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32 Malignant Tumours
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a
de f
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Fig. 32.5 A 52-year-old female affected by right maxillary sinus small cell neuroendocrine carcinoma G3
(T4aN0M0). Pretreatment MRI scans in coronal views
((a) T1W with contrast; (b) T2W) and (c) axial CT scan
show focal erosion of the anterior and posterior maxillary
walls and extension into the premaxillary soft tissue.
Given the good response to induction chemotherapy, the
therapy could stratify patients in “responders”,
eligible for exclusive radiochemotherapy, and
“nonresponders”, who may benet from surgery followed by adjuvant radiotherapy or
radiochemotherapy [31] (Fig.32.5).
Sinonasal Undierentiated
Carcinoma (SNUC)
SNUC is a rare, highly aggressive, undifferentiated carcinoma that lacks by denition squamous
or glandular differentiation. The average age at
diagnosis is 50–60 years, and it shows a male
predominance (2–3:1). The most common sites
involved are the nasal cavity and ethmoid sinus,
and it is usually locally advanced at presentation,
frequently showing orbital, skull base and intracranial involvement.
Nodal metastases occur in less than 15% of
cases, whereas distant metastases are frequent.
patient underwent exclusive radiochemotherapy. MRI
scans in coronal views ((d) T1W with contrast; (e) T2W)
and axial CT scan (f) at 8-month follow-up demonstrate
local control of disease. Legend: T tumour, white arrows
focal erosion of the anterior and posterior maxillary walls,
black arrows the erosion of the maxillary walls is no more
visible
The differential diagnosis is broad and includes
lymphoma, non-keratinizing SCC, ENB and
high-grade NEC; immunohistochemistry demonstrates positivity for cytokeratins and neuronspecic enolase [32].
In 2014 Bishop etal. reported a subset characterized by a lack of SMARCB1 tumoursuppressor gene (also known as INI-1), the
presence of rhabdoid features and a more aggressive behaviour with tendency for regional and
distant metastases [33].
Gray etal. demonstrated a higher prevalence
of HPV in SNUC (64.3%) than previously
reported, thus suggesting a role in the carcinogenic process with a trend towards improved survival [34].
SNUC is a chemosensitive tumour, which
generally presents in local advanced stages
(almost 70% of cases are T4) and may benet
from aggressive multimodality treatment:
Induction chemotherapy followed by either
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P. Battaglia et al.
chemoradiation or surgery with postoperative
irradiation provides the best survival
outcomes.
Different studies have demonstrated the feasibility and effectiveness of induction chemotherapy, which may reduce the incidence of distant
metastases; the most frequently employed regimen is cyclophosphamide, doxorubicin and
vincristine.
Mucosal Melanoma (MM)
MM is an aggressive malignant neoplasm,
accounting for 1% of all melanomas, characterized by a high tendency for recurrence and systemic spread. It does not show gender
predominance and the incidence peak is in the
seventh decade.
Mucosal and cutaneous melanomas are biologically distinct; indeed, MM is characterized
by a complex array of abnormalities with high
rates of KIT mutations (20–40%), followed by
NRAS (15%) and rare BRAF mutations (0–3%)
[35].
In the sinonasal tract, the most common site of
origin is the nasal cavity and tumours originating
in the paranasal sinuses are associated with worse
survival [36].
In 50% of cases, MM is amelanotic, therefore
contributing to a diagnostic delay and a broader
differential diagnosis (that includes ENB, SNUC
and NEC). According to the seventh edition of
the AJCC cancer staging, all MMs are considered
T3–T4 and associated with extremely poor prognosis (5years overall survival <30%).
The treatment of choice is surgery and minimally invasive endoscopic approaches should be
preferred to external aggressive surgeries, which
may be associated with impaired immune balance, hence a higher risk of local recurrence or
systemic dissemination [37].
Adjuvant radiotherapy is generally delivered
in cases of positive surgical margins, although
MM is known to be radioresistant. According to a
large multicentre retrospective study, carbon-ion
irradiation achieves superior local control and
notable survival benet compared to conventional radiotherapy [38].
Recently, novel targeted therapies such as
tyrosine kinase inhibitors (given the high prevalence of KIT gene mutations) and immunotherapy have shown encouraging results;
moreover the combination of radiation, in particular carbon- ion radiotherapy, with concurrent immunotherapy might synergistically
promote tumour response and prolong survival
[39] (Fig.32.6).
