CAUSES OF

PROPTOSIS
OTORHINOLARYNGOLOGY
NASOPHARYNGEAL
CARCINOMA
INTRODUCTION
• NPC is a squamous-cell carcinoma arising from epithelial lining of the nasopharynx
• Most common malignancy in the nasopharynx
• Race: More in Chinese
& North African people
• Sex: Male
predominance of 3:1
• Age : Incidence rate
rise after second
decade of life
: Median age is
50 years
• Gross: Proliferative,
Ulcerative, Infiltrative
Types
• The most common
location is Fossa of
Rosenmuller
 ETIOLOGY
GENETIC

• Commonest in Chinese population

• Genomic studies revealed HLA A2, HLA B46, HLA B17 associated with increased risk of NPC

VIRAL

• EBV
• HPV

ENVIRONMENTAL

• Lack of Vitamin C in diet

• Burning of incense & woods: polyaromatic hydrocarbon: carcinogen
• Alcohol consumption & Cigarette smoking
• Occupational exposure to dust, smoke and chemical fumes
 Upper neck swelling: Painless / Opthalmologic symptoms
unilateral / bilateral - Diplopia & Opthalmo-
plegia (involvement of CN
CLINICAL Nasal symptom
III, IV, VI)
- Proptosis (orbit invasion)
FEATURES - Blood-stained post-nasal
- Blindness (involvement of
discharge
CN II)
- Progressive nasal obstruction
- Epistaxis
Neurologic
- Headache: indicates skull
Otological symptoms base erosion
- Hearing loss - Facial pain: Trigeminal
- Otalgia - Jugular foramen
syndrome: CN IX, X, XI
- Otorrhea involved by lateral
- Tinnitus retropharyngeal lymph
node
Routes of invasion of NPC to Orbit

1) The pterygopalatine fossa and

inferior orbital fissure are the most
common routes of invasion.

Inferior orbital fissure forming direct

communication between the
pterygopalatine fossa and the apex
of the orbit.

NPC involving the pterygopalatine

fossa and infratemporal fossa may
thus infiltrate directly into the orbit
through the inferior orbital fissure.
2) Tumours in the ethmoid and/or
sphenoid sinuses may erode the
Lamina Papyracia to reach the medial
orbit and retrobulbar region.

Second most common pathway of

orbital invasion.

On rare occasions, NPC involving the

maxillary sinus may invade the inferior
orbit via the floor of the orbit.

As the lamina papyracia and the orbital

floor are thin, they are relatively weak
barriers for protecting against tumour
infiltration.
DIAGNOSTIC EVALUATION

• Indirect nasopharyngoscopy
with mirror
• Direct nasopharyngoscopy with
fiber-optic scope
• Rigid 0’ and 30’ scope
 CT Scan
- Extent of tumor
- Neck node involvement
- Skull base erosion
 Contrast-enhanced computed tomography scans

(a) Right nasal cavity tumor with invasion of the right maxillary (b) with invasion of the right orbit and
sinus and frontal sinus . destruction of the medial orbital wall
Figure 3 Computed tomography (CT) images. Axial (A, C, D) and coronal (B) images reveal a soft
tissue mass (★) in the extraconal and intraconal space of the right orbit.
 MRI
- Radiologic modality of choice
- Determine if any Intracranial
extension of the tumor
involves the brain parenchyma
or the cavernous sinus

