This document discusses carcinoma of the maxilla and sinonasal malignancies. It covers the anatomy of the maxilla bone, signs and symptoms of sinonasal cancers, risk factors like wood dust exposure, staging criteria, treatment options including surgery and combinations of surgery and radiation/chemotherapy, and surgical approaches like lateral rhinotomy and midfacial degloving. It also describes craniofacial resection techniques for tumors invading the skull base.

1. Dr R Grace
Postgraduate MS ENT
NMCH, Nellore

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• Maxilla is the second largest bone of the face,
between the orbit and the upper teeth, form the
upper jaw.
• Superiorly - inferior and medial rims of the orbit.
• Laterally, the zygomatic process of each maxilla
articulates with the zygomatic bone
• Medially - the frontal process of each maxilla
articulates with the frontal bone.
• Inferiorly, each maxilla ends as the alveolar
process, which contains the teeth and forms the
upper jaw.

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• Sinonasal malignancies spread most commonly by local invasion.
• The ethmoid and maxillary sinuses are intimately related to the orbit and separated from it
by thin bone that is deficient in places to permit the passage of nerves and blood vessels.
• These anatomical features favour relatively early tumour spread into the orbital contents.
• The roof of the frontal sinus is similarly thin and this, in combination with perforations in
the superior part of the nasal cavity through which the olfactory nerves pass, means that
tumours developing at these sites tend to spread intracranially

9. Anterior
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Two pathways
Posterior
• The lymphatics of the
anteroinferior part of the nasal
cavity and skin of the nasal
vestibule drain via the anterior
pathway to the first eschelon
facial, parotid and
submandibular lymph nodes.
• These in turn drain into the upper
deep cervical chain.
• The remainder of the nose and
paranasal sinuses drain through a
posterior pathway which runs
anterior to the Eustachian tube to
first eschelon nodes in the
retropharyngeal space and then
onwards to the upper deep cervical
chain.

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• BENIGN
• Inverted papilloma
• Osteoma ( Gardner’s syndrome )
• Chondroma
• Fibrous dysplasia
• Hemangioma
• Leiomyoma
• Schwannoma
• MALIGNANT – Sinonasal malignancies
• Squamous cell carcinoma (80%)
• Adeno carcinoma (5%)
• Adenoid cystic carcinoma (5%)
• Hemangiopericytoma
• Melanoma
• Undifferentiated carcinoma
• Malignant fibrous dysplasia
• Dental origin : Ameloblastoma
• Sarcoma; Rhabdomyosarcoma
Fibrosarcoma Angiosarcoma
Chondro/osteo sarcoma
• Others; lymphoma

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Introduction
• Carcinoma of maxilla include sinonasal malignancies, which are uncommon, accounting for
less than 1% of all neoplasms.
• Initially they produce little in the way of symptoms and in the early stages can be misdiagnosed
as more common benign conditions such as rhinosinusitis.
• As a result, the diagnosis is often delayed and only made at a relatively advanced stage when
symptoms caused by local disease extension supervene.

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• Incidence: 0.5–1 per 100 000 per year.
• They account for 0.2–0.8% of all malignancies and 3% of upper
aerodigestive tract neoplasms.
• Age of presentation: fifth and sixth decades of life.
• Gender distribution: Men is twice that of women.

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• Inhalation carcinogens is responsible for around 40% of reported sinonasal malignancies.
• Foremost among these occupational hazards is exposure to hard woods in the
furniture industry. Biologically
active
compounds
in wood
dust
Impair
mucociliary
clearance
Predispose
to
carcinogene
sis

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• The type of wood is a significant factor.
• Hardwood exposure – adenocarcinoma
• Soft wood exposure - squamous cell carcinoma (SCC).
• African mahogany - most carcinogenic.
• This wood is often burned by bantu tribesmen, who have the highest incidence of
maxillary sinus cancer in the world.
• Sinonasal adenocarcinoma that develops in wood-workers has a better prognosis than
other nasal adenocarcinomas.

