Moderator- Dr. Baldev Singh (Professor & Head) Presenter- Dr.

Gaurav Kataria

NEOPLASIA
Means Process of new growth

Defined as an abnoramal mass of

tissue, the growth of which exceeds & is uncoordinated with that of normal tissues and persist in the same excessive manner after cessation of the stimuli which evoked the change.

Types
Benign Usually smooth, localised and covered with mucous membrane Malignant Usually friable, having granular surface & have bleeding tendency

Features
Characterized by Pleomorphism Abnormal nuclear morphology Mitoses Loss of polarity Hyperchromatism Other changes like tumor giant cells and polymorphism

SINONASAL NEOPLASM
3% of aerodigestive malignancies
1% of all malignancies male : female 2:1

Sixth to seventh decade

Predominately of older males Exposure: Wood, nickel-refining processes Industrial fumes, leather tanning Cigarette and Alcohol consumption

CLASSIFICATION EPITHELIAL
Benign

Squamous papilloma Inverted papilloma Columnar papilloma Adenoma Nasal dermoid

Malignant

Squamous cell carcinoma Transitional cell carcinoma Adenocarcinoma Adenoid cystic carcinoma Melanoma Olfactory neuroblastoma Undifferentiated carcinoma Basal cell carcinoma

Nonepithelial
Benign
Fibroma Chondroma Osteoma Neurilemmoma Neurofibroma Hemangioma leiomyoma

Malignant

Soft-tissue sarcoma Rhabdomyosarcoma Leiomyosarcoma Fibrosarcoma Liposarcoma Angiosarcoma Myxosarcoma Hemangiopericytoma Connective tissue sarcoma Chondrosarcoma Osteosarcoma Malignant histiocytoma Osteogenic sarcoma Neurogenic tumors

Lymphoreticular tumors
Lymphoma Plasmacytoma

Giant cell tumor

Metastatic tumours

 Tumors of the nasal cavity proper are

approximately evenly divided between benign and malignant neoplasia, with inverted papilloma predominating in the benign group& squamous cell carcinoma in the malignant. On the other hand, most sinus tumors are malignant with squamous cell carcinoma being the most prevalent.

 The maxillary sinus is most commonly involved with tumor, followed by the nasal cavity, the ethmoids and then the frontal & sphenoid sinuses.

Maxillary sinus most common (55 %) Nasal cavity 35 %

Ethmoid sinuses 9 %
Frontal and sphenoid sinuses (1 %)

Benign Sinonasal Tumors

EPITHELIAL Squamous papilloma Inverted papilloma Columnar papilloma Salivary Adenoma Nasal dermoid

NON EPITHELIAL

Ossifying Fibroma Chondroma lipoma Osteoma Ameloblastoma (adamantinoma) Fibrous dysplasia Neurilemmoma Neurofibroma Hemangioma meningioencephalocoele Schwanoma,meningioma, glioma Angiofibroma leiomyoma

Squamous Papilloma
Mainly occurs in skin of the nasal vestibule & septum (

lower part ) Usually solitary, pedunculated or sessile warty lesions Also k/a everted papilloma Sometimes causes nasal obstruction Treatment local excision with cauterisation of base to prevent recurrence Cryosurgery or laser can also be used

Papilloma
Nasal papillomas are true neoplasms and, while their etiology is unclear, they are known to arise from the nasal respiratory epithelium, which undergoes metaplastic change and proliferation.
Papillomas differ from inflammatory polyps, which are more common, in that inflammatory polyps are associated with allergic rhinitis and are actually reactive lesions, not a tumor.

Three types: Fungiform papillomas 50 % of all arises from nasal septum, cylindrical papillomas 3% of total from lateral wall or sinus inverted papillomas 47 % of total from lateral wall

Inverted Papilloma
Also k/a transitional cell papilloma & ringertz tumor
4 % of all nasal neoplasms 4 / million population, rare in blacks

Male > female, 3: 1 to 10: 1

Age affected 40- 70 years Most common site lateral wall of nose Presents as unilateral, friable, pale , pink mass arising

from middle meatus Diagnosis made by punch biopsy

 Their etiology is unclear.