MRI>CT for displaying both

superficial and deep
nasopharyngeal soft tissue
and for differentiating tumor
from soft tissue
 b) CECT neck
revealing
heterogeneously
enhancing, infiltrating
mass arising from the
roof of the
nasopharynx and
Figure shows: disrupting the medial
a) Cervical swelling and left eye proptosis on clinical wall of the left orbit.
inspection.
 Case Study
A 68-year-old male presented with a huge left eyelid mass for more than one year. Vision was 6/120 in the right eye and finger
counting in the left eye. Ophthalmic examination revealed a protruding mass, about 3.7 cm in diameter, at the medial canthal region
(Figure 2a). The surface of the tumour was irregular, with ulceration and whitish discharge.
Orbital CT scan showed a large mass occupying the orbital and nasal cavities, which seems to originate from the upper nasal
cavity and invade the medial orbital wall (Figure 2b). The left eye was compressed and deviated laterally.
A biopsy of the left eyelid mass was performed and it revealed NPC.
The patient then received radiotherapy. Nevertheless, he died 6 months later.
 Case Study
This 43-year-old female patient was diagnosed with undifferentiated NPC at another hospital 2 years prior to her
presentation at our clinic, with initial symptoms of left mandibular and temporal pain. She came to our hospital for
help and received radiotherapy with the diagnosis of NPC at the T4N0M0 stage. She developed left trigeminal
pain and limitation of extraocular movement 1 year after the radiotherapy.

Ophthalmic examination revealed limited extraocular movement of all directions in the left eye.
Vision was 6/6 in both eyes.

An MRI study showed recurrence of NPC over the left cavernous sinus with intracranial extension. Concurrent
chemotherapy and radiotherapy were given and the patient seemed to be in stable condition thereafter.
After 1 year, a CT scan revealed enlargement of the pterygopalatine fossa and inferior orbital fissure (
Figure 3a).
Tumor recurrence with multiple cranial neuropathies was also noted 5 months later. Ophthalmic examination
then revealed left eye proptosis with lagophthalmos and total ophthalmoplegia.
Vision was 6/6.7 in the right eye with no light perception in the left eye.
The pupil was dilated and fixed in the left eye. Fundus examination showed venous tortuosity and congestion
in the left eye.
A CT scan showed a heterogenous enhancing mass lesion, about 3 cm in diameter, over the left anterior
inferior temporal fossa, left orbit, and lower temporal lobe (Figure 3b).

The patient continued to receive chemotherapy. However, the tumour progressed to a large soft-tissue mass,
about 7 cm in size, in the left infratemporal fossa, nasopharynx, orbit, and skull base.

In spite of chemotherapy with a regimen of cisplatin, ifosfamide, and mitomycin C, the patient died in the 52nd
month of follow-up.
 Management of NPC
• Determine disease stage:
Stage 1 (T1,N0,M0) 🡪 Tx with definitive RT to
nasopharynx & Elective RT to neck
Stage 2, 3, 4B 🡪 Concurrent
chemoradiotherapy
Stage 4C (AnyT, Any N, M1) (Distant
Metastasis) 🡪 Palliative treatment

IF Recurred/ Persistent:-
Local – Nasopharyngectomy OR Re-irradiation
with external beam RT or brachytherapy
Regional – Neck dissection, Re-irradiation,
Chemotherapy
Distant – Palliative chemo, RT to palliative sx,
Palliative care
REFERENCES
• https://doi.org/10.1016/j.bjorl.2015.0
4.006
• https://www.moh.gov.my/moh/resour
ces/Penerbitan/CPG/Kanser/QR%20Na
sopharyngeal%20Carcinoma.pdf
• http://dx.doi.org/10.18203/issn.2454-
5929.ijohns20150905
• https://doi.org/10.1038/sj.eye.6701358
SINONASAL MALIGNANCIES
TABLE 7.1 Patterns of local spread

Primary site Anteriorly Posteriorly Medially Laterally Superiorly Inferiorly

Anterior cranial fossa, frontal

Frontal sinus Skin Ethmoid sinus, nasal cavity
lobes

Sphenoid, nasopharynx, clivus, Anterior cranial fossa, frontal

Ethmoid sinus Skin Nasal cavities, cribriform plate Orbit Nasal cavity
pituitary gland lobes

Pterygopalatine fossa,
Maxillary sinus Cheek, skin infratemporal fossa, temporal Nasal cavity Cheek, skin Orbit Palate
bone, middle cranial fossa

Clivus, pituitary gland, Middle cranial fossa, cavernous

Sphenoid sinus Ethmoid sinuses Pituitary gland, hypothalamus Nasopharynx
posterior cranial fossa sinus

