The nasopharynx lies behind the nasal cavity and above the soft palate. It is bounded anteriorly by the choanae and posterior nasal septum, inferiorly by the soft palate, and posterosuperiorly by the skull base. The nasopharynx contains lymphoid tissue and its epithelium changes from ciliated to squamous with age. Juvenile angiofibromas are vascular tumors that typically present in adolescent males with nasal obstruction and bleeding. Nasopharyngeal carcinoma is strongly associated with Epstein-Barr virus and presents with neck masses or cranial nerve palsies. Treatment involves radiation therapy while surgery is reserved for biopsy or residual/recurrent disease.
2. • Lies behind the nasal cavity and above the soft
palate.
• diameter :
length-4cms.
width-4cms.
ap diameter-3cms.
3. Boundaries
• Anterior wall:
• Choanal orifice(free communication between the
nose and nasopharynx.)
• Posterior margin of nasal septum.
• Floor:
• Upper surface of the soft palate.
• Nasopharyngeal isthmus.
• Posterosuperior wall:
• Extends from the base of the skull from the superior
end of the posterior free edge of nasal septum to the
level of upper surface of the soft palate
4. • Posterosuperior wall:
• Extends from the base of the skull from the superior
end of the posterior free edge of nasal septum to the
level of upper surface of the soft palate .
• It is formed by the anteroinferior surface of the body
of sphenoid, basilar part of occipital bone –
basisphenoid.
• Upper portion of posterior wall lies in front of the
anterior arch of atlas.
• It consists of a collection of lymphoid tissue –
nasopharyngeal tonsil.
5.
6. • Fossa of rosenmuller:
• 2.5cms depth in adults.
• Anteriorly - ET orifice
levatorpalati.
• Posteriorly - mucosa covering the pharyngobasilar
fascia.
-retropharyngeal space containing node of
rouviere.
• Medially - nasopharynx cavity.
• Laterally – mandibular nerve, tensor palati,
parapharyngeal space
• Superiorly – foramen lacerum, floor of the carotid canal.
• Posterolaterally – opening of carotid canal and petrous
apex posteriorly. foramen ovale and foramen spinosum-
laterally.
7. Epithelial lining
• Nasopharyngeal mucosa is thrown into folds and
crypts with serous and mucous glands in the
submucosal layer.
• Surface area – 50 cmsq.
• During fetal life there is gradual change from
respiratory ciliated epithelium to squamous
epithelium in the posterior and lower part of
nasopharynx.
• True squamous metaplasia – only in postnatal life,
completed by 10 yrs.
• Most of the epithelium in adults is squamous type.
• Fossa of rosenmuller – columnar epithelium.
8. Lymphatic drainage
• Extensive submucosal lymphatic plexus.
• Responsible for cervical node metastasis in nasopharyngeal
carcinoma.
• First order drainage sites – retropharyngeal nodes.
• Uppermost one i.e Node of rouviere is the main and constant
lateral group. lies anterior to mass of atlas, anteromedial to
internal carotid artery.
• Efferent vessels drain to the uppermost deep internal jugular
chain(in the retrostyloid parapharyngeal space
compartment.)
• Then drain downwards Posteriorly to the accessory nerve
group and Anteriorly to JD group.
• Nasopharynx is a midline structure, high rate of bilateral neck
node metastasis.
9. ANGIOFIBROMA
• Also called juvenile angiofibroma,
• nasopharyngeal angiofibroma,
• bleeding fibroma of adolescence,
• Fibroangioma.
• Vascular swelling.
• Prepubertal and adolescent males. mean age of
presentation-14 yrs.range-7 to 19yrs.
• Strong tendency to bleed.
• Pts have signs of delayed maturity(secondary sexual
characteristics)
10. • Pathophysiology:
• The proposed origin of JNA is said to be from the
posterolateral wall in the roof of the nasopharynx.
• Usually in the superior margin of sphenopalatine
foramen and the posterior aspect of the middle
turbinate.
• Foetal histology confirms presence of endothelial
tissue in this area.
• Rather than invading the surrounding tissues this
tumor distorts and displaces, and pressure necrosis
takes place to destroy and push through its bony
confines.
• Intracranial extension-10 to 20%.
11. • Pathology:
• Gross:
• Firm, spongy, lobulated swellings, nodularity
increases with age.
• Pink or white color.
• Part of the tumor in the nasopharynx is pink(covered
with mucous membrane.
• Part of it which escaped into the extrapharyngeal
areas is white.
• Section:
• Reticulated, whorled or spongy appearance.
• Lacks a true capsule.
• Edge is sharply demarcated
12.
13. • Theories of Pathogenesis:
• Ringertz (1938) – tumor arose from the periosteum
of nasopharyngeal vault.
• Som and neffson – inequalities in the growth of
bones of skull base results in hypertrophy of
underlying periosteum,in response to hormonal
influence.
• Bensch and ewing – tumor probably arose from
embryonic fibrocartilage between basiocciput and
basisphenoid.
• Brunner – origin from conjoined pharyngobasilar and
buccopharyngeal fascia.
