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G. Nayak et al.
Fig. 6.2 NCCT PNS orbit (coronal cut) is showing
homogenous mass lling both side frontal, ethmoid
sinuses with focal hyperostosis (black arrow) in the region
of the right frontal outow tract. The right maxillary sinus
is also lled up by secretions as the ostium is not wide and
drilling of the thick bone of the lateral wall of the
pyriform aperture from the anteromedial part of
the maxillary sinus. This signicantly improves
the surgical accessibility to the posterolateral
wall and infratemporal fossa. The commonly
reported complications are bleeding, infraorbital
hypoesthesia, epiphora, orbital fat exposure, alar
collapse, etc.
Radiation therapy as adjuvant therapy is used
for local recurrences at some centres, but this
remains controversial [
17]. However, regular fol-
low- up and subsequent surgical management
remains the treatment option for the vast majority
of recurrent cases.
6.1.5 Schneiderian Papilloma
(Oncocytic Type)
These types of papilloma are a variant of
Schneiderian papilloma where the columnar cell
lining have oncocytic features. The high content
of cytochrome C oxidase and mitochondria establishes this oncocytic appearance. Other synonyms
are cylindrical cell papilloma, columnar cell papilloma [1]. In contrast to inverted papilloma, there
air–uid level is visible within the sinus. MRI Nose and
PNS (axial cut) are showing characteristic “cerebriform”
pattern (black arrow) in nasal cavity and ethmoid sinuses.
(Courtesy—Dr. Hitesh Verma, Associate Professor,
AIIMS, New Delhi, India)
is equal distribution among the sexes and HPV
has not been isolated from these variants.
6.1.6 Schneiderian Papilloma
(Exophytic Type)
These are the papillomas arising from the
Schneiderian membrane with rich papillary
fronds and brovascular cores covered by layers
of epithelium [
1]. Other synonyms are transi-
tional cell papilloma, fungiform papilloma, septal papilloma. These tumours are more commonly
seen in men (2–10 times) between 20–50years of
age. In contrast to inverted papilloma, these variants are localised more on the lower anterior
nasal septum with no signicant lateralisation.
They are infrequent on the lateral nasal wall.
Malignant change in exophytic papilloma is rare.
6.1.7 Salivary Gland Adenoma
Only 25% of glandular tumours are benign in the
nose and paranasal sinus tract. The common
veining tumours are pleomorphic adenoma, myo-
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epithelioma, and oncocytoma. Pleomorphic adenoma presents with non-specic signs and
symptoms like nasal obstruction, discharge, and
occasional bleeding. These are more common
between 20 and 50 years of age. Most of the
cases arise from the submucosa of the bony and
cartilaginous part of the nasal septum. Treatment
is surgical with wide local excision.
6.1.8 Benign Tumours ofBony
andCartilaginous Origin
6.1.8.1 Osteoma
Osteomas are benign, slow-growing osteoblastic
lesions and the most common type of benign
sinonasal tumours [18]. They are incidental ndings on computed tomography of patients with
sinus symptoms. These tumours are mostly
asymptomatic and occasionally present with
symptoms of headache, nasal discharge, epiphora, forehead swelling, ocular pain, etc. The most
common location of osteoma is in the frontal
sinus followed by ethmoid, maxillary, and more
rarely the sphenoid sinus (Fig. 6.3). However,
rarely, the turbinates can be involved causing
obstruction to frontal recess, nasolacrimal duct
and can also lead to mucocele formation.
Osteomas have slight male preponderance and
are usually diagnosed between the second and
fth decades.
These tumours are histologically divided into
three categories as shown in Table 6.2 [19].
Osteomas can also be associated with Gardner
syndrome, a form of familial adenomatous polyposis with epidermoid cysts, lipomas, and desmoid tumours [20]. High-resolution computed
tomography of paranasal sinus and orbit assesses
the site of attachment and gauges the extent of the
lesion. Differential diagnosis of nasal osteoma
includes other broosseous lesions like brous
dysplasia and ossifying broma.
Table 6.2 Histological types of osteoma
Types of
osteoma Histology
Ivory
osteoma
Mature
osteoma
Mixed
osteoma
Lobulated, made of compact dense bone
containing a minimal amount of brous
tissue
Spongy, mature bone with conspicuous
brous tissue
Findings of both ivory and mature
osteoma
Fig. 6.3 Water’s view is showing radio-opaque mass in
the region of the right ethmoid and frontal sinus oor.
NCCT PNS orbit (Coronal cut) is showing ivory osteoma
of the right anterior ethmoid region. (Courtesy—Dr.
