TUMORS OF MAXILLA AND THEIR MANAGEMENT DR KAMLESH DUBEY

INTRODUCTION Nose and PNS are rarest sites of origin of head & neck tumors. Accounts for < 1% of all malignancies. More common in 5th & 6th decade. M:F = 2:1 Late presentation with advanced disease is more common. Great variety of different histological types. Overall prognosis is poor. Distal mets is seen in 10 % at presentation

ANATOMY Maxillary tumors because of its close proximity to orbit, ethmoids, skull base, ITF always challenging to treat.

ANATOMY Orbit is the key to management of tumors of maxilla always work in subperiosteal plane.

ANATOMY Ohngren line- imaginary line between medial canthus ligament and angle of mandible.

ANATOMY Lymphatics ; Anterior pathway; Post pathway; Facial , parotid, submandibular node - upper deep cervical nodes. Post pathway; retro/ lateral pharyngeal nodes- upper deep cervical nodes. Primary lymph drainage is to retropharyngeal nodes , clinical e/o early mets is absent Only 10 % have nodal mets at presentation. Clinically palpable node is poor prognosis.

AETIOLOGY Inhalant carcinogens- 40% sinonasal malignancies. Chemicals Wood dust – adenocarcinoma (1000 times ) Hard wood- adenocarcinoma Soft wood – SCC Nickel – SCC Chemicals Chromium polycyclic hydrocarbons aflatoxin mustard gas Radiation – thorotrast Viruses - HPV 24 % inverted papilloma 4 % SCC

TUMORS OF MAXILLA Benign Inverted papilloma Osteoma ( Gardner’s syndrome ) Chondroma Fibrous dysplasia Hemangioma Leiomyoma Schwannoma

TUMORS OF MAXILLA MALIGNANT; Dental origin; Sarcoma; Others; lymphoma Squamous cell carcinoma (80%) Adeno carcinoma (5%) Adenoid cystic carcinoma (5%) Hemangiopericytoma Melanoma Undifferentiated carcinoma PNET Malignant fibrous dysplasia Dental origin; Ameloblastoma Sarcoma; Rhabdomyosarcoma Fibro sarcoma Angiosarcoma Chondro / osteo sarcoma Others; lymphoma

PATTERNS OF TUMOR SPREAD Maxillary sinus Anterior- cheek , skin Posterior- PPF, ITF,MCF, temporal bone Medial – nasal cavity, ethmoids Superiorly- orbit Inferiorly- palate, alveolus.

CLINICAL FEATURES Nasal obstruction Epistaxis Facial pain Protosis, vision disturbance Epiphora Hearing loss Cheek swelling, parasthesia Trismus Cranial nerve deficits

INVESTIGATIONS Endoscopy Imaging CT scan; Diagnostic Biopsy Site & extent Bone, skull base erosion Orbit invasion Neck- nodal status

INVESTIGATIONS MRI Better soft tissue delineation Differentiate between secretions, tumor Dural / intracranial involvement Vascularity- flow voids MRA- great vessel encasement, cavernous extension Angiography; Proximity to great vessels , sphenoid Vascular tumors Embolisation.

INVESTIGATIONS PET Biopsy HPE, Immunohistochemistry FNAC USG- B mode Distant metastasis Follow up Biopsy endoscopic guidance Sublabial approach HPE, Immunohistochemistry FNAC Neck Node USG- B mode Assesment of orbit

TNM staging Primary tumor (T) TX: Primary tumor cannot be assessed T0: No evidence of primary tumor Tis: Carcinoma in situ T1: Tumor limited to maxillary sinus mucosa with no erosion or destruction of bone T2: Tumor causing bone erosion or destruction including extension into the hard palate and/or the middle of the nasal meatus, except extension to the posterior wall of maxillary sinus and pterygoid plates T3: Tumor invades any of the following: bone of the posterior wall of maxillary sinus, subcutaneous tissues, floor or medial wall of orbit, pterygoid fossa, ethmoid sinuses T4a: Tumor invades anterior orbital contents, skin of cheek, pterygoid plates, infratemporal fossa, cribriform plate, sphenoid or frontal sinuses T4b: Tumor invades any of the following: orbital apex, dura, brain, middle cranial fossa, cranial nerves other than maxillary division of trigeminal nerve (V2), nasopharynx, or clivus