14. • Sites of origin:
• Previously – vault of the nasopharynx or choana.
• Modern methods – sphenopalatine foramen.
• Large tumors are bilobed dumb bell swellings
straddling the sphenopalatine foramen.
• One component filling the nasopharynx and other
extending out into the pterygopalatine and
infratemporal fossa.
• The central stalk occupies the sphenopalatine
foramen at the upper end of the vertical plate of
palate.
15. • It may displace the maxillary nerve upwards and
sometimes optic nerve and invade orbit through the
inferior orbital fissure – proptosis.
• Blood supply of the tumor:
• Maxillary artery.
• Ascending pharyngeal artery
• Un named branches from internal carotid artery.
16. Signs and symptoms:
• 2 cardinal symptoms
1. nasal obstruction.(stasis,sepsis,hyposmia or
anosmia)
2. unprovoked intermittent epistaxis.(occasional
show or a life threatening bleed)
• Voice – nasal intonation.
• If soft palate is pushed down – plummy quality voice.
• Blockage of eustachian tube – CHL and otalgia.
• Headache – CRS, intracranial extensions.
• Diplopia – secondary to the erosion of mass into the
cranial cavity causing pressure on optic chiasma
• Failing of vision – pressure on optic nerve.
17. • Anterior rhinoscopy:
-- abundant mucopurulent secretions.
-- bowing of the septum to the uninvolved side.
• Posterior rhinoscopy:
-- pink or red mass filling the nasopharynx.
• Gross physical signs are evident when the tumor has
involved the nose and infratemporal fossa.
• Nasal bones are splayed out.
• Swelling in the cheek and temple
• Intraoral palpation in the area between the
ascending ramus of mandible and the side of maxilla
– fullness bcoz of tumor that has crept around the
back of the antrum
18. • Impaction of bulky mass in the infra temporal fossa
results in extreme signs, such as trismus and bulging
of the parotid gland.
• Proptosis is a definite sign that the orbital fissures
have been penetrated.
• The classic frog face seen in patients with extensive
disease is due to massive escape of the disease.
19.
20. • Fisch staging classification:
• Done for prognosis and therapeutic approaches
Stage I: Tumor limited to the nasal cavity
Stage II: Tumor extension into the pterygopalatine
fossa, or maxillary, sphenoid or ethmoid sinuses.
Stage IIIa: Tumor extension into the orbit or
infratemporal fossa without intracranial involvement.
Stage IIIb: Stage IIIa with extradural (parasellar)
intracranial involvement
Stage IVa: Intradural without cavernous sinus,
pituitary, or optic chiasma involvement
Stage IVb:Involvement of the cavernous sinus,
pituitary, or optic chiasma
21. • Investigations:
• Standard radiographs of pns – opacity in the nasal and
sinus areas.
• Xray skull lateral view – mass in the nasopharynx.
• Tomography in fronto-occipital plane –
1. helpful in localizing the tumor.
2. areas of bone destruction
3. invasion of the sphenoid sinus.
• Lateral tomogram:
-- forward bulging of the posterior antral
wall(Hallman Miller sign) typical if the tumor fills
pterygopalatine fossa.
22. • CT &MRI appearances that are confirmed by
angiography.
• Invasion of sphenoid sinus, erosion of grater wing of
sphenoid, extension into the pterygopalatine and
infratemporal fossa is detectable.
• Selective angiography – recurrent angiofibroma.
• Vascular blush in the postnasal space and adjacent
areas with contrast – characteristic of angiofibroma.
(need for biopsy)
• MR angiography – to know the size and location of
the feeding artery of the tumor (embolization)
25. • Treatment:
• Depends mainly on the extent of the lesion.
• Surgery is the preferred modality of treatment, if left
alone they expand into neighbouring cavities. for all
stages of the mass up to stage IVa while radiotherapy
is used for stage IVb.
• Mainly three lines of treatment are available:
1. Surgery
2. Irradiation
3. Hormonal (purely supportive in nature)
27. • Surgical treatment:
• There are various approaches for removal of tumor.
• If tumor is confined to nasopharynx with a small pedicle –
transpalatal approach.
• Tumor extended out into posterior ethmoids and
infratemporal fossa – lateral rhinotomy, medial maxillectomy.
• Alternative approach – midfacial soft tissue degloving.
Anterior, medial, posterior and lateral walls of maxillary
antrum can be removed.
• This produces a large cavity, confluent with nasal cavity and
postnasal space, gives adequate access for tumor removal
and control of bleeding. extensions into the infratemporal
fossa and inferior part of orbit can be removed.
