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

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

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

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

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

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

Olfactory Neuroblastoma Rhadomyosarcoma Small cell neuro endocrine carcinoma Malignant Pecoma

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

Microscopy Sinunasal Mucosal Malignant Melanoma MELANIN DEPOSIT

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

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

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

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

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

Maxillary sinus tumor

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)

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

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)

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.

Team work, works