1. Clinico-pathology conference KFMSR
An interesting case of paranasal tumor
Department of Otorhinolaryngology, Head & neck Surgery
Dr. Vijay R , Asst Prof, ENT

2. History Mrs.XX 37 Yrs working as a tobacco binder presenting with
Left eye purulent discharge - 6months
Ext DCR(Dacryocystorhinostomy) was done at an outside hospital -5months
Recurrence of symptoms within – 4 months
Left sided cheek swelling – 4months
Left sided hypoaethesia - 3 ½ months
Left nose block – 3months
Epistaxis – 1months

3. Clinical Findings Clinical examination O/E
ECOG – 0 to 1, KS >80<100
AR – soft bulge within left nostril
DNS to right
Oral SMF , Gr II trismus
No palpable neck nodes
No bony tenderness
Sys exam – WNL
Ophthal W/U – B/E aquity 6/6
ECOG – Eastern cooperative oncological grading
KS – Karnofsky score

4. Imaging Partially enhancing soft tissue density Left maxilla
Anterior ethmoids
Extending into nasal cavity proper with partial erosion roof maxilla and erosion of anterior wall but posterior wall of maxilla is preserved
Obliterated Nasolacrimal duct pathway
Obliterated infraorbital foramen

5. Biopsy from left maxilla
GROSS: Multiple pale white tissue bits measuring 1x0.5 cm(AE)
MICROSCOPY:
Infiltrating tumour composed of cells arranged in nests, singly scattered and focal alveolar pattern.
Round to spindle cells with scant to moderate amount of eosinophilic cytoplasm and hyperchromatic nuclei few showing prominent nucleoli. Some areas showed nuclear moulding.
No mitosis/necrosis/lymphovascular invasion

6. Probable Diagnosis ? MALIGNANT SMALL ROUND CELL TUMOUR
Differential Diagnosis:
Alveolar Rhabdomyosarcoma
Small cell neuroendocrine carcinoma
Malignant melanoma
Olfactory neuroblastoma
Malignant PECOMA

7. Olfactory Neuroblastoma
Rhadomyosarcoma
Small cell neuro endocrine carcinoma
Malignant Pecoma

8. Maxillectomy Specimen
Specimen sent in 3 parts
Largest one measuring 6x4.5x4 cm
Smallest measuring 3x3x1 cm

9. Microscopy Sinunasal Mucosal Malignant Melanoma
MELANIN DEPOSIT

10. Malignant Melanoma
Approximately 1% of all malignant melanomas occur in the nasal cavity and paranasal sinuses.
Paranasal sinuses- antrum(80%) followed by ethmoid

12. Malignant Melanoma
Prognosis: Poor with a 5 year survival rate of 15 to 30%

13. Surgery - Procedure Pathology (Diagnostic Nasal Endoscopy & Biopsy)
Alveolar Rhabdomyosarcoma
Small cell neuroendocrine carcinoma
Malignant melanoma
Total maxillectomy
Sinunasal mucosal melanoma
Surgery - Procedure

14. Maxillary sinus tumors
Most common site (60-70%)
Squamous cell carcinoma- MC (80%)
Multi factorial – mustard gas, nickel dust (AC), thorotrast, isopropyl oil, chromium,DDS & wood dust(SSC – t21)
Furniture, leather & textile industry
HPV, EBV – Inverted papilloma
Malignant melanoma -very rare <1%
irritants and carcinogens , such as tobacco smoke, implicated in the development of this malignancy

15. Extra axial/eccentric proptosis Retro orbital pain Diplopia blindness
Epiphora
Chemosis
Extra axial/eccentric proptosis
Retro orbital pain
Diplopia
blindness
Cheek mass
Hypoaesthesia
Anaesthesia rarely
NLD involvement (<1%)
Peau de orange
LN +
Ca maxilla
Cross road tumors
Trismus
Neuralgic pain
Pterygoid involvement
Extension intracranially
Through natural foramens
SOM
Malocclusion
Loosening of teeth
halitosis
Palatal erosion
Oro antral fistula

16. Maxillary sinus tumor

18. Approaches Endoscopic modified Denker s procedure
Moure Lateral rhinotomy approach
Classical Weber Ferguson approach
Modified transconjunctival approach
Intracranial extension
Orbital exenteration
Skull base involvement (Craniofacial resection planned)

19. Reconstruction
Immediate – GP/patty mix with initial obturator with or without skin grafting
Intermediate – temporary obturator made from initial obturator
Permanent – when the treatment modalities are complete and no more shrinkage is expected
- permanent obturator
- bone graft with dental implants
Ocular support/titanium mesh – if whitnalls/lt canthal ligament is transected

20. Complications Cornea injury – tarsoraphy/sheilding
Bleeding – III internal max artery
CSF leak – CFR, high osteotomy
Orbital injury – periorbital injury
Velopharyngeal insufficiency (VPI) - Temp
Eustachian tube injury - scarring
Epiphora – NLD injury
Infection, flap necrosis, prosthetic disturbance
Trismus, discosmesis, persisting VPI (very rare)

21. Maxillary sinus tumor
Though postoperative ChemoRT has no proven increased survival rate, still it is internationally accepted as an adjunct
Adjunct only not mainstay in treating maxillary tumors unlike laryngeal tumors.
Even if periorbita is involved exenteration is a standard procedure rather than subjecting the patient to ChemoRT which would anyway destroy patients vision.

22. Team work, works