Long-term follow-up and endoscopic review
is essential. Interval MRI surveillance scans are
recommended to detect hidden recurrence in
inaccessible areas such as the infratemporal
fossa.
Key Learning Points
• Malignant tumours of the paranasal sinuses
are rare and account for less than 5% of head
and neck cancers.
• The diagnosis is generally delayed and
tumours present in advanced stages because of
non-specic clinical features.
• Unilateral signs and symptoms (e.g. nasal
obstruction, rhinorrhoea, epistaxis, swelling)
unresponsive to medical treatments must raise
suspicion; particular attention must be paid in
patients with occupational exposure to leather
or wood dust.
• A thorough diagnostic workup requires clini-
cal examination with nasal endoscopy, imag-
ing (CT scan, MRI scan with contrast, total
body CT scan) and biopsy; because of the
wide spectrum of histological entities, a path-
ological second opinion should be considered
to conrm the diagnosis.
• Multidisciplinary management is of utmost
importance in the management of sinonasal
malignancies.
• A correct histological diagnosis is mandatory
in order to plan appropriately among different
multimodal treatment protocols.
Permission
No copyright content has been used in the writing of the
present chapter.
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def
427
Fig. 32.6 A 71-year-old male affected by left nasal fossa
mucosal melanoma T3N0M0. Preoperative MRI scans in
coronal views ((a) T1W with contrast; (b) T2W) show the
tumour occluding the left nasal fossa and inltrating the
nasal septum. The patient underwent a left transnasal
endoscopic medial maxillectomy type IIIb with removal
of the nasal septum and drilling of the hard palate.
Intraoperative view (c) shows the tumour in the left nasal
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Juvenile Angiobroma
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HaissanIftikhar, Ann-LouiseMcDermott,
andShahzadaAhmed
33
Natural History oftheDisease
Juvenile angiofibroma is histologically classified as a benign lesion, although it commonly demonstrates aggressive behaviour
with rapid growth and bony erosion of the
sphenoid sinus floor, clivus and pterygoid
plates.
Angiobroma has been referred to as Juvenile
nasopharyngeal angiobroma (JNA) for many
years but this is strictly no longer correct as the
tumour does not arise from the nasopharynx.
The term ‘juvenile’ reects the predilection for its
occurrence in adolescent boys but it can sometimes present in young adult men.
The predilection of angiobroma for adolescent males suggests a hormonally inuenced
tumour. Studies of steroid receptors have had
variable results, generally nding the tumours to
be positive for androgen receptors whose hormone has physiological peaks around puberty.
This also explains why Angiobromas are almost
never seen in girls, and when the tumour is seen
H. Iftikhar · S. Ahmed (*)
University Hospitals Birmingham NHS Trust,
Birmingham, UK
e-mail: shahz.ahmed@nhs.net
A.-L. McDermott
Department of Otolaryngology, Birmingham
Childrens Hospital, Birmingham Womens and
Childrens Hospital NHS Foundation Trust,
Birmingham, UK
in older men, it is likely to have originated in
adolescence.
The exact anatomical site of origin of an
angiobroma is not completely clear: It is initially thought to have its origins from the upper
margin of the sphenopalatine foramen at the
junction of the sphenoidal process of the palatine
bone and the pterygoid process or alternatively
from the pterygoid canal.
The tumour can then expand in many
directions:
1. It may extend into the sphenoid sinus without
inltrating the mucosa.
2. It may spread beyond the sphenoid sinus to
affect the central skull base and its foramina.
3. Occasionally, the cavernous sinus is affected
and in advanced cases, intracranial spread
may occur.
4. The tumour may pass through the sphenopala-
tine foramen, expanding laterally to the pterygopalatine fossa, with anterior bowing of the
posterior maxillary sinus wall demonstrated
on axial CT imaging, known as the HolmanMiller sign [1].
5. Anterolateral spread through the inferior
orbital ssure leads directly to the orbit.
6. Rarely, it can travel through the superior
orbital ssure to the intracranial cavity.
However, the tumour typically stays in the
extradural space and rarely inltrates the dura
and brain (Fig.33.1).
