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G. Nayak et al.
6.2.1.2 Histopathology
It is circumscribed, non-capsulated mucosa covered lobular mass lesion. Colour depends on the
vascular component of the tumour (pinkish- white
to red). Microscopically, it is formed by brocellular stoma with spindle cells and haphazardly
arranged dense collagen matrix with the irregular
vascular pattern. The vessels are dilated and lacks
true muscle layer [42].
Differential Diagnosis Angiomatous polyp,
infected antrochoanal polyp, benign (hemangioma, paraganglioma) or malignant tumour
(hemangiopericytoma) are differential tumours
for angiobroma. The demographic features,
clinical features, and pattern of spread helps in
differentiating these tumours from JNA [35, 43].
6.2.1.3 Treatment
Surgical treatment is the gold standard treatment.
The choice of approach can vary between centres
based on the surgeon's experience and hospital
setup. The majority of centres are shifted from
open surgical methods (lateral rhinotomy, Weber–
Fergusson, transpalatal, sublabial, midfacial
degloving, Le Fort 1 approach) to endoscopic
method because of improving anatomical knowledge, advancement in technology, and expertise
[44]. Endoscopic Denker’s, septal window, ante-
rior maxillotomy, and small posterior sublabial
incision allow easy dealing of ITF extension of
JNA by endoscopic approach (Fig.6.15). Posterior
septectomy increases space within the nasal cavity. Literature supports the use of endoscopic excision till stage IIIa of the Radkowski staging system
[44]. Tumour removal in toto or piecemeal showed
a similar recurrence pattern if the extension of
tumour is well assessed intraoperatively.
Open anterior (lateral rhinotomy, Weber
Ferguson or maxillary swing) and lateral approach
(subtemoral approach with or without craniotomy)
is indicated for large volume tumour with gross
skullbase involvement and when tumour is lateral to
ICA, signicant residual vascularity after embolization (equal to more than 50%) (Figs.6.16 and 6.17).
SS
JNA
IT
Fig. 6.15 Endoscopic picture is showing a bloodless
eld with coblator while separating JNA from the
surrounding
Fig. 6.16 Maxillary swing approach. (a) Weber–Fergusson’s incision is made, (b) plating for postoperative approxi-
mation, (c) raising of palatal ap

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171
a
c
Zygomatic arch
b
mandible
Temporal
fossa
angiofibroma
Temporalis muscle
Fig. 6.17 Preauricular subtemporal approach for right side. (a) JNA 3b, (b) ap elevation, (c) exposure of tumour
Excision of residual tumour lies in close prox-
imity with ICA, dura, optic nerve and cavernous
inexperienced surgeon, and early age of onset are
the major factors for tumour residual.
sinus is challenging as tumour handling can lead
to signicant morbidity [45]. Hypotensive anaesthesia, reverse Trendelenburg position, proper
decongestion, preoperative embolisation, and
coblation of peritumoral tissues are the measures
to reduce the amount of intraoperative bleeding
signicantly (Fig. 6.15). Large-volume tumour,
an extension of tumour into vidian canal and
inltration into the cancellous bone of skull base,
Medical Management Immunohistochemistry
shows the presence of oestrogen α, oestrogen β,
androgen, and vascular endothelial growth factor
(VEGF) receptors in tumour tissue. It is an oes-
trogen suppressive tumour and testosterone
responsive tumour. Diethylstilbestrol was ini-
tially used for shrinkage but it has a long list of
adverse effects. Flutamide therapy is a non-

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Fig. 6.18 Radiology is
showing a reduction of
the stage from
pretreatment II C to II A
of the Radkowski stage
G. Nayak et al.
Fig. 6.19 Clinical photograph is showing bilateral proptosis, with fullness in the right cheek. CECT is showing hyperintense mass with 360-degree involvement of the bilateral carotid artery
steroidal, anti-androgenic drug without feminising side effects and limited reversible adverse
effects. The dose is 10mg/kg/day for 6weeks.