Hitesh Verma, Associate Professor, AIIMS, New Delhi,
India)
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G. Nayak et al.
Treatment of osteoma depends upon the signs
and symptoms. These tumours are slow-growing
in nature and there is a general consensus in
favour of a wait-and-watch policy provided there
are no symptoms and the lesion does not encroach
critical structures like optic nerve, orbit, frontal
mucocele, facial deformity. Local resection by
endoscopic or external approach is the treatment
of choice.
6.1.8.2 Chondroma
Chondromas are extremely rare and any cartilaginous tumour more than 2cm in this site should be
considered potentially malignant unless proven
otherwise.
6.1.9 Fibroosseus Lesion
Fibroosseous lesions comprise a diverse group of
conditions of cranio facial skeleton which
includes developmental lesions, reactive or dysplastic lesions, and neoplasms. These lesions are
characterised by the replacement of normal bone
by variable broblastic connective tissue matrix
[21]. The WHO and Eversole classication of
broosseous lesions is shown in Table 6.3 and
Table6.4, respectively [1, 22].
6.1.9.1 Fibrous Dysplasia
Fibrous dysplasia (FD) is a benign dysplastic
skeletal lesion where the normal bone is replaced
by disorganised and immature bony and brous
tissue. FD may affect a single bone (monostotic)
or more than one bones (polyostotic). Monostotic
FD is more common in the craniofacial skeleton
with the maxilla affected more commonly than
the mandible. Polyostotic FD may be associated
Table 6.3 WHO classication of broosseous lesions
Fibroosseous conditions Subtypes
Ossifying broma
Fibrous dysplasia
Osseous dysplasia (a) Periodical osseous
dysplasia, focal osseous
dysplasia, orid osseous
dysplasia, familial
gigantiform cementoma
Central giant cell
granuloma
Cherubism
Aneurysmal bone cyst
Solitary bone cyst
Table 6.4 Eversole classication of broosseous lesions
Fibrosooesus pathology Disease Subtypes
1. Bone dysplasia a. Fibrous dysplasia i. Monostotic
Ii. Polyostotic
Iii. Polyostotic with endocrinopathy (McCune–
Albright syndrome)
b. Osteitis deformans/Paget’s disease
c. Pagetoid heritable bone dysplasia
of childhood
d. Segmental odontomaxillary
dysplasia
Cemento-osseous
2.
dysplasia
3. Nammatory/reactive
process
4. Metabolic disease Hyperparathyroidism
5. Neoplastic conditions a. Ossifying broma
a. Focal cemento-osseous dysplasia
b. Florid cemento-osseous dysplasia
a. Focal sclerosing osteomyelitis
b. Diffuse sclerosing osteomyelitis
c. Proliferative periostitis
b. Juvenile ossifying broma i. Trabecular
ii. Psammomatoid
c. Gigantiform cemetoma
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with endocrine abnormality and skin pigmentation and this variant is known as McCune–
Albright syndrome [23].
FD occurs in the rst two decades of life with
equal preponderance in males and females. The
most common symptoms are painless swelling
and asymmetry of the facial skeleton. Involvement
of nose, paranasal sinuses, and foramina of the
skull base, orbit can cause symptoms such as
nasal obstruction, headache, proptosis, visual
abnormality, hyposmia/anosmia. Diagnosis of
this condition requires clinical, radiological, and
histological assessment. Key radiological features on CT scan show radiolucent/radiopaque
depending upon the degree of calcication leading to a typical “ground glass” appearance
(Fig.6.4). This mottling appearance blends with
the surrounding normal bones with ill-dened
margins [24].
Histological feature depends upon the grade
of ossication and calcication within the immature matrix and the trabeculae assumes classic
“Chinese gure script” pattern [22, 23]. These
lesions tend to become quiescent after puberty.
Only cosmetic deformity can be corrected by surgical contouring of the affected site. However,
continuous growth in later life with visual symp-
toms will require surgical decompression of the
optic nerve [25, 26].
6.1.9.2 Ossifying Fibroma
Ossifying broma are true benign tumours
among all the broosseous lesions. Ossifying
broma of craniofacial skeleton divided into following types:
• Ossifying broma of Odontogenic origin
(Cemento-ossifying broma)
• Juvenile Ossifying broma (JOF)
(a) Trabecular Juvenile Ossifying broma
(TrJOF)
(b) Psammomatoid Juvenile Ossifying
broma (PsJOF)
• Gigantiform cementoma
Ossifying bromas are generally unifocal
lesions except the gigantiform cementoma which
are typically multifocal in presentation [22, 23].