© The Author(s), under exclusive license to Springer Nature Switzerland AG 2023
A. C. Swift et al. (eds.), Contemporary Rhinology: Science and Practice,
https://doi.org/10.1007/978-3-031-28690-2_33
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Fig. 33.2 Haematoxylin and eosin for tumour cells
embedded in a delicate vascular stroma
Fig. 33.1 Routes of spread of angiobroma
Theory andGrowth
Juvenile angiobroma is thought to arise from
incomplete regression of the rst branchial arch
artery. This theory helps explain a number of key
points:
• The rst branchial arch artery recedes close to
the sphenopalatine foramen and the pterygoid
base (which is the region it arises). The remnant of this artery forms part of the maxillary
artery whose terminal branch is the sphenopalatine artery. The predominant blood supply
to an angiobroma is from the internal maxillary artery and its branches including the
sphenopalatine artery. This supports the ‘branchial arch artery theory’.
• The connection of the rst arch artery to cavernous segment of the internal carotid artery
(ICA) also explains how the vascular supply
of Angiobromas is derived from both external and internal carotid arteries [2].
Pathology
Juvenile angiobroma consists of brovascular
tissue (Fig.33.2).
The tissue density varies across the crosssectional area of the tumour as does the range of
vascularity between individual patients. The centre of the tumour is often more brous with fewer
vessels compared to the higher vessel density
near the surface of the pseudocapsule [3]. This
pushes the surgical dissection plane more supercially following the surface of the pseudocapsule to minimize the risk of massive
haemorrhage.
The cross-section of the vessel walls shows
incomplete irregular tissue architecture with a
decient tunica media. As a consequence, the
vessels do not contract after injury and can lead
to profuse haemorrhage. The lack of tunica media
and associated vessel contraction can also lead to
profuse bleeding.
Clinical Features
Symptoms and signs: The most common presenting features are progressive unilateral nasal
obstruction and/or profuse spontaneous epistaxis,
which may or may not be life threatening.
Orbital extension will cause proptosis and
possible visual disturbance, especially if the optic
nerve is compressed.
Lateral growth to the infratemporal fossa or
masseteric space will cause unilateral facial
swelling. Headache and neurological decits can
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Fig. 33.3 Nasal endoscopy (right nostril) demonstrating
angiobroma occupying the nasal cavity
be encountered in rare instances with intracranial
extension of angiobroma.
Clinical examination: Anterior rhinoscopy
typically reveals a smooth well-dened mass in
the nose (see Fig.33.3).
Nasal endoscopy is essential to delineate the
tumour extent that may occupy the nasopharynx
and obstruct the contralateral choanae. Eustachian
tube obstruction and a unilateral middle ear effusion are commonly identied in such cases.
431
Fig. 33.4 CT scan (axial cut) demonstrates angiobroma
in the left pterygopalatine fossa extending laterally into
the infratemporal fossa, medially extending into the nose
and nasopharynx and producing the typical radiological
Holman-Miller sign [1] with anterior bowing of the posterior maxillary sinus wall (arrow)
Radiology
MRI imaging is the modality of choice that facilitates an accurate diagnosis and differentiation
from other tumours, whilst computed tomography (CT) provides information on bone erosion
and bony surgical landmarks.
The typical CT scan ndings demonstrate the
angiobroma as an enhancing expansile mass in
the pterygopalatine fossa that produces the typical radiological Holman-Miller sign [1] with
anterior bowing of the posterior maxillary sinus
wall demonstrated on axial computed tomography (CT) imaging (Fig.33.4).
Magnetic resonance imaging (MRI) is necessary
to demonstrate the anatomical extent and involvement of structures surrounding the angiobroma.
MRI with gadolinium typically depicts a high-signal lesion with characteristic feeding vessels
throughout the tumour that appear as ‘ow voids’
(salt and pepper appearance) best appreciated on
T1, T2 and unenhanced MRI (Fig.33.5).
Fig. 33.5 MRI demonstrating ow voids (salt and pepper
appearance) of angiobroma on the right side
The overall vascularity of individual tumours
however is variable, with a spectrum, some more
brous and some predominantly vascular.
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Finally, CT angiography is imperative for surgical planning to identify the feeding vessels supplying the angiobroma. These vessels may be
branches of both the external and internal carotid
artery systems, and some of these branches may
then be embolized to substantially reduce blood
ow to the angiobroma in readiness for surgery
(Fig.33.6).