They are known to have high recurrence rates. They are associated with malignancy and also

have locally aggressive growth patterns, which makes them technically difficult to remove. There is also controversy over the appropriate surgical approach for tumor removal. There is a role for radiation therapy

 Inverted papillomas arise from the Schneiderian membrane, which is an invagination of the olfactory ectoderm that occurs during the fourth week of embryonic development. The mucosa creates a transitional zone between the endodermally-derived respiratory epithelium of the nasopharynx and keratinizing squamous epithelium with the nasal vestibule.

 Grossly, inverted papillomas appear more opaque than inflammatory polyps, and this is because they have a thick epithelial layer.

Inverting papilloma traces its name to the

histologic appearance with squamous epithelium inverted in the polyps

 They are commonly located in the

nasal cavity and they typically involve an adjacent sinus. The most common location is the lateral wall of nose, but other common locations include the ethmoid sinus and maxillary sinus. They have even been found in the nasopharynx.

Clinical features
Symptoms
Nasal obstruction Facial swelling

Hyponasal speech
Epistaxis & blood stained discharge Rhinorrhoea

Pressure & pain

Orbital symptoms- proptosis,diplopia, epiphora Hyposmia, anosmia

 Most common symptoms are unilateral and include

can be an incidental finding on examination. These tumors are rare. They occur about 0.6 cases per 100,000 per year and they occur approximately 1/25th as often as inflammatory polyps. The recurrence rates cited in the literature varies anywhere from 11% to 78%, and this depends a lot on the treatment modality used.

nasal obstruction, nosebleed and nasal discharge. It

 They are associated with malignancy, 5% to 15% malignancy rates are most generally accepted. Inverted papillomas are more commonly associated with squamous cell carcinomas. There are four types of association:
Metachronous squamous cell carcinoma. Carcinoma in situ within the IP Synchronous lesions Malignant transformation

Differential diagnosis
A.C. polyp
Allergic fungal sinusitis Sq. cell ca

Adenocarcinoma
esthesioblastoma

Treatment
Traditionally procedures have been either a transnasal procedure with polypectomy or confined transnasal polypectomy with additional sinus procedure, such as Caldwell-

Luc. The gold standard was lateral rhinotomy with medial maxillectomy. Radiation therapy can be used as the sole therapy for inverted papilloma or it can be used postoperatively. The absolute indication for radiation therapy is when an inverted papilloma is associated with squamous cell carcinoma

 The patients who should get radiation therapy are those who had advanced incompletely resected or unresectable lesions that are biologically aggressive, or patients where morbidity in resection would be more pronounced that morbidity of tumor radiation Enbloc ethmoidectomy , medial maxillectomy & mid facial degloving also used

Haemangioma
M.C site nasal septum Nasal tip commonest site in external nose Other sites turbinates,nasopharynx, external nose, upper jaws,

frontal bone, nasal bone & maxilla Age group mainly 20-50, with F > M M.C presenting feature.nasal obstruction and recurrent epistaxis. Delayed treatment causes broadening of nose and deformity

Types
Capillary haemangioma ( bleeding polypus of septum)- soft, dark

red, pedunculated or sessile arising from anterior part of nasal septum, local excision done .Laser excision is TOC

 Cavernous haemangioma
Arises from turbinates & lateral wall of nose Surgical excision with cryotherapy is TOC

In extensive lesions radiotherapy along with surgical

excision used

Osteoma
Benign osteogenic tumor of slow growth containing

mature bone Most commonly seen in frontal sinus followed by ethmoid & maxillary 15-40 yrs age group m.c. affected

Types
Localized Ivory osteoma of compact bone frontal sinus Cancellous bone - maxillary/ ethmoid sinus

 Mainly asymptomatic, becomes symptomatic when

they block the sinus drainage & mucocele forms Clinical features asymptomatic sinusitis mucocele Being discovered incidentally on X- rays Complete removal of the tumor with its base attachment is done by bicoronal osteoplastic flap technique

 Ethmoid osteomas cause proptosis & always invaded

the orbit If they extend posteriorly they can be contiguous with sphenoid bone & are close to optic nerve So simple excision not sufficient they require frontoethmoidectomy Gardners syndrome is an autosomal dominant syndrome of osteomas with other soft tissue tumors and intestinal polyposis. Treatment of osteomas is excision by osteoplastic flap technique or through Lynch-Howarth external frontoethmoidectomy approach