TNM staging NX: Regional lymph nodes cannot be assessed Regional lymph nodes (N) NX: Regional lymph nodes cannot be assessed N0: No regional lymph node metastasis N1: Metastasis in a single ipsilateral lymph node, 3 cm or less in greatest dimension N2: Metastasis in a single ipsilateral lymph node, more than 3 cm but 6 cm or less in greatest dimension, or in multiple ipsilateral lymph nodes, 6 cm or less in greatest dimension, or in bilateral or contralateral lymph nodes, 6 cm or less in greatest dimension N2a: Metastasis in a single ipsilateral lymph node more than 3 cm but 6 cm or less in greatest dimension N2b: Metastasis in multiple ipsilateral lymph nodes, 6 cm or less in greatest dimension N2c: Metastasis in bilateral or contralateral lymph nodes, 6 cm or less in greatest dimension N3: Metastasis in a lymph node more than 6 cm in greatest dimension

TNM staging Stage 1 - T1 N0 M0 Stage 2 - T2 N0 M0 Stage 3 - T1,2 N1 M0 T3 N0,N1 M0 Stage 4a- T4 N0,N1 M0 any T N2 M0 Stage 4b- any T N3 M0 Stage 4c- any T anyN M1

SCC 50 % arise in the antrum Advanced disease at presentation Local recurrence, distal metastasis more common Main treatment modality- Sx- PORT Primary Irradiation- inoperable tumors Chemotherpy – palliative

ADENOCARCINOMA Aetiology; Wood dust, furniture machinery, leather work Well defined tumor- better survival Long term survival poor Management; as that of SCC

ADENOID CYSTIC CARCINOMA Common in antrum Perineural spread Distal metastasis more common HPE; High grade- bad prognosis Low grade- better prognosis

radiotherapy- surgery TREATMENT T1/ T2 surgery or radiotherapy T3, T4 surgery- radiotherapy radiotherapy- surgery combined CT+ RT

SURGICAL APPROCAHES Lateral rhinotomy Weber Ferguson incision Mid facial degloving

ANASTHESIA Orotracheal intubation Throat Packing in oropharyngx Hyperventilation, mannitol- if cranial cavity is opened Adequate arrangement of blood & blood products

PRE OP PREPARATION Broad spectrum antibiotic cover Dental evaluation ( prosthesis, obturator) Neurosurgeon review; intra cranial extension, CFR Plastic surgeon; pre op decision for flaps

Lateral rhinotomy Moure`s incision (1902) Excellent exposure to nasal cavity, medial maxillary wall Indication; medial maxillextomy Cosmetically acceptable

Weber Ferguson incision Vertical limb- Weber Horizontal- Ferguson 5mm below lid margin Useful in total maxillectomy, combined orbital exenteration.

Mid facial degloving Denker & Kahler 1926 ( modified CWL approach ) 1927 Portmann & Retrouvay – mid facial degloving Mucosal incision- b/l upper GBS between maxillary tuberosity. Nose- full trans fixation , inter cartilaginous incision Excellent exposure to nasal cavity, antrum, post nasal space, pterygo palatinefossa. Combined with Le Fort 1 osteotomy – wide exposure to clivus, skull base

MID FACIAL DEGLOVING

MAXILLECTOMY Types Total Partial Infra structure Supra structure medial Subtotal

It involves removal of entire maxilla along with pterygoid plates TOTAL MAXILLECTOMY It involves removal of entire maxilla along with pterygoid plates Indication; Tumors from mucosa, filling entire antrum Soft tissue & bone sarcomas Approach; Weber ferguson Mid facial degloving

TOTAL MAXILLECTOMY Removal of entire maxilla and pterygoid plates Supine position, 15 ̊ head extension Ant wall of maxilla exposed – elevating cheek flap.

TOTAL MAXILLECTOMY Subciliary incision- Orbital periosteum elevated from floor of orbit Lacrimal sac retracted laterally after cutting medial canthus ligament, NLD Post dissection continued upto post ethmoidal artery.

