Neurosurgery notes/Tumours/Cyst and tumour like lesions/Esthesioneuroblastoma

Esthesioneuroblastoma

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Cyst and tumour like lesions

General

  • Aka:
    • Olfactory neuroblastoma
    • Olfactory esthesioneuroblastoma
    • Esthesioneurocytoma
    • Olfactory placode tumour
  • Growth
    • Slow growing
    • Begin as masses in superior olfactory recess → involve the anterior and middle ethmoid air-cells unilaterally → destroy surrounding bone → enter
      • Anterior cranial fossa
      • Orbits
      • Ostia of paranasal sinus → sinus opacification

Numbers

  • Incidence: 0.4/1million
  • Age: 3-90 yrs
    • Bimodal peak
      • 20-30 yrs
      • 60-70 yrs

Origin

  • Olfactory neural crest cells in the upper nares

Clinical presentation

  • Nasal stuffiness
  • Rhinorrhea
  • Epistaxis
  • Often present late as there is large amount of space for tumour to grow

Grading system

  • Histological- Hyams 1988
      • Microscopic Features
      1
      2
      3
      4
      Pleomorphism
      −
      +
      ++
      +++
      Lobular architecture
      +
      +
      +/−
      +/−
      Neurofibrillary matrix
      +++
      +
      +/−
      −
      Rosettes
      +
      +
      +/−
      +/−
      Mitoses
      −
      −
      +
      +++
      Necrosis
      −
      −
      +
      +++
    • High (1/2) vs low (3/4)
      • Low-grade tumors (Hyams 1 to 2):
        • <5 mitosis per 10 high-power fields
        • 10 yr survival 50% (Van Gompel et al 2012)
      • High-grade tumors (Hyams 3 to 4):
        • ≥5 mitosis per 10 high-power fields
        • 10 yr survival 28%
  • Clinical- Kadish 1976
    • There are a few different ones (Biller, Dulguerov and calcattera) but Kadish is the most fq used. Survival-Konuthula et al 2017
        • Modified Kadish
        Features
        10-yr survival
        Treatment
        A
        Confined to Nasal Cavity
        80%
        Endoscopic resection
        B
        Extends to Paranasal Sinus
        88%
        Endoscopic resection
        C
        Local Extension (orbit or cribriform plate)
        77%
        Bifrontal craniotomy with associated lateral rhinotomy
        D
        Distant Metastasis
        50%
        Bifrontal craniotomy with associated lateral rhinotomy
    • Paradoxically B is better than A, (Kadish stage did not correlate with survival for early-stage disease) reason
      • Selection bias
      • B receive more aggressive treatment than A
      • A is operated by non oncological surgeons
      • Database data collection error

Imaging

  • General characteristic
    • Dumpbell shaped mass: as it grows through the cribriform plate into the brain
  • CT
      • Bony destruction
        • Slow growing → bone is not aggressively destroyed but is remodelled and reabsorbed
      • Soft tissue attenuation
      • Focal calcification
      • CT+C: homogeneous enhancement
      CT
      CT
      CT+C
      CT+C
       
  • MRI
    • T1: heterogeneous intermediate signal
    • T2: heterogeneous intermediate signal
    • T1 C+ (Gd): variable enhancement (usually moderate to intense)
    • If invades intracranially → forms peritumoral cyst between esthesioneuroblastoma and brain. Used for differential diagnosis
      • T1
      T1
      T1+C fat sat
      T1+C fat sat
      T2
      T2
  • Angiography/DSA
    • Prominent tumour blush with arteriovenous shunting and persistent opacification.
  • Nuclear medicine
    • Same with other neuroblastoma
    • MIBG-avid

Histopathology

  • Macroscopic
    • Multilobulated pink-grey tumour
  • Microscopically
    • Variable differentiation,
    • Well-formed neural tissue to undifferentiated neuroblasts with pseudorosette formation

Treatment

  • Surgery → chemo/radio
  • See above

Prognosis

  • Median overall survival (Van Gompell) is typically 7.2 yrs
  • Mean progression free survival (Van Gompell) is 4.8 yrs
  • Recurrent disease occurs in 2 pattern
    • Intracranial recurrence → repeat transcranial resection or stereotactic radiosurgery
    • Distant metastasis (even with lymph node disease) → modified radical neck dissection to understand extent of the disease → chemotherapy (platinum)