AETIOLOGY

Fibrous dysplasia

Normal bone replaced by collagen, fibroblasts, and osteoid material Most commonly involves maxillary but some times ethmoid & frontal sinuses F> M Usually diagnosed in infancy or childhood & growth may cease after puberty Presents as painless, slow growing swelling, nasal obstruction & progression to deformity Common site in maxilla is canine fossa area or zygomatic area, alveolus is frquently involved

3 histological forms Active form rich in cellular connective tissue with numerous mitotic figures Quiescent more mature connective tissue with fewer mitosis

Inactive form degeneration of connective tissue & matrix

Functional disabilities
Orbital - proptosis, impaired vision if o.n.

compressed, epiphora Oral dysarthria, malocclusion, difficulty in chewing Polystotic form with cutaneous pigmentation & endocrine abnormality called Albrights syndrome

Radiography
X ray shows ground glass appearance of tumors ( fine

orange peel texture ) CT shows obliteration of sinuses with obliteration of infraorbital margin Treatment Surgical resculptring of the involved bone to achieve a good cosmetic & functional result

Ossifying Fibroma
Encapsulated, slow growing benign fibro-osseus

tumors composed of fibrous tissue mixed with varying account of mature bone F>M 3 rd to 4 th decade Most common site is maxilla Lesions occuring in sinuses & cranial bones are more aggressive Diagnosed accidentally on radiographs

Clinical features Facial swelling Deformity Proptosis Malocclusion Treatment surgical excision Prognosis excellent after surgical excision

CT Picture

Chondroma
Can arise from ethmoid, nasal cavity or nasal septum
Smooth, firm & lobulated tumors Other types mixed fibro, osteo & angiochondromas

Treatment is wide surgical excision because of their

tendency of malignant transformation

Schwannoma
Sinonasal schwannoma accounts for 4% of peripheral

benign nerve tumors Arises from surface of nerve fibre Age group 1 st decade to 8 th decade Positive S-100 protein staining Usually lacks the capsule Can present as massive lesions with bony erosions in nasal cavity No malignant degeneration Usually involves trigeminal & ANS Surgical excision by lateral rhinotomy

Meningioma
Extracranial meningioma arises from ectopic

arachnoid tissue Can also spread to frontal sinus Can also present intranasally Locally recurrent & difficult to operate Surgical excision Radiotherapy can also be used for inoperable tumors

Glioma
30 % are intranasal
10 % both intra & extra nasal Seen mainly in infants & children

Presents as firm polypoidal mass sometimes

protruding at the anterior nares Surgical excision is TOC

Neurofibroma
Arises within nerve fibres
Associated with Von Recklinghausens disease Malignant transformation in 15 % cases

Complete surgical excision TOC

5 yrs survival rate 30%

Leiomyoma
Sinonasal leiomyoma very rare
Presnt as purplish mass causing nasal obstruction Treatment complete excision

No recurrence after complete excision

Intranasal meningoencephalocele
Herniation of brain tissues & meninges through

foramen caecum or cribriform plate Presents as smooth polyp in the upper part of nose between the septum & m.t. Usually seen in infants & young children Mass increases in size on crying or straining CT scan essential to demonstrate defect in skull base If it is misdiagnosed & avulsed, it results in meningitis or CSF rhinorrhoea

 Treatment is frontal crainotomy severing the stalk

from the brain & repair of dural or bony defect Intranasal mass can be removed as secondary procedure after cranial defect is sealed

Angiofibroma
Uncommon, benign, slow growing tumors
Most commonly arise from sphenopalatine foramen or

the posterolateral wall of roof of the nose Locally destructive tumors causing invasion into the nasal cavity, maxillary sinuses, orbit & some times in cranium

Extensions
Nasal cavity
Paranasal sinuses Pterygomaxillary fossa

Infratemporal fossa
Cheek Orbit Cranial cavity

Features

M> F Profuse & recurrenty epistaxis Progressive nasal obstruction Denasal speech Facial swelling Conductive hearing loss Serous otitis media Mass in nasopharynx Proptosis Broadening of nasal bridge Involvement of 2,3,4,6 cranial nerves

