Olfactory neuroblastoma

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

9. POST OP DNE

10. 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

11. 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

12. 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

13. 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

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

15. 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

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

17. DIFFERENTIAL DIAGNOSIS Ewings sarcoma Lymphoma Poorly differentiated sarcoma & carcinoma

18. HYAM’S histological grading Based on degree of differentiation , tumor architecture , mitotic index , nuclear polymorphism , fibrillary nature of matrix & tumour necrosis

19. 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

20. 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

21. 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

22. 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