Radiologically OF are well-dened unilocular
mass and due to the expansile nature, the cortex is
thinned out (Fig.6.5). The radiolucency on computed tomography scan can vary and depends
upon the degree of calcication. Treatment is usually surgical excision by enucleation or curettage.
The recurrence rate is low in most of the cases.
Fig. 6.4 NCCT Head is showing brous dysplasia
involving all skull base bones. (Courtesy—Dr. Hitesh
Verma, Associate Professor, AIIMS, New Delhi, India)
Fig. 6.5 Unilocular expansile mass lesion in right anterior ethmoid region. It is pushing lamina papyracea and
eyeball laterally
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6.1.10 Benign Vascular Tumours
6.1.10.1 Lobular Capillary
Haemangioma (Pyogenic
Granuloma)
Sinonasal haemangioma accounts for up to 25%
of non-epithelial neoplasms in this region.
Lobular capillary haemangioma are benign vascular lesions and are also known as haemangiomatous granuloma, granuloma gravidarum,
granulation tissue-type haemangioma, etc. The
term “pyogenic granuloma” is a misnomer as neither there is any infective aetiology nor any granuloma formation [27].
A lobular capillary haemangioma is a circumscribed lesion comprising lobules of capillaries
lined by endothelial cells and supported by prominent pericytes. The lobules are separated by
bro-myxoid stroma with few mitotic gures but
never atypical [28]. Most commonly affects
women in their second to third decade of life.
This can be explained by the high incidence of
pregnancy during this time period. These tumours
may occur during the rst trimester of pregnancy,
but the incidence increases until the seventh
month of pregnancy. They occur in approximately 2% of all pregnant females with the most
common presentation being a rapidly growing
nasal mass with recurrent epistaxis. The most frequently affected site is the septum followed by
the turbinate and the sinuses [29].
Management of these lesions depends upon the
timing and severity of presentation. Most of them
resolve spontaneously after the end of pregnancy.
Surgical intervention is considered in patients with
multiple episodes of epistaxis or whose lesions fail
to resolve after pregnancy. Endoscopic surgical
resection and cauterisation is the standard of care
with a high success rate [30, 31].
6.1.11 Other Rare Lesions
1- Neurogenic tumours
Neurobroma and schwannoma are the neurogenic benign tumour of the nose and paranasal
sinuses. Neurogenic tumours are a relatively rare
entity of this region. Maxillary sinus is the most
common site of origin. Symptomatology is the
same with other benign lesions and surgical excision is the treatment of choice.
2- Dermoid
Dermoid cyst is the commonest midline congenital mass lesion of the nasal dorsum. It is
formed due to incomplete obliteration of neuroectoderm of frontonasal region. It can present in the
form of cyst, sinus, and stula with or without
intracranial extension (Fig. 6.6). Both CT and
MRI are required to assess tract extent and for
treatment plan (Fig. 6.7). The presence of bid
crista raised the possibility of intracranial extension of the tract. MRI of the skull base region is
required in such suspected cases to map the tract
extent. External rhinoplasty, vertical midline, lateral rhinotomy, horizontal trans-nasal, and endoscopic approaches are used to excise the nasal part
of the tract. The choice of approach depends on the
external opening of the tract. Neurosurgical team
is required to excise the intracranial part [32].
6.2 Part B: Angiobroma,
Its Medical andSurgical
Management
Angiobroma is rst described by Hippocrates in
the fth century. Other names are, e.g., juvenile
nasopharyngeal angiobroma, nasopharyngeal
broma, juvenile angiobroma. It accounts for
0.05% of all head and neck tumours. It is more
prevalent in familial adenomatous polyposis
(FAP) group patients. It is a benign, locally
aggressive unencapsulated vascular tumour. It
has both vascular and brous elements. Various
authors proposed different theories for its origin.
The most recent and accepted theory is that it is a
vascular malformation and maybe it arises from
persistent tissue of the rst branchial artery. The
nasopharynx is the most common site of involvement. It takes origin from sphenopalatine foreman and extends medially in the nasal cavity,
laterally into pterygopalatine fossa, and superiorly into the cranial cavity. The symptomatology
can vary from unilateral nasal obstruction to lifethreatening epistaxis. It appears grossly as
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Fig. 6.6 Clinical photographs are showing cystic, stulous external presentation. (Courtesy—Dr. Hitesh Verma,
Associate Professor, AIIMS, New Delhi, India)
Fig. 6.7 NCCT PNS is showing bid crista galli and corresponding MRI is not showing intracranial extension of tract.