The response rate ranged from 7 to 44% and it
was found more in post-puberty patients
(Fig.6.18). It is also effective in reducing episode
number and amount of blood loss in the preoperative period [46, 47].
Radiotherapy is indicated for unresectable
tumours (Fig. 6.19), in patients with persistent
residual lesion even after multiple surgeries and
when residual lies close to the vital structure
where surgery is very much challenging. The
dose ranges from 30 to 55Gy with tumour con-
trol rate ranging from 70 to 90%. The side effects
are cataract, panhypopituitarism, growth retardation, temporal lobe necrosis, and secondary
malignancy. Recent techniques such as IMRT
can reduce the risk of complication rate, but limited literature is available [48, 49].
Surveillance
The advised radiological investi-
gation is MRI and it can be performed after pack
removal or at 3 months postoperative period after
[46]. The surrounding inammation interferes in
the diagnosis of residual lesion. PMSA PET scan
is indicated when MRI is doubtful. When no
lesion is found in 5years’ follow-up period, then

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Fig. 6.20 Preoperative
CECT and postoperative
surveillance (3-months
interval) MRI scan for
residual detection
173
the patient can be labelled as disease-free
(Fig.6.20).
6.3 Part C: Cancer ofNose
andParanasal Sinuses
Cancers of the nose and PNS are very rare, and
account for 0.2–0.8% of all neoplasms. Overall,
they form <3% of all head and neck cancers. The
symptoms of nose and PNS tumours are similar
to those of common disorders such as chronic
rhinosinusitis and thus cause a delay in diagnosis.
The commonest age of presentation is in the fth
and sixth decade of life with a male predilection
(Male:Female:: 2:1) [50]. The most commonly
involved sinus is the maxillary (70%)>ethmoid
(20%) > frontal and sphenoid sinuses. A wide
variety of histologies may be encountered
although Squamous Cell Carcinoma (SCC) is the
most common malignant tumour. The TNM staging system is slightly complex and different for
different regions of the nose and paranasal
sinuses. A correct documentation of staging is
required for treatment planning, prognostication,
and information transfer.
6.3.1 Aetiology
Several carcinogenic compounds have been
identied with inhalation of these carcinogens
being responsible for ~40% of reported sinonasal malignancies. Exposure to industrial fumes,
nickel rening processes, alkaline battery manufacture industry, leather tanning, soldering,
welding has been reported to cause cancer of
paranasal sinuses.
Squamous cell carcinoma has been associated
with softwood dust, nickel, mustard gas, asbestos
industry workers. African mahogany appears to
be the most carcinogenic of all agents.
Adenocarcinoma, especially the interstitial variant, is mostly associated with hardwood dust in
the furniture industry, clothes and leather industry. Biologically active compounds in wood dust
impair mucociliary clearance and predispose to
carcinogenesis [
51].
Human Papilloma Virus (HPV) infection has
an increased risk of malignant transformation of
inverted papilloma. However, no signicant association between cigarette smoking and alcohol in
sinonasal cancers has been noted.
6.3.2 Patterns ofTumour Spread
The most common route of spread is by local
invasion. Orbit and cranium are seperate from
nose and paranasal sinuses by thin bone. The
paranasal sinuses other than the maxillary sinus
are in close proximity to orbit and cranial cavity
which makes them more vulnerable even at low
volume of tumour. Natural foreman allows early
spread of tumour.
Local Invasion Periosteum, perichondrium, and
dura appear to act as a momentary fence and

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oppose tumour growth to some degree [52, 53].
The bone of the anterior maxilla, medial wall and
oor of orbital, roof of the frontal sinus and olfactory cleft are very thin and readily destroyed by
tumour. The clinical presentation is based on the
area of involvement. Maxillary sinus tumours
generally present with medial spread or anterior/
inferior wall breach. Inferior orbital ssure and
thin orbital oor allows early orbital involvement
but periorbita is the relatively tough lining that
limits orbital tissue involvement to certain extent.
