1. OLFACTORY NEUROBLASTOMA
Dr.K.Anandhakumar
Post Graduate DLO

2. OLFACTORY NEUROBLASTOMA
46Y/F referred to our institution as neuro
endocrine tumor left nasal cavity
c/o bleeding through left nasal cavity one month
ago lasted for 2 days

3.  Under gone excision biopsy 2 month ago in
private hospital for left nasal cavity mass as
sino nasal malignancy and biopsy showed as
neuro endocrine tumor

4. MENSTRUAL H/O
 Irregular menstrual cycle
 h/o PS done 16 years back

5. TRANS NASAL ENDOSCOPIC EXCISION OF LEFT
NASAL CAVITY MASS

6. BIOPSY REPORT
 Malignant round cell neoplasm – features
suggestive of olfactory neuroblastoma

7. IHC
 SYNAPTOPHYSIN – focal positive in tumor
cells
 CYTO KERATIN _ negative
 CHROMOGRANIN _ Negative

8. POST OPERATIVE CT

11. POST OP DNE

12. DISCUSSION
 Olfactory neuroblastoma first described by
BERGER & LUC in 1924.
 Probable origin :
 sphenopalatine ganglion ,
 vomero nasal organ of Jacobson,
 neuroepithelial cells of the olfactory membrane,
 ectopic olfactory epithelium in nasal mucosa &
APUD cells

13. DISCUSSION
 Olfactory neuroblastoma –arises from basal
cells within the olfactory neuro epithlium.
 Represents < 5% of all sinonasal
malignancies
 Incidence : Bimodal distribution , peak at 20
& 50 years of age
 Common in females

14. PATHOPHYSIOLOGY
 Undifferentiated tumour of neuroectodermal
origin derived from olfactory neuroepithelium
 Tumour cells are mitotically active that
develop into sustentacular & neuronal cells
 It contains variable arrangement of small
cells
 There exist variable presence of true
rossettes & neurofibrillary material

15.  Neuro endocrine tumor capable of causing
paraneoplastic syndromes by secreting
peptides
 Can cause Cushing’s syndrome ,
inappropriate anti diuretic hormone secretion
or hypertension produced by vasoactive
peptides

16. CLINICAL FEATURES
 COMMON :  LESS COMMON:
 nasal obstruction ,  sinus pain ,
 epistaxis ,  visual changes ,
 persistant nasal  head ache,
discharge  proptosis ,
 Diplopia,
 Hyposmia,
 Anosmia,
 facial pain,
 facial swelling & syncope

17. INVESTIGATIONS
 CT : homogenous soft tissue mass in the nasal cavity producing
some erosion of lamina papyracea ,
 cribriform plate & fovea ethmoidalis
 MRI : T1 weighted image : hypo intense to gray matter
 T2 weighted image : iso / hyper intense

18. IMMUNO HISTO CHEMISTRY
 Synaptophysin
 Neuro filament protein
 S100
 Chromogranin
 Neuron specific enolase

19. DIFFERENTIAL DIAGNOSIS
 Ewings sarcoma
 Lymphoma
 Poorly differentiated sarcoma & carcinoma

20. HYAM’S HISTOLOGICAL GRADING
 Based on
 degree of differentiation ,
 tumor architecture ,
 mitotic index ,
 nuclear polymorphism ,
 fibrillary nature of matrix & tumour necrosis

21. KADISH CLINICAL STAGING SYSTEM
 WITH MORITA’S MODIFICATION
 A – limited to nasal cavity
 B – involving nasal cavity & sinuses
 C – extension beyond nasal & paranasal
sinuses cavities
 D – tumour with metastasis to cervical nodes
or distant sites

22. TREATMENT
 Surgery followed by adjuvant radiotherapy
 Surgery
 CRANIOFACIAL approach involving ENT , Head &
Neck and neurosurgical treatment
 Radiotherapy
 For stage A & B 4500 – 5500 rads for 5 weeks
 For stage C 6000 – 6500 rads for 7 weeks

23.  Recurrent metastatic disease –
Chemotherapy
 Cisplatinum – 60 mg/ sq. m on day 1
 Etoposide – 120 mg / sq. m on day 1,2,3
 Cyclophospamide ,thio – TEPA ,high dose
CAV with stem cell support also used

24. PROGNOSIS
 5 & 10 Year survival rate – 80 % & 50 % with
radical surgical procedure followed by RT
 POOR PROGNOSTIC FACTORS:
 Age > 50 at presentation
 female gender
 Tumour recurrence
 metastasis