Anterior cranial fossa, frontal

Nasal cavities Skin Sphenoid sinus, nasopharynx Maxillary sinuses Palate
lobes

An ethmoid carcinoma spreading into the orbit and producing

PROPTOSIS.
Squamous cell carcinoma
- SCC is the most common sinonasal malignancy.
- The Highest incidence is in 7th decade of life
- Male predominance
- Mostly arise from lateral wall of nasal cavity with 50% developing on turbinates
- Macroscopic: Some sinonasal SCCs have a polypoid appearance, while others are more obviously fungating,
friable and keratinizing

Adenocarcinoma
- 9% of sinonasal malignancies
- Male predominance , 6th-7th decades of life
- Generally found in upper nasal cavity and ethmoid sinuses
- Slow growth rate, Rarely metastasize

Adenoid cystic carcinoma

- <5% of sinonasal malignancies
- Maxillary sinus most common affected site
- Patient usually presented with long history of facial pain that can defy diagnosis for many months if not
OLFACTORY NEUROBLASTOMA /
AESTHESIONEUROBLASTOMA (OAN)
• OAN Rare Neuroectodermal malignant tumor originating from basal
cells within the olfactory neuro-epithelium.
• 5% of all Sinonasal malignancies
• Age: 20 – 50 years of age
• Woman more predominance than men
• Neuroendocrine tumour capable of causing paraneoplastic syndromes
by secreting peptides.
• Can cause Cushing’s syndrome, inappropriate ADH hormone or HTN
produced by Vasoactive Peptides
• OAN is one of a group of ‘small round blue cell tumours’ and needs to
be differentiated from sinonasal undifferentiated carcinoma (SNUC),
neuroendocrine tumour, small cell carcinoma, rhabdomyosarcoma and
lymphoma.
• Histopathology: OAN typically expresses neuroendocrine markers
(neurone specific enolase, synaptophysin and chromogranin) and is
negative for keratins.
• Hyams et al.16 developed a four histological grading system for OAN
based on:
- degree of differentiation
- tumour architecture
- mitotic index
- nuclear polymorphism
- fibrillary nature of the matrix
- tumour necrosis
Clinical Most common: Less common:
- Visual changes
Features - Nasal obstruction
- Epistaxis - Headache
- PROPTOSIS
- Persistent nasal
- Diplopia
discharge
- Hyposmia
This fast-growing tumor can be asymptomatic until it fills
- Facial pain
the nasal cavity and causes obstruction and/or epistaxis. - Facial swelling &
It may spread into the cranium, orbit, and paranasal sinuses.
Syncope
- Sinus pain
Orbital invasion can lead to vision loss, ophthalmoplegia,
and proptosis.
Disease-specific staging systems have been devised for OAN. The most useful and commonly used
systems are those attributed to Kadish et al,17 Morita et al.18 and Dulguerov and Calcaterra19

Dulguerov staging system19

Kadish staging system17 (with Morita’s modification)18
Stage
Stage Characteristic

Tumour involving the nasal cavity or paranasal sinuses (excluding the A Limited to nasal cavity
T1
sphenoid sinus) sparing the most superior ethmoidal cells
B Involving nasal cavity and sinuses
Tumour involving the nasal cavity or paranasal sinuses (including the
T2
sphenoid sinus) with extension to or erosion of the cribriform plate C Extension beyond nasal and paranasal sinuses cavities
Tumour extending into the orbit or protruding into the anterior cranial D Tumour with metastasis to cervical nodes or distant sites
T3
fossa without dural invasion

T4 Tumour involving the brain

N0 No cervical lymph node metastases

N1 Any form of cervical lymph node metastases

M0 No metastases

M1 Any distant metastases.

In brief, ONB is a rare endonasal tumor. It may manifest with
initial findings of cranial nerve palsy, proptosis, and/or
compressive optic neuropathy. Therefore, this rare etiology
must be considered in the differential diagnosis of cranial
nerve palsy, external ophthalmoplegia, and proptosis.
THANK YOU
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