 On CT scan antral sign or holman miller sign which is

pathognomic Surgical excision is TOC Transpalatine approach Transpalatine + sublabial Extended lateral rhinotomy Transmaxillary Maxillary swing approach Infratemporal approach

Salivary adenoma
Rare tumors
Usually arises from nasal septum Presents with nasal obstruction

Wide surgical excision TOC

Adamantinoma
Also c/a ameloblastoma
Locally aggressive tumor Arises from odontogenic tssue

M.C involves maxillary sinus

Surgical excision is TOC

Nasal dermoid
Lined by squamous epithelium Male = female Contain skin appendages like hair follicles, sebaceous

& sweat glands Solid or cystic painless mass Present as widening of upper part of nasal septum with splaying of nasal bones & hypertelorism Pit or sinus may be seen in the midline of nasal dorsum with hair protruding out of it. Complete local excision TOC

Malignant sinonasal tumors

Epithelial

Squamous cell carcinoma Transitional cell carcinoma Adenocarcinoma Adenoid cystic carcinoma Melanoma Olfactory neuroblastoma Undifferentiated carcinoma Basal cell carcinoma Soft-tissue sarcoma Rhabdomyosarcoma Leiomyosarcoma Fibrosarcoma Liposarcoma Angiosarcoma

Nonepithelial

 Myxosarcoma Hemangiopericytoma Connective tissue sarcoma Chondrosarcoma Osteosarcoma Malignant histiocytoma Neurogenic tumors

Lymphoreticular tumors Lymphoma Plasmacytoma Giant cell tumor

Malignant Sinonasal Tumors

EPIDEMIOLOGY < 1 % of all neoplasms 3% of tumors of upper aero digestive tract 0.44% of all body cancers in India Incidence 0.5-1 / 100,000/ yr 5th -6th decade M:F 2:1 Avg delay between the first symptom and diagnosis six mths

Origin (Scott Brown) Maxillary sinus most common (55 %) Nasal cavity 35 % Ethmoid sinuses 9 % Frontal and sphenoid sinuses (1 %) Despite their infrequence, they represent both a diagnostic and therapeutic challenge because the presenting signs and symptoms may be indistinguishable from benign or inflammatory disorders.

AETIOLOGY Wood workers 70 times increased incidence particularly in ethmoid African mahogany most dangerous Biologically active compounds in wood dust impair mucociliary clearance and predispose to carcinogenesis Hardwood exposure - adenocarcinoma Soft wood exposure - squamous cell carcinoma

 Nickel

Relative risk >250 Interval between exposure to nickel and tumor 18 to 36 years Chromium Leather industry Polycyclic hydrocarbons Smoking synergistic with wood dust Aflatoxin (found in certain foods and dust) Mustard gas Thorotrast (thorium dioxide used in paints for watch dials) Radiation Viral EBV, HPV Use of snuff (cocaine) Genetic role suggested but not proven

Sinus anatomy
Maxillary antrum Significance
Superior orbit, ethmoids Posterior pterygoids, infratemporal fossa

Ethmoid sinus Significance

Superior fovea, cribiform Medial lamina papyracea

Sphenoid sinus Significance Superior optic nerve, pituitary Lateral ICA, cavernous sinus Lateral wall < 0.5mm Inferior vidian nerve

Frontal sinus Significance Inferior orbit Posterior anterior cranial fossa

Lymphatic drainage
The anterior nose has the same lymphatic

drainage as the external nose. These tend to spread to the submental or level I area. The posterior nose tends to drain to the retropharyngeal nodes as well as the lateral pharyngeal nodes, which eventually drain into the level II.

Squamous cell carcinoma

Most common malignant tumor (80%)

Location: Maxillary sinus (70%) Nasal cavity (20%) 90% have local invasion by presentation Commonly associated with bone erosion of lateral nasal wall, palate, alveolus & sometimes pterygoid plates, orbit, cribriform plate & posterior ethmoids & have poor prognosis. Spread Antral to- nasal cavity, ethmoid, orbit via IOF, Infratemporal fossa, buccal sulcus,palate or alveolar ridge through dental foramina, & soft tissues of cheek by erosion of anterior wall