Posterior breach involves infratemporal fossa,
pteryomaxillary space and pterygoid plates
which is relatively rare [
54]. Öhngren described a
line running from the medial canthus of the orbit
to the angle of the mandible. Superiorly based
tumours are more aggressive and poorly differentiated, whereas inferiorly based tumours are more
amenable to treatment with a better prognosis.
Orbit is involved by the lateral and inferior spread
of ethmoid and frontal sinus tumours. Anterior
cranial fossa is involved by the superior spread of
tumours. Cavernous sinus is involved by lateral
spread, nasopharynx by inferior spread, nasal
cavity and ethmoid sinuses by anterior spread of
sphenoid sinus tumours.
Regional spread: Nodal disease is a marker of
•
locally advanced disease and lymphatic spread
to regional nodes is seen in around 25–35% of
patients. Submandibular and jugulodigastric
nodes are the most commonly involved lymph
nodes by anteriorly placed tumours, when
tumour breaches anterior bony wall, whereas,
posteriorly placed tumours involve retropharyngeal lymph nodes. Anterior spread has a
better prognosis than posterior spread.
•
Distant metastases: It is unusual at the time of
presentation and develops in less than 20% of
patients with adenocarcinomas, whereas, for
SCC, it is around 10% [55]. The most common sites for metastases are bone, brain, liver,
lung, and skin (Fig.6.21).
6.3.3 Clinical Features
G. Nayak et al.
Fig. 6.21 Tumour of left maxillary sinus involving ITF,
pterygoid plates, pterygoid muscles, and nasal cavity
common. The usual manifestations are unilateral
nasal obstruction, persistent sinusitis, recurrent
epistaxis, foul-smelling nasal discharge, facial
pain, etc. Epiphora is due to blockage of the
nasolacrimal duct (Fig.
6.22).
Oral symptoms are seen in 25–30% of patients.
These symptoms manifest in the form of loose
teeth, pain in maxillary teeth, and palatal fullness
(Fig. 6.23). Muscles of mastication, nerves are
involved by tumour extension into infratemporal
fossa or by pterygoid muscle inltration and
patients are present with symptoms of trismus.
Numbness of the cheek is the sign of inferior
orbital nerve inltration. Distortion of nose and
face is a late feature (Fig.
6.22). Pattern of eye-
ball displacement is helpful in pinpointing the
involved sinus. Auditory manifestations are in the
form of hearing loss, secondary to serous otitis
media due to nasopharyngeal extension. Cranial
nerve II, III, IV, V1, V2, and VI nerves are seen
after orbital apex and cavernous sinus involvement. Cranial nerve involvement is a manifestation of advanced disease; it indicates poor
prognosis.
The clinical presentation is depending on the site
of involvement. Nasal symptoms are the most
Diagnosis Clinical staging requires detailed
local examination with neck evaluation for cervi-

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cal lymphadenopathy, cranial nerve function, and
ophthalmological evaluation. Nasal endoscopy is
done with a diagnostic purpose to see the characteristic of mass and with a therapeutic purpose to
obtain tissue for biopsy (Fig.6.24). Contrast CT
scan of nose and paranasal sinuses 3–5mm axial,
coronal, and sagittal images are required to document staging of the tumour. Coronal images are
obtained for involvement of the orbital oor and
skull base and axial images are obtained for
extension through the posterior wall of the maxillary sinus into the pterygopalatine fossa and
infratemporal fossa (Fig. 6.25). It also helps to
evaluate the tumour involvement of the retroorbital and orbital apex region. Sometimes,
reconstructed images are required to pinpoint
bony erosion. However, the limitations of CT
scan are in the evaluation of periorbita, dural
involvement to see whether the tumour has
invaded or is just abutting these structures. It is
Fig. 6.22 Nasal mass (Right) with fullness of right nasal
cavity ulceration of skin in medial canthus and lateral/
inferior displacement of right eyeball (clinical stage is
T4). (Courtesy—Dr. Hitesh Verma, Associate Professor,
AIIMS, New Delhi, India)
Fig. 6.23 Palatal bulge
and ulceration with
loosening of teeth by
inferior spread of
maxillary sinus tumour
Fig. 6.24 Nasal endoscopy is revealing a mass in the left
nasal cavity (clinical stage T2)

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Fig. 6.25 CECT Nose and PNS showing the extent of a sinonasal tumour (T4a stage). MRI is showing right periorbital
inltration with retained section in right maxillary sinus (T4a stage)
G. Nayak et al.
difcult to differentiate tumour from soft tissues
and secretions owing to their similar densities.