The combination of characteristic MRI ndings
of signal voids representing major intralesional
vessels, ‘nger-like’ projections of angiobroma
tumour extension into the surrounding soft tissues
and submucosal invasion of the basisphenoid
strongly support the diagnosis of angiobroma.
These characteristic signs have not been
reported in any other nasal lesions [3]. Biopsy is
contraindicated due to the highly vascularized
nature of the tumour.
Fig. 33.6 Angiography demonstrating vascular blush
attributed to the highly vascular nature of angiobroma
Biopsy
Biopsy carries a serious risk of catastrophic
haemorrhage and is denitely contraindicated
in the outpatient setting. In most cases, the
diagnosis is obvious and biopsy is not
recommended. In the rare event that a biopsy is
thought necessary, it should only be done
in theatre with adequate planning and
precautions.
Staging
Staging the tumour is now an integral part of
tumour assessment during the preoperative
workup and helps plan surgical intervention.
Several angiobroma staging systems have
been proposed according to tumour location and
extension to involve the infratemporal fossa,
orbit and cranial cavity. Sessions et al. [3] proposed the rst staging system in 1981. Since
then, there have been many others including
Andrews etal. [4] based on tumour growth and
spread. Radkowski [5] proposed a classication
mostly based on the size and extent of the angiobroma, and Onerci [6] revised this classication based on whether the tumour was amenable
for endoscopic excision or would require a combined approach. In 2016 a new staging system
was described by Snyderman et al. [7] that
reected the changes in surgical techniques of
recent years, encompassing the endonasal endoscopic techniques. It included the vascularity
and routes of cranial base extension providing
better prediction of immediate morbidity and
tumour recurrence [7, 8] (Table33.1).
Table 33.1 Endoscopic staging system for angiobroma described by Snyderman etal. (2016) [7]
Stage Description
I No signicant extension beyond site of origin and remaining medial to the midpoint of the
pterygopalatine fossa
II Extension to the paranasal sinuses and lateral to the midpoint of the pterygopalatine fossa
III Locally advanced with skull base erosion or extension to additional extracranial spaces, including
orbit and infratemporal fossa, no residual vascularity following embolization
IV Skull base erosion, orbit and infratemporal fossa residual vascularity
V Intracranial extension, residual vascularity M, medial extension; L, lateral extension
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Preoperative Embolization
The tendency of an angiobroma to bleed leads
many surgeons (including our group) to consider
preoperative embolization in the majority of
cases. This requires an experienced interventional neuroradiologist and is ideally undertaken
at a maximum of 48h before surgery. Any delay
in surgery (>48h) has the potential to reopen collateral vessels and increase the risk of signicant
intraoperative haemorrhage. Depending on the
experience and preference of the neuroradiologist, embolization may be either under general
anaesthetic or local anaesthetic. The aim is to
occlude the feeding vessels, thereby reducing
haemorrhage intraoperatively. This can be done
either by trans-arterial embolization (TAE) or
rarely by direct intra-tumoural embolization
(DIE). The latter is usually undertaken via a
direct endonasal route in theatre, and this requires
both surgical and interventional neuroradiology
teams to be working together simultaneously.
Many surgeons however do not use any embolization and rely on intraoperative identication of
the main arterial feeders before tumour
resection.
Trans-arterial embolization (TAE): TAE utilizes particles such as polyvinyl alcohol or microspheres [9]. Similarly, coils, glue and ethylene
vinyl alcohol copolymer can also be used. These
materials are precisely injected to embolize the
feeding vasculature arising predominantly from
external carotid artery branches. These include
maxillary artery, sphenopalatine artery, ascending pharyngeal and descending palatine artery.
Embolizing the supply from branches of internal
carotid artery such as that by the vidian artery,
ophthalmic artery and meningohypophyseal
trunks carries the risk of stroke and blindness by
accidental dislodgement of the particles into the
brain.
Limitations of TAE are small tortuous vessels,
multiple small collaterals, ligation of external
carotid artery secondary to previous surgery and
anastomoses between the extracranial and intracranial circulation.
Intra-tumoural embolization involves direct
intraparenchymal injection (DIE) of the embolic
material. This bypasses the limitations of TAE
but requires the interventional radiologist to
attend theatre at the time of denitive resection.