From ethmoid to maxillary antrum, orbit, nose, sphenoid by direct spread & anterior fossa through fovea or cribriform plate Lymphatic drainage: From maxillary sinus to retropharyngeal nodes then to submandibular nodes from ethmoid sinus to jugulodiagstric & subdigastric nodes Surgical resection with postoperative radiation

Squamous cell carcinoma In nose it may arise from vestibule, anterior part of nasal

septum or lateral nasal wall Male > female Age group 50-70 Types Vestibular arises from lateral wall of nasal vestibule & may extend in to columella, nasal floor & upper lip with metastases to parotid nodes Septal arises from mucocutaneous junction & causes burning & soreness in nose.also k/a nose pickers cancer Lateral wall it is the m.c. site. Extends in to ethmoid or maxillary sinuses.presents as polypoidal mass. Metstases rare & treatment is combination of radiotherapy & surgery

Adenocarcinoma

2nd most common malignant tumor 9 % of total sinonasal malignancies Male > female , 5th to 7 th decade Present most often in the superior portions Strong association with occupational exposures like nickel & wood dust Common in ethmoid sinuses

GRADES
High grade

Solid growth pattern with poorly defined margins 30% present with metastasis . Pain & deformity also present. Bad prognosis Low grade Uniform and glandular with less incidence of perineural invasion/metastasis

Adenoid Cystic Carcinoma

3rd most common malignancy Around 5 % of total sinonasal malignancies Arises from minor salivary glands with in nasal cavity &

maxillary antrum M.c. affected site maxillary sinus Perineural spread & Vascular spread also present istologically 3 types tubular, cribriform & solid Perineural spread occurs through infraorbital nerve, maxillary nerve, greater palatine nerve & sphenopalatine foramen.

Adenoid Cystic Carcinoma

Despite aggressive surgical resection and radiotherapy,

most grow insidiously Neck metastasis is rare and usually a sign of local failure Postoperative RT is very important Prognosis worst

Olfactory Neuroblastoma (Esthesioneuroblastoma)

Tumor of olfactory placode Originate from basal stem cells of neural crest origin. Around 5 % of all sinonasal malignancies Peak at 20 and 50 yrs. With bimodal distribution If peak at 20 yrs, less local recurrence & more metastasis If peak at 50 yrs, more local recurrece & less metastasis Both sex equally affected Presents as cherry red, polypoidal mass in upper third of nasal cavity. Vascular tumor & bleeds profusely on biopsy Kadish Classification A: Confined to nasal cavity B: Involving the paranasal cavity C: Extending beyond these limits D: With mets to neck or distant sites

UCLA Staging system

T1: Tumor involving nasal cavity and/or paranasal sinus,

excluding the sphenoid and superior most ethmoids T2: Tumor involving the nasal cavity and/or paranasal sinus including sphenoid/cribriform plate T3: Tumor extending into the orbit or anterior cranial fossa T4: Tumor involving the brain Aggressive behavior Local failure: 50-75% Metastatic disease develops in 20-30% Treatment: En bloc surgical resection with postoperative RT

Chondrosarcoma
Rare septal lesion
Divided in 1-3 grades histologically Low grade are difficult to distinguish from chondroma

Ring form calcification seen on CT

Prognosis poor if present in posterior part of nose &

PNS & often associated with skull base erosion & destruction

Osteogenic sarcoma
Rare tumor of nose & PNS but most common bone

tumor Usually affects maxilla Can also involve sphenoid bone Presents as an enlarging, firm mass which is rock hard on palpation Surgical excision difficult due to involvement of skull base 5 yrs survival rate 10 30 %

Rhabdomyosarcoma
Common in children
M.c. sinus malignancy in children Embryonal variety m.c. which have good prognosis

It presents in nose & PNS as polypoidal outgrowth

Positive for desmin immunological marker Treatment is triple therapy with radio, chemo &

surgery.