MRI is prescribed in such cases as it provides us
better soft tissue details and it differentiates
tumour from secretion [54, 56]. Angiography and
embolisation have limited indications in highly
vascular malignant lesions, such as hemangiopericytoma. Histopathology is compulsory for
tissue diagnosis. Unusual presentation and abnormal recurrence are the indications for PET scan.
6.3.4 Histopathology
Squamous cell carcinoma (SCC) is the leading
histopathological diagnosis. Adenocarcinoma
and adenoid cystic carcinoma accounts for
around 10% of nose and PNS tumours. The
tumours have a male preponderance and are seen
mostly in sixth to seventh decades of life. Sinonasal tumour mostly arise from the lateral nasal
wall whereas tumours arise from paranasal
sinuses. SCC is well-differentiated in a majority
of cases (85%). Esthesioneuroblastoma originates from basal cells of the olfactory epithelium.
It accounts for less than 5% of sinonasal malignancies and it has bimodal age distribution (peak
at 20 and 50 of age). It has female preponderance
with the potential of inducing paraneoplastic syndrome by releasing peptides. Sinonasal undifferentiated carcinoma, melanoma, and
hemangiopericytomas are other common differential diagnoses for sinonasal tumours [
57, 58].
Staging it is devised for carcinomas of the max-
illary sinus, ethmoid sinus, and the nasal cavity
and is not applicable to mesodermal tumours,
olfactory neuroblastoma (OAN) for which other
grading systems have been devised. TNM
Classication (AJCC 2018) is proposed for nose
and paranasal sinuses lesions [
59].
6.3.5 Stage withDescription
6.3.5.1 T Staging
Maxillary Sinus
• T1 Tumour conned to the maxillary antrum
mucosa without bone erosion.
• T2 Tumour produces bone erosion or destruc-
tion, except the posterior wall of the antrum,
and it also includes tumour extension into the
hard palate and/or middle meatus.
• T3 Tumour inltrates in whichever of the sub-
sequent: maxillary sinus posterior wall, sub-

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cutaneous tissues over maxillary sinus,
ethmoid sinus, medial wall and oor of orbit,
pterygomaxillary space.
• T4a Tumour inltrates in whichever of the
subsequent: anterior orbit stufng, pterygoid
plates, cheek skin, cribriform plate, infratemporal fossa, sphenoid or frontal sinuses
(Fig.6.25).
• T4b Tumour inltrates in whichever of the
subsequent: apex of orbit, dura, brain, cranial
nerves except maxillary division of trigeminal
nerve, nasopharynx, or clivus.
Ethmoid Sinus
• T1 Tumour conned to the ethmoid sinuses
with or without bony erosion.
• T2 Tumour extends into the nasal cavity.
• T3 Tumour extends into anterior orbit and/or
maxillary sinus.
• T4a Tumour inltrates in whichever of the
subsequent: anterior orbital stufng, skin over
nose or cheek, negligible anterior intracranial
extension, pterygoid plates, sphenoid or frontal sinuses.
• T4b Tumour inltrates in whichever of the
subsequent: orbit apex of orbit, dura, brain,
cranial nerves except maxillary division of trigeminal nerve, nasopharynx, or clivus.
Nasal Cavity
• T1 Tumour involves only one subunit of the
subsequent: septum, oor, lateral wall, and
vestibule of the nasal cavity.
• T2 Tumour involves two subunits or ethmoid.