Depending upon the anatomy of the tumour and
the preference of the interventional neuroradiologist and surgeon, DIE may be done under uoroscopic guidance to prevent reux into the internal
carotid artery vasculature.
It is the lead author’s practice to request navigation protocol CT imaging immediately post
embolization, thereby allowing surgeons to navigate to the embolization coils and feeding vessels
intraoperatively using their surgical image guidance system. This facilitates better control of
bleeding and more proximal tumour resection
[10].
Preoperative Workup
Considerations
In young patients or those who refuse blood
transfusion, a cell saver should be considered. A
group and save sample should be taken from all
patients with blood cross-matched in selected
cases.
Surgery
Surgery should only be performed in centres with
surgeons experienced in the management of
angiobroma particularly those cases with extension beyond the sinuses.
Endoscopic Endonasal Approach
The key to a successful resection is to establish
the true extent of the angiobroma preoperatively
and to create wide surgical corridors to allow
controlled and safe resection under endoscopic
visualization.
Bleeding can be minimized by tumour dissection in a submucosal plane. Very large tumours
are sometimes disassembled using a coblation
dissection wand to allow segmental removal.
Extensive Angiobromas may occasionally bleed
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so much that surgery has to be staged due to
blood loss. In this situation the patient is stabilized ± transfused and angiography repeated to
embolize any feeder vessels from the external
carotid artery that are still patent. The timing of
repeat surgery should ideally be within 1 or
2weeks as additional collateral vessels from the
internal carotid artery start to supply the tumour
that are more challenging to control. The use of
warm irrigation at 49°C has been shown to be
effective in reducing diffuse bleeding from sinonasal mucosa [11]. Haemostatic agents such as
FLOSEAL (Baxter) and Surgiow® Hemostatic
Matrix (Ethicon) can aid in the management of
venous bleeding from the cavernous sinus, pterygoid and the basilar plexuses, but intra-arterial
injection should be avoided.
Wide exposure to visualize the extent of
tumour invasion and growth is of paramount
importance. This can be achieved in a number of
ways:
(a) At the outset, a wide ipsilateral medial max-
illectomy with modied Denker’s approach,
also known as Sturman-Caneld approach
[12], with complete removal of ethmoidal air
cells, and a bilateral sphenoidotomy, is performed. This is augmented by a posterior
septectomy that assists in accessing the sphenoid sinus and can also be utilized for a ‘4
hand 2 nostril’ (two surgeon) approach. The
medial wall of the maxillary sinus should be
drilled up to the nasolacrimal sac, and then
the nasolacrimal duct is cut obliquely with
sharp scissors or a scalpel to give wide access
to the posterior maxillary wall.
(b) Tumour involvement of the pterygopalatine
and infratemporal fossae can be approached
by removing the entire posterior wall of the
maxillary sinus as far as its attachment to the
anterolateral wall. Care must be taken to
avoid injury to the infraorbital nerve and
maxillary nerve in the pterygopalatine fossa.
The maxillary periosteum on the external
surface of the maxilla should be distinguished from the surface of angiobroma for
identication of a clear dissection plane.
Early surgical clip placement on the internal
H. Iftikhar et al.
Fig. 33.7 Intraoperative view of angiobroma being
resected, embolization coils can be seen whilst dissecting
angiobroma
maxillary artery will avoid inadvertent damage and bleeding during surgery. Complete
tumour removal, by drilling out the basisphenoid and the vidian canal, is paramount as
microscopic nests at these regions can lead to
tumour recurrence. The vidian canal often
bleeds as it receives blood from the second
genu of the internal carotid artery. This is
controlled with bone wax (Fig.33.7).
In those cases where an angiobroma extends
into the cavernous sinus or transgresses the foramen rotundum, the authors undertake removal of
this portion in the nal operative steps.
Angiobromas usually have a tough pseudocapsule and the tumour can often be safely teased out
from these structures as the pseudocapsule is
loosely adherent to surround structures. Care
should be taken to avoid direct pressure on the
internal carotid artery, which could compromise
blood supply to the brain.
External Approaches
Historically Angiobromas were resected utilizing open approaches, typically a lateral rhinotomy, midfacial degloving or infratemporal fossa
type C approach. With advances in the understanding of endoscopic skull base anatomy and
enhanced magnied endoscopic visualization,
there has been a paradigm shift to transnasal
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