 Vascular tumor arise from pericyte of capillaries Low grade malignant potential Painless grey to tan coloured polypoidal, spongy,

Haemangiopericytoma

haemorrhagic mass Age 5-80 yrs, male = female Presents with epistaxis, nasal obstruction, facial swelling & proptosis Positive for vimentin, CD34, & clotting factor X111 a Occisionally causes paraneoplastic syndrome Wide local excision is TOC, RT used in inoperable cases Recurrence rate 18 %, 2.5% metastatize

Malignant Melanoma
1 % of

nose & PNS cancers M.C. site septum & lateral nasal wall ( in nasal cavity m.c. site septum followed by middle & inferior turbinates) Origin from sinus mucosa rare Peak age 5th to 8th decade, M >F Present as polypoidal slaty grey or bluish black mass Tumor spreads by lymphatics & blood stream Less than 40% have neck nodes Wide surgical excision is TOC Prognosis poor, with 5 yr survival rate 30% Melanoma is positive for S-100, vimentin & HMB -45 immunological markers.

Malignant fibrous histiocytoma

Arises in previous bone diseases like fibrous dysplasis
Associated with irradiation in that area Presents as gradually expanding mass which is hard on

palpation If it invades skull base it is difficult to resect it

Plasmacytoma
Solitary plasmacytoma may be seen without

generalised osseous disease in nasal cavity Males > females Age around 40 yrs Tumors of well differentiated B lymphocytes Treatment is RT later followed by surgery 20 % develop multiple myleomatosis

Lymphoma

Non-Hodgkins type Median age 50 Male > Female Common site nasal septum Most have no other evidence of lymphoma Express T cell markers like CD2, CD43, CD 45RO Ass. With EBV Treatment is by radiation, with or without chemotherapy Survival drops to 10% for recurrent lesions Adverse prognostic factors are asian patients, age > 60, advanced Ann Arbor stage

Basal cell carcinoma

External nose is m.c. site for BCC in head & neck
6 th to 8 th decade Male = female

Presentation vary from small nodular growth to

chronic ulcer or ulceronodular lesions Border of ulcer may be rolled out or flat Wide & aggressive surgical excision is TOC as recurrence is common Distant metastasis rare

Picture showing B.C.C. of nose

Metastatic tumors
Renal cell carcinoma m.c. lungs

breasts
urogenital tract gastrointestinal tract Palliation necessary

Carcinoma of Maxillary sinus & Ethmoid sinus

Arises from sinus lining & may present as sinusitis
Then it spreads to destroy the bony confines of the

maxillary sinus & invade surrounding structures

Ohngrens classification

Ohngren line
Running from the medial canthus of orbit to angle of

mandible Separates tumours into two groups Those that developed above the line Those that developed below it Ohngren suggested Superiorly based cancers more aggressive and poorly differentiated Tumours arising from below line more amenable to treatment With newer imaging & surgical techniques, no longer used now

Lymphatic drainage
Lymphatic drainage of nose and paranasal sinuses

relatively scanty Two lymphatic pathways Anterior Anteroinferior part of nasal cavity and skin of vestibule Drain to facial, parotid and submandibular lymph nodes the first echelon nodes These drain into the upper deep cervical chain Posterior Remainder of nose and the paranasal sinuses Pathway which runs anterior to the Eustachian tube to first echelon nodes - the retropharyngeal lymph nodes Further drain to upper deep cervical chain

PATTERNS OF TUMOUR SPREAD Local spread Tend to fill sinus cavity before eroding bony walls Periosteum, perichondrium and dura seem to act as a temporary barriers Bone of the antronasal wall, canine fossa and orbital floor very thin easily destroyed Only 25 percent of maxillary sinus carcinomas are contained within the antrum at time of presentation Regional spread Lymphatic spread apparent in 25-35 % of patients at some time during the course of their disease Only 10 % have nodal disease at time of presentation Submandibular and jugulodigastric nodes most commonly involved

 Bilateral lymph node involvement likely when tumor

near midline Distant spread Adenocarcinomas 18% Squamous cell carcinomas 10% Common sites bone, brain, liver, lung, skin

Clinical features

SYMPTOMS Nasal: 50% Obstruction, epistaxis, rhinorrhea Oral symptoms: 25-35% Pain, trismus, alveolar ridge fullness, malocclusion, erosion Ocular: 25% Epiphora, diplopia, proptosis Facial Paresthesias, asymmetry Neck mass Ears Hearing loss, serous otitis media

PHYSICAL FINDINGS Nasal, facial, or intraoral mass Intranasal mass Often necrotic, but polypoid mucosa may obscure Facial swelling antral tumor erodes into cheek Widening of the upper alveolar ridge Loose teeth Palatal mass and ulceration