(Courtesy—Dr. Hitesh Verma, Associate Professor, AIIMS, New Delhi, India)
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pinkish- grey colour lobular mass.
Microscopically, blood vessels are immature
with poorly developed surrounding muscular layers. Mutation and expression of β catenin, p-53,
C-KIT, C-MYC, VEGF receptor, and BMI-1 are
found in the tissue of angiobroma. The most
accepted treatment modality is surgical which
can be endoscopic or external. The choice of
approach is based on tumour extent and surgeon
experience. Radiotherapy and hormonal therapy
are the other treatment modalities.
6.2.1 Extensions
ITF
G. Nayak et al.
Nasal
cavity
PMF
SPF
PP
nasopharynx
Sphenopalatine foreman (SPF) is present in the
lateral nasal wall. The nasal cavity communicates
with the pterygopalatine fossa via a sphenopalatine foreman. The foreman is covered by palatine
bone inferiorly and sphenoid bone superiorly.
Sphenopalatine artery and nerve to the lateral
nasal wall and nasopalatine nerve enter into the
nasal cavity via the foreman. Angiobroma gets
origin from the superior aspect of foreman and it
tends to spread submucosally in close by lessresistant anatomical sites. It follows the standard
pathway of spread because of its xed site of origin and characteristic tumour behaviour. It can
extend medially into the nasal cavity, paranasal
sinuses, nasopharynx, and oropharynx. It can
invade within the cancellous bone of the basisphenoid region. The lateral extension involves the
pterygopalatine fossa (PPF), where It can push the
posterior wall of the maxilla anteriorly which is
known as the Holmen–Miller sign. It further
extends laterally and it involves the infratemporal
fossa (ITF), temporal fossa, and cheek (Fig.6.8). It
can extend behind the pterygoid plates (PP) [33].
Superiorly, it enters into the intracranial cavity
mostly via inferior orbital ssure, orbit, superior
orbital ssure (Fig. 6.9) [34]. The intracranial
extension is found in 10–20% of cases but dural
inltration is very rare. Other pathways are:
1. Nasal cavity®ethmoid roof®erosion of fovea
ethmoidalis or cribriform plate®intracranial.
2. Nasal cavity®erosion of skull base at planum
and sphenoid sinus roof® or lateral wall and
intracranial.
Fig. 6.8 CECT PNS orbit is showing characteristic
extensions of JNA
3. From ITF it may erode the greater wing of the
sphenoid and middle cranial fossa (lateral to
cavernous sinus).
4. It can enter the cranial cavity via the posterior
wall of PPF.Foramen rotundum, vidian canal
are the natural communication site with the
cranium.
5. Rarely Infratemporal fossa® temporal
fossa®intracranial by the erosion of squamous part of the temporal bone.
Demographic Features [35] It is seen in the
rst two decades of life. It is found exclusively in
males and it may be explained by the presence of
high androgen, oestrogen receptors. Nasal
obstruction (70–90%) and recurrent epistaxis
(40–60%) are the most common ways of presentation. Facial pain, persistent rhinosinusitis, and
headache are the other symptoms. Conductive
hearing loss and ear fullness occur after the
blockage of eustachian tube opening in the nasopharynx. Proptosis and facial asymmetry appears
in the advanced stage. Clinical examination
shows the presence of mucoid discharge in the
nasal cavity, anterior displacement of the soft
palate, and widening of space behind the last
upper molar. Nasal endoscopy reveals a single
large pinkish-red lobular mass lling choana and
posterior part of the nasal cavity. It pushes the
posterior part of the middle turbinate anteriorly.
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Investigations
Contrast CT of the nose and paranasal sinuses is
the rst basic investigation (Fig.6.8). Angiobroma
is highly intense after contrast administration
because of rich vascularity. Angiobroma spreads
in a characteristic way, so CT also helps in conrming the diagnosis. Radiology helps inlocating
the site of origin and it also helps in mapping the
Fig. 6.9 Contrast enhancing mass
is involving of both cavernous
sinuses, sphenoid sinus,
retropterygoid space and right ITF
extension of tumour which indeed helps in deciding the treatment policy [36, 37]. Erosion of anterior most of vidian canal favours the theory that it
arises from medial most of PPF [38]. CT angiography is helpful by providing feeder detail preoperatively (Fig.6.10). Digital subtraction angiography
(DSA) is an invasive method and it also helps in
the mapping of feeding vessels. DSA-based
Fig. 6.10 The clinical photograph is showing the widening of space behind the last right molar with a bulged and
displaced soft palate. Two different swellings are appreci-
ated by extension of JNA in the temporal fossa and cheek
region
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Fig. 6.11 CT angiography is showing feeder and tumour blush. Loss of tumour vascularity post embolisation is visible
