/Esthesioneuroblastoma

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Esthesioneuroblastoma


Overview[edit]

  • Also known as olofactory neuroblastoma
  • Originating from the olofactory epithelium in the roof of the nasal cavity
    • Exact cell of origin controversial
    • Data suggest ENB is derived from immature olofactory neurons of neuroectodermal origin
  • Lines superior one third of nasal septum, cribriform plate, and superior turbinate
    • Tumor typically occurs in the vault of nasal cavity or lateral wall adjacent to ethymoid sinuses
  • Estimated to be 3-5% of nasal malignant tumors
  • Bimodal age distribution
    • 10-20 years
    • 40-60 years
  • Clinical presentation
    • Unilateral symptoms: nasal obstruction, epistaxis
  • Nodal spread
    • Rare in early stage (<10%)
    • More common in advanced stage (30-50%)

Staging[edit]

Kadish System

  • Kadish A: confined to nasal cavity (18%)
  • Kadish B: extends to paranasal sinuses (32%)
  • Kadish C: extends beyond nasal cavity and paranasal sinuses (49%)
  • Kadish D: lymph node or distant metastases


TNM Staging


Outcome[edit]

  • Survival
    • Kadish A: 83%
    • Kadish B: 49%
    • Kadish C: 39%
    • Kadish D: 13%
  • Predictors of survival
    • Kadish stage
    • Treatment modality
    • Age at diagnosis


Treatment[edit]

  • Rare disease, so most studies are small, single institution, retrospective
  • Surgery frequently primary modality
  • Some evidence that postop RT improves local control, particularly for more advanced disease
  • Some evidence that concurrent chemo-RT may be beneficial
  • Elective nodal irradiation controversial, particularly if chemo-RT used for primary disease
  • Treatment recommendations (Hansen, 2nd ed)
    • Small, low grade tumors: surgery or RT alone (65-70 Gy)
    • Large tumors: consider preop RT (50 Gy), surgery, or postop chemo-RT (60 Gy)


  • Ulsan University, Korea; 2011 (1996-2007) PMID 20421144 -- "Radiotherapy for esthesioneuroblastoma: is elective nodal irradiation warranted in the multimodality treatment approach?" (Noh OK, Int J Radiat Oncol Biol Phys. 2011 Feb 1;79(2):443-9. Epub 2010 Apr 24.)
    • Retrospective. 14 patients, various combinations of modalities, elective nodal irradiation 4 patients. Median F/U 2.2 years
    • Outcome: 3-year OS 73%. LR 21%, regional recurrence 21%, distant failure 14%. No nodal failure in patients treated with chemo-RT, regardless of ENI
    • Conclusion: Elective nodal irradiation may play a limited role, if patients treated with chemo-RT
  • Chiba; 2007 (1999-2005) PMID 17398027 -- "Proton-beam therapy for olfactory neuroblastoma." (Nishimura H, Int J Radiat Oncol Biol Phys. 2007 Jul 1;68(3):758-62. Epub 2007 Mar 29.)
    • Retrospective. 14 patients (Kadish A 2/14, Kadish B 5/14, Kadish C 7/14). Dose 65 GyE in 2.5 Gy/fx. Median F/U 3.3 years
    • Outcome: 5-year LC 84%, 5-year RFS 71%, 5-year OS 93%
    • Toxicity: Liquorrhea 1 patient, no other Grade 3+
    • Conclusion: Excellent local control and survival outcome, without serious side effects
  • Harvard; 2002 (1992-1998) PMID 12173330 -- "Neuroendocrine tumors of the sinonasal tract. Results of a prospective study incorporating chemotherapy, surgery, and combined proton-photon radiotherapy." (Fitzek MM, Cancer. 2002 May 15;94(10):2623-34.)
    • Prospective. 19 patients with olofactory neuroblastoma or neuroendocrine carcinoma. Kadish Stage B 4/19, Kadish Stage C 15/19. Induction cisplatin/etoposide x2 cycles, then proton/photon RT to 69.2 CGE concomitant boost. If response, further 2 cycles of chemo. Median F/U 3.7 years
    • Outcome: 5-year OS 74%; 5-year LC 88%
    • Toxicity: 1 patient unilateral vision loss after induction chemo. 4 patients frontal/temporal lobe damage by MRI. 2 patients soft tissue/bone necrosis. No radiation-induced visual loss
    • Conclusion: This approach is successful, with radical surgery reserved for nonresponders