 Proptosis Cranial nerve deficits

CN II, III, IV, VI

CN V ( V1 and V2) Complete H&N exam and Endoscopy

Diagnostic Assessment CT scan Three-dimensional image of the lesion Bone destruction, orbital & intracranial involvement MRI Better soft tissue delineation Ability to differentiate between tumor bulk and retained secretions Combined with CT for planning surgery for sinus neoplasms Angiography If the lesion demonstrates enhancement during initial CT study If it approximates carotid system In evaluation of unusual tumors involving the sphenoid sinus and skull base In vascular tumors for assessment of tumor extent, feeding vessels and in combination with embolization

 Ultrasound

B-mode scanning orbital masses PET Follow-up after concomitant chemoradiation Assessing presence of metastatic disease Endoscopy and Biopsy Punch biopsy Chances of bleeding Tumors contained within the sinus cavities should be biopsied transnasally Dental / prosthetic consultation

 Workup for distant metastasis

CXR PA view USG abdomen

Workup for surgery Hb, TLC, DLC INR, Platelet count Bld Grouping Urine RE,ME BS F/PP LFT, RFT, Electrolytes ECG Lipid profile

Staging of Maxillary sinus cancer (AJCC )

Staging of nasal cavity & ethmoid sinus T1 tumor restricted to any one subsite, with or without bony

invasion T2 Tumor invading two subsites in a single region or extending to involve an adjacent region within the nasoethmoidal complex, with or without bony invasion T3 Tumor extends to invade the medial wall or floor of the orbit, maxillary sinus, palate, or cribriform plate T4a moderately advanced local disease Tumor invades any of the following : anterior orbital contents, skin of nose or cheek, minimal extension to anterior cranial fossa, pterygoid plates, sphenoid or frontal sinus T4b- very advanced disease tumor invades any of the following : orbital apex, dura, brain, middle cranial fossa, cranial nerves other than V 2, nasopharynx or clivus

TREATMENT
General principles Most patients have very advanced disease at presentation All investigations & accurate staging Choice between treatment for cure and palliation Options for patients potentially curable Surgery Radiotherpy Chemoradiotherapy Combinations Infusion & perfusion techniques

Surgical options
External Ethmoidectomy
Inferior Medial Maxillectomy Medial Maxillectomy

Radical Maxillectomy
Craniofacial Resections Extended Craniofacial Resection Minimally Invasive Approaches

Surgical approaches
Endoscopic
Lateral rhinotomy Transoral/transpalatal

Midfacial degloving
Weber-Fergusson Combined craniofacial approach

External Ethmoidectomy
Indications Removal of benign tumors of the ethmoidal region Approach to biopsy and drainage for tumors of sphenoethmoidal region and medial orbit Bony Excision - medial orbital wall and the ethmoidal labyrinth Surgical Approach - incision on the lateral wall of the nose Benefits allows excellent cosmesis and preservation of functional tissue Limitations For limited tumors (middle turbinate) Tendency to form a fistula to nasal cavity on irradiation

Inferior Medial Maxillectomy

Indications
Resection of medial wall of the antrum and inferior turbinate Most often used for inverted papilloma

Bony Excision margins

Laterally - vertical line dropped from the infraorbital foramen Inferiorly - floor of the nose Superiorly - lacrimal fossa and the middle meatus Posteriorly - dorsal end of the inferior turbinate Surgical Approach - Lateral rhinotomy Benefits Adequate exposure and resection for limited tumors Preserve functional tissue Provide a very acceptable cosmetic result Limitations - provides en bloc removal of limited area

Indication - larger benign or intermediate tumors involving the entire lateral nasal wall but without extension to the orbit, anterior cranial fossa, lateral maxilla, or alveolus Bony Excision - lateral nasal wall, including all turbinate tissue, and the contents of the ethmoid and maxillary sinuses Surgical Approach - Weber Fergusson with Lynch extension and lip split Bony cuts Removal of ant maxillary wall medial to infra orbital foramen Orbital cut from inferior rim carried medially to lamina papyracea Nasomaxillary suture line cut extending from cut 2 into pyriform aperture Cut in lateral nasal wall near floor upto post wall of maxillary sinus Vertical cut from post. nasal floor to post end of sup turbinate & post ethmoidal cells Benefit - en bloc resection with little cosmetic deformity Limitations - Removal of all turbinate tissue results in an abnormal nasal cavity, often requiring chronic management of crusting