in DSA pictures
G. Nayak et al.
embolisation of feeder from the branches of external carotid artery reduces bleeding intraoperatively (Fig. 6.11). It is most effective within
24–48hrs before surgery and it can reduce 60–70%
of blood loss. Initial studies create doubt on the
effectiveness of embolisation on tumour excision
by secondary inammation but advancement in
technology and embolisation materials improves
the surgical outcome. Selective and super-selective embolisation reduces accidental slippage of
material into the internal carotid system.
On MRI [37], angiobroma is hypointense on
T1 and intermediate to hyperintense on T2.
Tumour shows hyperintensity after contrast
administration which appears as bags of worms
(Fig. 6.12). MRI is reserve for a condition like
doubtful diagnosis, invasion into cranial fossa,
relationship with cavernous sinus and dura, to
detect a residual tumour in the postoperative
period, to assess the response of medical manage-
Fig. 6.12 Bag of worm appearance in MRI T2 image
ment. The temporary control of the carotid system
in the neck or balloon occlusion of the internal
carotid artery may be required to deal with tumour
receiving major blood supply from ICA.
Recent studies show the presence of prostatespecic membranous antigen in angiobroma tissue. PMSA PET scan is indicated for the detection
of residual tumour in the postoperative period where
MRI raised residual possibility (Fig.6.13) [39].
and ascending pharyngeal artery are the other
common arteries. As the tumour grows, it can
get vascularity from other vessels of the ipsilateral side or contralateral side (36%). Internal
carotid artery feeder generates when mass
extends into the orbital, cranium and with gross
skull base involvement but vidian artery can
supply tumour in early stage because of its
proximity with the site of origin. Tumour
Feeding vessels The branches of the internal
maxillary artery are the most common feeder.
Sphenopalatine is the most common feeding
branch of the maxillary artery. Palatine arteries
receives feeder from the ophthalmic artery and
sometimes direct branch from the internal
carotid artery. The vertebral and basilar artery
can also provide feeders [40].
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6.2.1.1 Staging System
Sessions proposed the rst staging system.
Chandler, Fisch, Radkowski, Pittsburgh, Andrew,
Onerci staging systems are the other staging system proposed at different points of time [35].
Radkowski staging system is the most popular
staging system.
• Stage 1a—JNA is limited to the nasal cavity
and/or nasopharynx (Fig.6.14a).
• Stage 1b—extension into one or more parana-
sal sinuses.
• Stage IIa—minimal extension into PPF
(Fig.6.14b).
• Stage IIb—JNA occupies PPF completely
with or without erosion of orbital bones.
• Stage IIc—extension into ITF with or without
cheek or posterior to the pterygoid plate
(Fig.6.14c).
• Stage IIIa—skull base bone erosion with minimal intracranial extension.
• Stage IIIb—gross intracranial extension ±
cavernous sinus (Fig.6.9).
Syndermann’s staging system [41] is based
on residual tumour blush post embolisation
(Fig.6.11). Residual tumour blush is because of
blood supply from the internal carotid artery.
• Stage 1—Tumour is conned medial to midpoint of PPF, nasal cavity, and no vascularity
after embolisation.
• Stage 2—Tumour extention into paranasal
sinuses and lateral part of PPF without any
residual vascularity.
• Stage 3—Extracranial tumour beyond stage 2
(ITF, orbit, skull base erosion) without residual vascularity.
• Stage 4—Stage 3 tumour with residual
vascularity.
• Stage 5—Intracranial extension. M (medial)
and L (lateral) are used for medial and lateral
to the cavernous sinus.
Fig. 6.13 PMSA PET scan showing dye uptake in sphenoid sinus and close by intracranial part. (Ongoing the-
sis—Dr. Prabhu, Dept of ENT, AIIMS, New Delhi, under
the guidance of Prof. Alok Thakar (ENT) and Prof Rakesh
Kumar (Nuclear Medicine))
a b c
Fig. 6.14 Different stages of JNA. (a) stage Ia, (b) stage IIa, (c) stage IIc
Diagnosis The typical demographic prole of a
patient with a characteristic radiological spread
pattern is sufcient enough to make a diagnosis.
The biopsy is not indicated because it can lead to
excessive bleeding.
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