• T3 Tumour inltrates into the orbit anteriorly
and/or maxillary sinus.
• T4a Tumour invades any of the following:
anterior orbital stufng, skin over nose or
cheek, negligible anterior intracranial extension, pterygoid plates, sphenoid or frontal
sinuses.
• T4b Tumour inltrates in whichever of the
subsequent: apex of orbit, dura, brain, middle
cranial fossa, cranial nerves except maxillary
division of trigeminal nerve, nasopharynx or
clivus.
N Staging
•
NX Local lymph nodes cannot be assessed.
• N0 No local lymph node metastasis.
• N1 Metastasis in a solitary ipsilateral lymph
node, 3cm or less in maximum measurement
exclusive of extranodal extension.
• N2
• N2a—Metastasis in a solitary ipsilateral
lymph node, more than 3 cm but not more
than 6cm in maximum measurement exclusive of extranodal extension.
• N2b—Metastasis in several ipsilateral lymph
nodes, none more than 6 cm in maximum
measurement exclusive of extranodal
extension.
• N2c—Metastasis in bilateral or contralateral
lymph nodes, none more than 6cm in greatest
dimension, without extranodal extension.
• N3a Metastasis in a lymph node more than
6cm in greatest dimension without extranodal
extension.
• N3b Metastases in a solitary or several lymph
nodes with clinical extranodal extension.
The staging template is mentioned in Table6.5.
The staging system for olfactory neuroblas-
toma is rst proposed by Kadish and then it was
modied by Morita [60]:
Stage 1: Tumour conned to nasal cavity
Stage 2: Tumour conned to the nasal cavity
and paranasal sinuses
Stage 3: Extension of tumour away from nose
and paranasal sinuses (intra-orbit or intracranial)
Stage 4: Any previous stage with cervical
lymph node or distal metastasis
Table 6.5 Staging template
Stage
O Tis N0 M0
I T1 N0 M0
II T2 N0 M0
III T1/2 N1 M0
T3 N0/1 M0
IVa T1/2/3 N2 M0
T4a N0/1/2 M0
IVb T4b Any N M0
Any T N3 M0
IVc Any T Any N M1

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G. Nayak et al.
Dulgurov proposed a new staging system for
esthesioneuroblastoma [61]:
• T1 is conned to the nose and PNS without
superior ethmoid cells and sphenoid sinus
involvement.
• T2 is T1+sphenoid sinus with or without erosion of cribriform plate.
• T3 is any stage with intraorbital and intracranial extension without dural inltration.
• T4 is brain parenchyma involvement.
The presence of regional and distal metastasis
is marked as N1 and M1. Dural involvement is
noticed in 25% and lymph node metastasis in 5%
of cases. Hyems etal. proposed a histopathological grading system on the basis of tumour cell
architecture, degree of differentiation, nuclear
pleomorphism, mitotic index, tumour necrosis,
and nature of the matrix. They graded histology
into four subtypes where type 1 is least aggressive and type 4 is most aggressive [62].
Surgical and other management for sinonasal
malignancies is mentioned in Chaps. 7 and 10.
6.4 Part D: Nasopharyngeal
Carcinoma
6.4.1 Summary
Nasopharyngeal carcinoma (NPC) is the most
common neoplasm arising in the nasopharynx
which shows remarkable geographical difference
in incidence across the world. NPC is uncommon
in the United State but is endemic in many areas
in the world, particularly southeastern China and
Hong Kong where the incidence is as high as
20–30 cases per 100,000 population. The main
factors believed to be associated with the development of NPC are EBV, genetic, and environmental; however, we are still unable to assimilate
these information to translate knowledge into
prevention, early detection, and improved survival outcome. The most common clinical presentation is palpable cervical lymphadenopathy
(50%) followed by blood-stained nasal discharge
(41%), deafness (30%), and cranial nerve palsy
in about 10% of cases. Nasopharyngosocopic
examination reveals growth and provides a suitable area for biopsy under direct visualisation.
