Clinico-pathology conference KFMSR

An interesting case of paranasal tumor Department of Otorhinolaryngology, Head &

neck SurgeryDr. 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 gradingKS – 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

MicroscopySinunasal Mucosal Malignant Melanoma

MELANIN DEPOSIT MALIGNANT MELANOMA

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 RhabdomyosarcomaSmall cell neuroendocrine carcinomaMalignant melanoma

• Total maxillectomy Sinunasal mucosal melanoma

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

Ca maxillaCross road

tumors

Epiphora Chemosis

Extra axial/eccentric proptosis

Retro orbital pain Diplopia blindness

Cheek mass Hypoaesthesia

Anaesthesia rarelyNLD involvement

(<1%)Peau de orange

LN +

MalocclusionLoosening of teeth

halitosisPalatal erosion

Oro antral fistula

TrismusNeuralgic pain

Pterygoid involvementExtension intracranially

Through natural foramensSOM

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