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• Other occupational hazards
• Exposure to nickel, which increases risk of developing sinonasal SCC 250-fold.
• Chromium, polycyclic hydrocarbons,
• Aflatoxin (found in certain foods and dust),
• Mustard gas and thorotrast (thorium dioxide used in paints for watch dials).
• Radiation, viral and genetic causes have also been proposed.
• There is no evidence that chronic sinusitis predisposes to cancer

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• The advanced stage of tumours at the time of presentation can make their precise origin
difficult to determine.
• Maxillary sinus tumours (55%) > nasal cavity (35%) > ethmoid sinuses (9%) > frontal and
sphenoid sinuses (1%).

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• Regional spread
• Lymphatic spread to regional nodes in 25–35% of patients
• Around 10% of patients present with nodal disease, which is usually a marker of locally-
advanced disease.
• The submandibular and jugulodigastric nodes are the most commonly involved.
• Bilateral lymph node involvement can occur in patients who develop tumours near the
midline.
• Distant metastases
• ACC (20%) > SCC (10%)
• The most common sites for metastases are bone, brain, liver, lung and skin.

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T1
• Limited to
mucosa
• No bony
erosion
T2
• Involves
medial wall
• Hard palate
• No posterior
wall
involvement

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T3
• Involves
lateral wall
• Ethmoid
sinus
• Floor of
medial wall of
orbit
T3
• Involves
anterior wall
• Posterior wall

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T4a
• Orbital contents
• Sphenoid sinus sinus
• Infratemporal and
pterygopalatine fossae
• cheek

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T4b
• Orbital apex
• Middle cranial fossa
• Dura and brain
• Cranial nerves other than
V2 of Trigeminal nerve
• Nasopharynx
• Clivus

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T1
• Limited to
ethmoidal
sinus
• With or
without bony
erosion
T2
• Extends into
nasal cavity

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T3
• Involves
anterior orbit
• Maxillary
sinus
T4a
• Invades
sphenoid and
frontal sinuses
• Anterior orbital
contents
• Minimal
intracranial
extension

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T4b
• Orbital apex
• Middle cranial fossa
• Dura and brain
• Cranial nerves other than
V2 of Trigeminal nerve
• Nasopharynx
• Clivus

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• Squamous cell carcinoma
• Adenocarcinoma
• Adenoid cystic carcinoma
• Olfactory neuroblastoma (aesthesioneuroblastoma)
• Sinonasal undifferentiated carcinoma
• Melanoma
• Hemangiopericytomas

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• Most common
• Seventh decade
• Arise from lateral wall of nasal cavity with 50% developing on turbinates
• Septal SCC’s anteriorly at mucocutaneous junction
• Transformation from inverted papilloma
• Well differentiated tumours
• Macroscopically polypoid appearance
• Poor overall outcome

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• 9% of all sinonasal malignancies
• Male predilection in sixth and seventh decades
• Found in upper nasal cavity and ethmoidal sinuses
• Histological subtypes
• Papillary- least aggressive
• Sessile, mucoid – worst prognosis
• Neuroendocrine
• Intestinal – wood workers
• Undifferentiated

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• 5% of all sinonasal malignancies
• Slow growth
• Early perineural and vascular spread
• Maxillary sinus mostly affected with history of undiagnosed chronic facial pain

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• Aesthesioneuroblastoma
• Arises from basal cells in olfactory epithelium
• < 5% of all sinonasal malignancies
• Bimodal distribution peaks at 20 and 50 years of life
• More common in women
• Neuroendocrine tumour causing paraneoplastic syndromes by secreting peptides
• Causing cushings syndrome, diabetes insipidus and hypertension
• Expresses neuroendocrine markers – neuron specific enolase, synaptophysin and
chromogranin.
• Differential diagnoses
• OAN belong to small round cell tumours
• S-100 differentiates it from sinonasal melanoma
• Negativity for vimetin, actin and desmin excludes it from rhabdomyosarcoma

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• OAN can often be predicted
from imaging characteristics,
based on its location focused
on the cribriform plate

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Sinonasal undifferentiated carcinoma
• Anaplastic carcinoma
• Highly aggressive and invasive
• Negative for EBV differentiating it from NPC.
Melanoma
• Women > men
• Nasal cavity and septum are common sites
• Polypoid mass or ulcerated lesion with or without pigmentation
• IHC positivity for S100 and HMB-45
Hemiangiapericytomas
• Develop from pericytes within capillary wall
• Radioresistant red-grey, firm polypoid masses
• Associated with steroid therapy, coincidental trauma hypertension and pregnancy
• 10-60% recurrence rate