The use of immunohistochemical markers will
help to differentiate NPC from other malignancies in the nasopharynx.
Computed tomography has been the cornerstone of radiological staging for many years;
however, magnetic resonance imaging has been
used increasingly because of its superior denition in detecting soft tissue changes and intracranial involvement. Bone scan and positron
emission tomography are other reliable investigations to look for distant metastasis.
In recent years, treatment of NPC had undergone several paradigm shifts resulting in improved
prognosis. Advances in the planning and delivery
of radiation treatment and the addition of chemotherapy have been demonstrated to improve disease control rates in locally advanced NPC. In
general, patients with stage 1 are treated by radiation therapy alone, whereas, stage II, III, and IV
are treated by concurrent radiotherapy and chemotherapy. Surgery should be considered as a
rst-line treatment of residual or recurrence disease at the primary site (rT1 and limited rT2).
Technical advances have enabled surgeons to perform endoscopic or robotic surgeries as salvage,
reducing the morbidity from traditional open
resections. Patients with NPC cases are followed
up and assessed with ofce-based rigid and/or
bro-optic nasal endoscopy. Positron emission
tomography–computed tomography, CT, or MRI
scan should be carried out at 3 months from completion of treatment to assess response.
6.4.2 Anatomy ofNasopharynx
It is also known as postnasal space, located in the
centre of the head behind the nasal cavity and
above the oropharynx. It is a region where thorough clinical examination as well as adequate
exposure for surgical resection is difcult.
Anatomically, the roof is formed by the body of
sphenoid bone, sphenoid further slants downwards
in front of arch of atlas and body of axis to form
the posterior wall. The oor is formed by the upper

ossa of Rosenmuller
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surface of the soft palate. The lateral wall is formed
by the eustachian tube opening in the upper part
and superior constrictor muscle in the lower part.
Fossa of Rosenmuller, also called lateral pharyngeal recess, is an important structure lying in the
lateral wall, which is bounded anteriorly by torus
tubarius and levator palatine muscle, posteriorly
by the posterior wall of the nasopharynx and retropharyngeal space, laterally by parapharyngeal
space, and inferiorly by the upper edge of superior
constrictor muscle (Fig. 6.26a, b). Its superior
boundary is the skull base with important openings including foramen spinosum, foramen ovale,
and carotid canal. The internal carotid artery is
Nasopharynx
on axial
section
separated from the apex of the lateral recess by
only a thin layer of broconnective tissue.
Blood supply of the nasopharynx is from
branches of the internal maxillary artery, ascending pharyngeal artery and venous drainage is to
the pterygoid plexus. Sensory supply is from
branches of the maxillary nerve and lymphatic
supply drains into the retropharyngeal and cervical lymph nodes.
Tumours of Nasopharynx A broad range of
neoplasms arise in the nasopharynx from epithelial to mesenchymal, lymphoid, and neuroectodermal cells (Table6.6) [63].
F
Torus tubaris
Eustachian tube
Fig. 6.26. Axial view of nasopharynx and the endoscopic view of nasopharynx from left choana
Table 6.6 (Pathology fourth edition WHO classication)
Carcinomas Nasopharyngeal
Salivary
gland
tumours
Benign and
borderline
lesions
carcinoma
Nasopharyngeal
papillary
adenocarcinoma
Adenoid cystic
carcinoma
Salivary gland anlage
tumours
Hairy polyp
Ectopic pituitary
adenoma
Craniopharyngioma
Nonkeratinising
SCC
Keratinising
SCC
Basaloid
SCC
8072/3 Soft tissue
8071/3 Haemato-
8083/3 Extra-osseous
8260/3 Extramedullary
8200/3 Notochordal
8272/0
9350/1
tumours
lymphoid
tumours
tumours
Nasopharyngeal
angiobroma
Diffuse B cell
lymphoma
plasmacytoma
myeloid sarcoma
Chordoma 9370/3
9160/0
9680/3
9734/3
9930/3
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