Dr Gowtham Kuncha

Dept of Neurosurgery
MMHRC
 25 years female
 On and off Nasal blockage for past 6 months
 On and off epistaxis for past 6 months
 Headache since 4 days
 Patient was conscious and oriented
 No pallor, icterus, cyanosis, clubbing,
lymphadenopathy, oedema
 Vitals
 Pulse 82/min, regular
 BP 130/80 mm Hg
 Afebrile
 Resp. 18/min
 CVS S1 S2 +
 RS BAE + and equal
 P/a soft, non tender
 GCS – 15/15
 Non axial proptosis of right eye
 Pupils bilateral equal size and reacting to
light
 Restriction of adduction in right eye
 Fundus – Bilateral Papilloedema +
 Spinomotor examination Normal
 Blood and urine routine examination were
within normal limits

 1 month prior – biopsy

 Non specific inflammatory granulation tissue
 MRI showed lesion with
 Heterogenous lesion predominantly
hyperintense (T1, T2, FLAIR) expansile mass
lesion in right nasal cavity with destruction
of nasal septum extending into left nasal
cavity
 Erosion of medial wall of right orbit
 Invasion of bilateral frontal lobes of brain
 Featuras suggestive of Olfactory
Neuroblastoma
 Diagnostic Nasal Endoscopy was performed
under LA, Biopsy was taken and sent for
Frozen Section
 Positive for Malignancy – Small round cell
tumor most likely Esthesioneuroblastoma
 GA, supine position
 Bicoronal flap
 Bifrontal craniotomy with medial orbitotomy
 Mild CSF rhinorrhoea
 Lumbar puncture – rhinorrhoea subsided
 Patient was discharged 12th POD and is on
regular follow-up
 EBRT 50Gy / 25x / 5 weeks
 Cisplatin weekly
 DISCUSSION
Esthesioneuroblastoma, first described by
Berger, Luc, and Richard in 1924 is an
uncommon neoplasm that arises from
olfactory epithelium high in the nasal vault
and frequently invades the skull base, cranial
vault, and orbit. Approximately 1000 cases
have been reported in the world literature
 Morphological, immunostructural and
immunohistochemical features of
Esthesioneuroblastome are similar to that of a
neuroblastoma
 Features include
 Lobular architecture with sheets of cells in
dense neurofibrillary background
 Oval to round nuclei with scanty ill defined
cytoplasm
 Occasional rosettes and pseudorosettes
 IHC – mostly positive for Neuron specific
enolase (NSE), Neurofilament, Synaptophysin,
Chromogrannin and Leu-7
 Although the microscopic features are
similar to neuroblastoma, coexpression of
Neuron specific enolase (NSE) and
cytokeratin point towards epithelial origin
of the cells rather than neural crest origin
• Nasal obstruction (70%)
• Epistaxis (46%)
• Nasal mass
• Hyposmia
• Headache
• Rhinorrhoea
• Proptosis
• Surgical resection has been the mainstay of
managing Esthesioneuroblastoma
• Significant reduction in recurrence when
managed with both surgery and
radiotherapy compared to surgical resection
alone
• Variable response to chemotherapy, data
insufficient
 945 cases
 18% stage A, 32% stage B, 50% stage C
 Overall outcome
◦ 68% survival disease free
◦ 12% alive with disease
◦ 18% dead
 5 years Survival
◦ 72% - surgery + radiotherapy
◦ 65% - surgery alone
◦ 53% - radiation alone
 Overall survival Disease specific survival

Stage A 83% 90%

Stage B 49% 68%

Stage C 38% 66%

Stage D 13% 35%

Thank you