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• Progressive unilateral nasal obstruction
• Bleeding
• Maxillary tumours
• Facial pain hypoaesthesia of skin on cheek in infraorbital nerve involvement
• Erosion of medial wall – epistaxis and epiphora
• posterior wall- trismus, maxillary and mandibular nerve deficits
• inferior wall- oral mass and loose teeth
• superior wall – proptosis and diplopia
• anterolateral wall- cheek swelling
•

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• Ethmoid sinus - Orbital symptoms
• Sphenoid sinus – cavernous sinus thrombosis and cranial nerve involvement

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• Endoscopy – ulcerative growth or polypoidal mass
• Imaging – CT, MRI, PET-CT
• CT : Site & extent Bone, skull base erosion
• Orbit invasion
• Neck- nodal status
• MRI - Better soft tissue delineation
• Differentiate between secretions, tumor
• Dural / intracranial involvement
• Vascularity- flow voids
• MRA- great vessel encasement, cavernous extension
• BIOPSY
• A Caldwell–Luc approach should be avoided if it is felt this would seed tumour or potentially
compromise a subsequent resection

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• General considerations
• Many patients still present with advanced disease and there will inevitably be some who
are incurable from the outset.
• Elderly patients concurrent general medical conditions might preclude any major
intervention.
• With a palatal resection the defect must be sealed with either an obturator fitted with teeth
to restore both speech and normal deglutition or by composite free flap and microvascular
techniques.
• Orbital resections leave an obvious cosmetic deformity but titanium implants have
revolutionized the construction and retention of facial prostheses.

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Palliative and curative surgery
• Patients with advanced malignant melanoma, for instance, may have a significant
reduction in local symptoms with debulking surgery and post-operative radiotherapy.
• While treatment is likely to be only palliative in patients with large adenoid cystic tumors
that have a long natural history, it is often possible to achieve significant periods of good
quality survival with local debulking of tumor and adjunctive radiotherapy.
• Patients who present early with T1 maxillary tumors may be cured with surgery alone but
the majority of patients will require combination therapy.

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• Combination of radiotherapy and surgery with the addition of chemotherapy in
selected cases.
• Intensity modulated radiotherapy techniques are generally adopted in order to
achieve tumour coverage whilst meeting organ at risk constraints.
• Post-operative radiotherapy should start within 6 weeks of surgery with 60–65
Gy delivered over 30 fractions.
• Local invasion of the anterior cranial fossa and skull base are not necessarily
contraindications given the development of modern surgical techniques.
• Distant metastases confer a poor prognosis and by definition render such patients
incurable. Involvement of the facial skin is likewise not a contraindication to
treatment and in practice many such patients do well.
• The involved area is best excised and repaired with either a rotation flap or free
flap.
• Concerns that radiotherapy interferes with healing are less with modern
radiotherapeutic regimens

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• OAN is treated with a combination of surgery and postoperative
radiotherapy. Chemotherapy has been used in more advanced cases.
• Multimodality therapy (surgery, radiotherapy and chemotherapy) has
generally been demonstrated to be the most effective approach in the
treatment of SCC and SNUC.
• In large volume tumours initial non-surgical treatment with
(chemo)radiotherapy or chemotherapy alone followed by
chemoradiotherapy appears to give better results.
• There is no role for chemotherapy in the initial treatment of mucosal
melanoma, the mainstay of which is surgical resection followed by
radiotherapy.