30. Anatomy
• Anteriorly -- nasal cavity
• Posteriorly -- skull base and vertebral
bodies
• Inferiorly -- oropharynx and soft palate
• Laterally --
– Eustachian tubes and tori
– Fossa of Rosenmuller - most common location
31. Anatomy
• Close association with skull base foramen
• Mucosa
– Epithelium - tissue of origin of NPC
• Stratified squamous epithelium
• Pseudostratified columnar epithelium
– Salivary, Lymphoid structures
32. Epidemiology
• Chinese native > Chinese immigrant > North
American native
– Both genetic and environmental factors
• Genetic
– HLA histocompatibility loci possible markers
33. Epidemiology
• Environmental
– Viruses
• EBV- well documented viral “fingerprints” in tumor
cells and also anti-EBV serologies with WHO type II
and III NPC
• HPV - possible factor in WHO type I lesions
– Nitrosamines - salted fish
– Others - polycyclic hydrocarbons, chronic nasal
infection, poor hygiene, poor ventilation
34. Classification
• WHO classes
– Based on light microscopy findings
– All SCCA by EM
• Type I - “SCCA”
– 25 % of NPC
– moderate to well differentiated cells similar to
other SCCA ( keratin, intercellular bridges)
35. Classification
• Type II - “non-keratinizing” carcinoma
– 12 % of NPC
– variable differentiation of cells ( mature to
anaplastic)
– minimal if any keratin production
– may resemble transitional cell carcinoma of the
bladder
36. Classification
• Type III - “undifferentiated” carcinoma
– 60 % of NPC, majority of NPC in young
patients
– Difficult to differentiate from lymphoma by light
microscopy requiring special stains & markers
– Diverse group
• Lymphoepitheliomas, spindle cell, clear cell and
anaplastic variants
37. Classification
• Differences between type I and
types II & III
– 5 year survival
• Type I - 10% Types II, III - 50%
– Long-term risk of recurrence for types II & III
– Viral associations
• Type I - HPV
• Types II, III - EBV
39. Clinical Presentation
• Xerophthalmia - greater sup. petrosal n
• Facial pain - Trigeminal n.
• Diplopia - CN VI
• Ophthalmoplegia - CN III, IV, and VI
– cavernous sinus or superior orbital fissure
• Horner’s syndrome - cervical sympathetics
• CN’s IX, X, XI, XII - extensive skull base
40. Clinical Presentation
• Nasopharyngeal examination
– Fossa of Rosenmuller most common location
– Variable appearance - exophytic, submucosal
– NP may appear normal
• Regional spread
– Usually ipsilateral first but bilateral not
uncommon
• Distant spread - rare (<3%), lungs, liver,
bones
41. Radiological evaluation
• Contrast CT with bone and soft tissue
windows
– imaging tool of choice for NPC
• MRI
– soft tissue involvement, recurrences
• CXR
• Chest CT, bone scans
42. Laboratory evaluation
• Special diagnostic tests (for types II & III)
– IgA antibodies for viral capsid antigen (VCA)
– IgG antibodies for early antigen (EA)
• Special prognostic test (for types II & III)
– antibody-dependent cellular cytotoxicity (ADCC)
assay
• higher titers indicate a better long-term prognosis
• CBC, chemistry profile, LFT’s
43. Staging
• Variety of systems used
– Am Jt Comm for Ca Staging
– International Union Against Ca
– Ho System
• Unique NPC prognostic factors often not
considered and similar prognosis between
stages
44. Staging
• Neel and Taylor System
– Extensive primary tumor +0.5
– Sx’s present < 2 months before dx - 0.5
– Seven or more sx’s +1.0
– WHO type I +1.0
– Lower cervical node dx +1.0
– -------------------------------------------------------
• ADCC assay titer considered if available
45. Staging
• Stage A = < 0
• Stage B = 0 to 0.99
• Stage C = 1 to 1.99
• Stage D = > 2
47. Treatment
• External beam radiation - complications
– More severe when repeat treatments required
– Include
• xerostomia, tooth decay
• ETD - early (SOM), later (patulous ET)
• Endocrine disorders - hypopituitarism,
hypothyroidism, hypothalamic disfunction
• Soft tissue fibrosis including trismus
• Ophthalmologic problems
• Skull base necrosis
48. Treatment
Surgical management
• Mainly diagnostic - Biopsy
– consider clinic bx if cooperative patient
– must obtain large biopsy
– clinically normal NP - OR for panendo and bx
• Surgical treatment
– primary lesion
– regional failure with local control
– ETD
49. Treatment
Surgical management
• Primary lesion
– consider for residual or recurrent disease
– approaches
• infratemporal fossa
• transparotid temporal bone approach
• transmaxillary
• transmandibular
• transpalatal
50. Treatment
Surgical management
• Regional disease
– Neck dissection may offer improved survival
compared to repeat radiation of the neck
• ETD
– BMT if symptomatic prior to XRT
– Post XRT
• observation period if symptoms not severe
• amplification may be more appropriate
51. Treatment
• Chemotherapy
– Variety of agents
– Chemotherapy + XRT - no proven long term
benefit
– Mainly for palliation of distant disease
• Immunotherapy
– Future treatment??
– Vaccine??
52. Conclusion
• 40% overall survival at 5 years
• Complete H&P, careful otologic, neurologic,
cervical and NP exams
• Three WHO types - all from NP epithelium
• Types II, III - better prognosis, EBV assoc.
• Treatment is primarily XRT