TOTAL MAXILLECTOMY ORAL CAVITY – PALATAL INCISION

BONE CUTS

TOTAL MAXILLECTOMY Bony cuts; Frontal process of maxilla Palate Zygoma Ethmoidal cells, pterygoids Brisk hemorrage

TOTAL MAXILLECTOMY SSG applied over cheek mucosa defect Cavity packing over palatal prosthesis Skin closed in 2 layers

TOTAL MAXILLECTOMY POST OP CARE Pack removal Cavity irrigation Oral exercises Permanent obturator

MEDIAL MAXILLECTOMY Enblock resection of lateral wall of nasal cavity including inf/ mid turbinate, lamina papyraceae Indication; Inverted papilloma Low grade tumors confined to lateral wall on nasal cavity Approach; Lateral rhinotomy Mid facial degloving

MEDIAL MAXILLECTOMY Lateral rhinotomy incision Antero lateral wall of maxilla exposed Nasal cavity entered, tumor assesd for resectability.

MEDIAL MAXILLECTOMY Antero lateral wall opening made Bony cuts; Supt to alveolus , lateral to pyramid Vertical – medial to inferior orbital nerve Fronto ethmoidal suture Post- post wall of antrum

MEDIAL MAXILLECTPMY Bone cuts

MEDIAL MAXILLECTOMY Cavity packed Primary closure Post op – irrigation of cavity Endoscopic medial maxillextomy Acheives same success rate Avoids scar

INFRA STRUCTURE MAXILLECTOMY It involves removal of alveolus , floor of antrum, inferior part of pterygoid plates Indication; Tumor limited to alveolus, hard palate with no bony erosion No postero superior extension Approach; Mid facial degloving Lateral rhinotomy

INFRA STRUCTURE MAXILLECTOMY BONY CUTS; Horizontal- midway between alveolus & ION Palate- midline Medial- lateral nasal wall below middle meatus Post- inferior portion of pterygoid plates

INFRA STRUCTURE MAXILLECTOMY POST OP CARE; Cavity irrigation Palatal prosthesis

Orbital exenteration; MANAGEMENT OF ORBIT Orbit preservation; No e\o tumor in orbital floor Breech in floor with intact periosteum Minimal periosteal invasion Orbital exenteration; Breech in orbital periosteum Orbital apex extension

RADICAL MAXILLECTOMY WITH ORBITAL EXENTERATION Indication; Tumor invading through orbital periosteum Approach; Weber Ferguson with sub & supra ciliary extension

RADICAL MAXILLECTOMY WITH ORBITAL EXENTERATION Skin of upper lid elevated upto suprior orbital rim. Orbital perioseteum seperated from roof of orbit EOM , optic nerve cut at level of orbital apex Defect lined with SSG Facial prosthesis applied

ORBIAL EXENTERATION POST OP DEFECT PROSTHESIS IN PLACE

RECONSTRUCTION SSG Palatal prosthesis Obturators Flaps; Free flaps; Temporo pareital galeal flap Temporalis Free flaps; Rectus abdomoinis Lattissimi dorsi Composite flap; Fibula osteo cutaneous flaps

RECONSTRUCTION FOREARM FREE FLAP DENTAL IMPLANTS

RADIATION IN TUMORS OF MAXILLA Various combiations Primary irradiation alone T1,2 lesions In operable tumors Patient unfit for surgery Combined with surgery T3,4 lesions Surgery – RT RT- surgery CT+ RT

Areas to be covered; 1* site, areas of tumor extension , other routes of local spread (i-e) Maxilla, alveolus, nasal cavity, ethmoids, PPF, often orbit, neck Anterior field Lateral field

RADIATION FIELDS ANTERIOR FIELD LATERAL FIELD

Protect; Contra lateral eye Brain stem Upper cervical cord

Dose 50-55 Gy/ 20#/ 4 wks 60-65 Gy/30-33#/ 6-6.5 wks Lymphoma; Complications Mucositis Eye Cataract Keratitis Optic nerve damage Osteo radio necrosis hypopituitarism

Intra arterial infusion – 5- FU along with RT Topical 5- FU ; CHEMOTHERAPY Cisplastin & 5- FU SNUC, RMS, lymphoma Intra arterial infusion – 5- FU along with RT Topical 5- FU ; Surgical debulking + topical 5 FU weekly Adenocarcinoma- ethmoids (knegt et al, Arch of otolarygology,2007 ). Palliation.

INOPERABLE TUMORS Irradiation; 6000 rads- 6 wks Salvage surgery CT+ RT Intra arterial 5-FU

PNS TUMORS IN CHILDREN Commonest malignant tumor is rhabdomyosarcoma Treatment; triple modality Tumors; radioinsensitive Rad >3000 rads retards facial skeletal growth

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