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• Choice of which is determined by the extent of the tumour and amount of bone that needs to
be removed.
• Partial maxillectomy: partial removal of the upper jaw skeleton.
• Two variants are in common use:
• Medial maxillectomy, which involves clearance of the lateral wall of the nose including the
ethmoid sinuses
• Palatal resection along with the adjacent alveolus, which is used for tumours of the oral cavity
that involve the hard palate.
• Total maxillectomy : total removal of the upper jaw, preferably as a bony box containing the tumour.
• Extended maxillectomy : required when the tumour extends beyond the upper jaw. If this involves
the skull base the term craniofacial resection is used

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• LATERAL RHINOTOMY
• Excellent exposure to nasal cavity and
medial maxillary wall
• Indication - medial maxillectomy
• Cosmetically acceptable
• To facilitate the various bony resections it is necessary to use the appropriate soft tissue
access procedure, of which three are commonly described:

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• Lateral rhinotomy incision with horizontal
infraorbital component and midline lip split
• Sublabial incision is performed after splitting
upper lip in midline
• This facilitates elevation of flap from anterior
wall of maxilla
• Extends through entire bucco gingival sulcus
up to maxillary tuberosity
• Infraorbital component
• This is the horizontal component of weber
Ferguson’s incision
• Made about 1 mm below the infraorbital rim

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• MIDFACIAL DEGLOVING
APPROACH
• 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, pterygopalatine
fossa.
• Combined with Le Fort 1 osteotomy –
wide exposure to clivus, skull base

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• Broad spectrum antibiotic cover
• Dental evaluation - prosthesis, obturator
• Neurosurgeon review - intra cranial extension, Craniofacial resection
• Plastic surgeon - pre op decision for flaps

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• Skilled anaesthesia is essential.
• Topical anaesthesia of the nasal mucosa with a decongestant solution
• Hypotensive general anaesthesia are of considerable benefit to the
surgeon.
• An oral tube (or tracheostomy) is required for a midfacial degloving.

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• Marking incision site
• Reflection of skin flap over maxilla
• Bone cuts
• Disarticulation of maxilla
• The facial skin flap is raised in a
submuscular plane and all the soft tissue
incisions are gently dissected free of the
bone to allow the subsequent osteotomies
• The body of the zygoma, midline of the
palate and pterygoid plates need to be
divided.

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• Involvement of the cribriform plate region
was recognized as one of the major
reasons for failure to control ethmoid
neoplasms with some of the above
approaches.
• Type 1: craniofacial
(transorbital) resection.
• This procedure is essentially an extended
medial maxillectomy using a lateral
rhinotomy incision.

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• Type 2: craniofacial (window
craniotomy) resection.
• a lateral rhinotomy approach is used for
anterior access and a bicoronal incision
utilized to provide a pericranial flap and
access to the frontal bone.
• A small midline ‘window’ craniotomy
usually utilizing the frontal sinus is made
giving access to the floor of the anterior
cranial fossa.

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• Type 3: craniofacial resection.
• This operation is performed in conjunction with a neurosurgeon and combines a
transfacial approach with a neurosurgical approach, such as a frontolateral craniotomy, to
allow the resection of extensive tumours

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• Careful rehabilitation, be it biological or prosthetic, aims to
ensure a good cosmetic and functional outcome with
separation of the nasal and oral cavities.
• Low defects not involving the orbital adnexae can often be
successfully treated with dental obturators.
• For the more extensive maxillary defects there is
consensus that a free flap is required.
• Composite flaps of bone and muscle harvested from the
iliac crest with internal oblique or the scapula tip with
latissimus dorsi can more reliably support the orbit and
cheek than soft-tissue free flaps and non-vascularized
grafts, and also enable an implant-borne dental or orbital
prosthesis.

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• Indications :
• Type 1 craniofacial resections
• Limited tumours
• Contraindications:
• Involvement of the nasal bones, frontal
sinus, dura lateral to the ethmoidal roof
and orbital contents are contraindications
to endoscopic approaches although these
may also be combined with transcranial
approaches or orbital exenteration where
appropriate.

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• Sinonasal malignancies are uncommon, tend to present late and majority of them are caused
by inhaled carcinogens.
• The main treatment modalities are surgery and radiotherapy.
• Chemoradiotherapy followed by surgery is advocated for snuc and advanced ethmoidal scc.
• Contraindications to aggressive treatment are spread to the sphenoid sinus, middle cranial
fossa, optic chiasma, cavernous sinus or prevertebral fascia, as well as the presence of distant
intracranial or systemic metastases.
• The prognosis of sinonasal malignancies has improved over the past three decades, but
remains poor overall, and is directly related to the degree of local control.
• Olfactory neuroblastoma and adenoid cystic carcinoma may relapse after a decade and thus
patients should be followed up for at least 15 years