Medial Maxillectomy

Medial maxillectomy

Total Maxillectomy
Indications - advanced carcinoma of maxilla Bony Excision - removal of maxilla along with nasal bone,

the ethmoid sinus, and in some instances, the pterygoid plates Surgical Approach - Weber Fergusson with a Defenbach (subciliary) extension Bony cuts Zygomatico maxillary suture line Orbital floor & medial orbital wall Naso maxillary suture line Hard palate Pterygoid process

 Can be combined with orbital exentration Preformed obturator - support for packing Benefits - When supplemented by irradiation, cure

rate 30% Limitations - Even when orbital exenteration is included - inadequate resection if ethmoidal roof, orbital apex or pterygoid region involved Therefore, careful evaluation & planning required before surgery

Craniofacial Frontoethmoidectomy
Indications - en bloc resection for tumors of the

ethmoidal and frontal regions Bony Excision Anterior cranium (including the frontal sinus) Floor of anterior cranial fossa Ethmoid +/- Eye Nasal septum

Benefits Provides direct visualization of the cribriform plate and fovea ethmoidalis Potential for en bloc removal Provides wide exposure to allow effective repair of dural tears Allows intraoperative irradiation or placement of a radioactive implant Limitations - If tumor extends to sphenoid sinus, cavernous or transdurally, en bloc resection cannot be achieved

Extended Craniofacial Resection

Indications - Extensive tumors involving the anterior skull base including pterygoid plates Bony Margins Posterior limits Foramen ovale Foramen rotundum ICA Remaining margins - as for craniofacial frontoethmoidectomy and total maxillectomy Surgical Approach Bicoronal and anterior or lateral facial incisions Closure - split-galea flap to cover dura Benefits - Thorough exposure and complete excision of otherwise unresectable tumors Contrindications - clear-cut pterygoid plate erosion and cranial nerve invasion.

Supplemental Management in Extended Craniofacial Resection

Intraoperative iodine seed implantation

Adenoid cystic carcinoma - more beneficial Undifferentiated carcinoma and squamous cell carcinoma - less optimistic Reconstruction Radial forearm Rectus abdominis musculocutaneous flaps Latissimus dorsi flap

Radiotherapy
Conventional

66-70 Gy (2.0 Gy/fraction; daily Monday-Friday) in 7 weeks Neck - Uninvolved nodal stations: 44-64 Gy (1.6-2.0 Gy/fraction) Concurrent chemoradiotherapy Primary and gross adenopathy: 70 Gy (2.0 Gy/fraction) Neck - Univolved nodal stations: 44-64-Gy (1.6-2.0 Gy/fraction) Postoperative RT Primary 60 66 Gy (2.0 Gy / fraction

 Neck

Involved nodal stations: 60-66 Gy (2.0 Gy/fraction) Uninvolved nodal station: 44-64 Gy (1.6-2.0 Gy/fraction) Preferred interval between resection and postoperative RT is <= 6 weeks

Chemotherapy

Primary Systemic Therapy / Concurrent RT

Cisplatin alone (preferred) (category 1) Cetuximab (category 1) Carboplatin/paclitaxel (category 2B) 5-FU/hydroxyurea Cisplatin/paclitaxel Cisplatin/infusional 5-FU Carboplatin/infusional 5 FU Carboplatin / paclitaxel

Postoperative chemoradiation
Concurrent single agent cisplatin 100 mg/m2 every 3 wks x 3 doses

Palliative therapy
Factors important for considering palliation Patient's symptoms and impact on life Extent of disease Distant metastases +/ Informed consent Some tumours have a long natural history Often possible to achieve significant periods of good quality survival With this in mind, some surgeons advocate local debulking of tumour with adjunctive radiotherapy as palliative treatment

Prognosis
Factors responsible for poor prognosis advanced stage of the disease at the time of diagnosis complexity of the anatomy reluctance in management PROGNOSIS 50 % at 5 yrs 31 % at 10 yrs 21 % at 15 yrs