9. EPIDEMIOLOGY
• Is a multifactorial disease.
• Incidence and geographic distribution depends on several factors
• Genetic susceptibility, diet, environment and personal habits
• Most common in China particularly in southern states and Taiwan.
• Chinese born in America have lesser incidence than those born in China.
• Burning of incense or wood (polycyclic hydrocarbon), use of preserved
salted fish (nitrosamines) along with vitamin C deficient diet may be
other factors operative in China. Uncommon in India but forms 0.5% of all
cancers
• In US increased incidence among black teenagers. Has a bimodal age
distribution. Childhood NPC usually diseases of adolescence.
• M:F 2:1
10. AETIOLOGY
• Exact aetiology is not known. The factors responsible are:
• Genetic – Chinese
• Viral – EBV virus has two important antigens: viral capsid antigen
(VCA) and early antigen (EA). IgA antibodies of EA are highly specific
for nasopharyngeal cancer but have sensitivity of only 70–80% while
IgA antibodies of VCA are more sensitive but less specific.
• Environmental – Air pollution, smoking of tobacco and opium,
nitrosamines from dry salted fish, smoke from burning of incense and
wood
11. PATHOLOGY
• Squamous cell carcinoma or its variants as TCC and
lymphoepithelioma is the most common (85%). Lymphomas(10%) &
rhabdomyosarcomas, malignant mixed salivary tumour or malignant
chordoma (5%) .
• Based on histology, WHO has lately reclassified epithelial growths into
3types:
12. WHO-1 is defined as well–to–moderately differentiated squamous or transitional cell carcinoma with keratin
production.
WHO-2 is nonkeratinizing carcinoma.
WHO-3 is undifferentiated carcinoma, including lymphoepithelioma. This entity consists of malignant epithelial
cells with lymphocytic infiltration.(vast majority)
13. CLASSIFICATION
• Histopathological basis into three types
• Type III is the most common in North America(1 in 100,000 children
dx annually)
• Frequency of different histopathological types may differ from
country to country. Have also been correlated to titres of EBV virus
and also in their response to radiotherapy
• Type II and type III are associated with higher titres of EBV virus and
have higher local control rates with radiotherapy.
14. • Grossly, the tumour presents in three forms:
1. Proliferative. When a polypoid tumour fills the nasopharynx, it
causes obstructive nasal symptoms.
2. Ulcerative. Epistaxis is the common symptom.
3. Infiltrative. Growths infiltrate submucosally.
15. SPREAD OF NASOPHARYNGEAL CARCINOMA
• Local spread- commonest site in NP, fossa of Rosenmüller.
• Anterior spread-blockage of choana and nasal cavity
• Inferior spread- towards oropharynx and hypopharynx
• lateral spread -parapharyngeal space and infratemporal fossa via sinus of Morgagni
• upward spread- towards intracranial structures.
• F. lacerum & F. ovale provide direct route of spread to middle cranial fossa-
diplopia or ophthalmoplegia.
• Vith CN – 1st to be involved. Spread along the post. skull base involves jugular
foramen (CN IX, X, XI), hypoglossal canal (CN XII) or sympathetic nerve (Horner
syndrome).
• Lymphatic spread- may be direct to these nodes or indirectly through
involvement of retropharyngeal(neck stiffness and torticollis) or
parapharyngeal nodes.
• Distant metastases – lung, bone, liver
16.
17. CLINICAL FEATURES
• Age – commonly 5th & 7th decades
of life but ma invol. Younger age
groups
• sex - M 3x more prone than F
• Symptomatology divided into ;
• Nasal. Nasal obstruction, nasal
discharge, denasal speech,
epistaxis.
• Otologic d/t obstr. of eust. tube,
there is CHL, serous or suppurative
OM. Tinnitus and dizziness may
occur
• Ophthalmoneurologic. This occurs
d/t extension of tumour to the
surrounding regions
• Cervical nodal mets – seen in 75%
of the patients, when first seen,
about half of them with bilateral
nodes. May be the only
manifestation of NPC
• Distant mets – may be present at
the time of dx
18. Presenting symptoms and signs of nasopharyngeal
cancer in order of frequency are:
• Cervical LN(mostcommon)(60–90%)
• •Hearing loss 73%
• •Nasalobstruction 78%-nasal symptoms
• •Epistaxis
• •CN palsies. 25% CN VI paralysis, mostcommon of these (squint, diplopia)
• •Headache(61%)
• •Earache
• •Neckpain
• •Weightloss
• Neck swelling 63%
19. DIAGNOSIS
• Complete blood count, EBV titres including IgA and IgG antibodies to
VCA, EA and NA – may correlate with GTV & decrease with tx
• Endoscopic evaluation – can be done under LA using rigid or flexible
endoscopes.
• Imaging studies – CT scan/MRI nasopharynx and neck. High-
resolution, contrast-enhanced CT of neck and nasopharynx is the
study of choice. X-ray /CT chest – mets? CT abd. /US, PET scan
• Biopsy – under LA/GA(EUA) . Strip of mucosa from fossa of
RosenmĂĽller or posterior wall of nasopharynx can be taken.
• Baseline Audiogram- establishes dx of serous OM, S/Es of radiation
and chemoRx which can cause sensorineural HL.
20. Tx
• Radiotherapy – Tx of choice in NPC. Stage I and Inasopharynx, radioRx alone
while stage III and IV require concomitant radiation and chemoRx or radiation
followed by chemoRx.
• ChemoRx – can be given concomitantly or post radioRx. Some stage III and IV
cancers of nasopharynx can be cured by radioRx alone but cure rate doubled
when chemoRx is combined with radiotherapy. Cisplatin or Cisplatin with 5-FU
• Treatment of recurrent and residual (persistent) disease-can occur in neck nodes
or in the nasopharynx.
• Positive nodes in the neck, require radical neck dissection with removal of
sternocleidomastoid muscle, CN XI and internal jugular vein. Ensure no disease exists in the
nasopharynx and there are no distant mets
• Recurrent or residual (persistent) disease in the nasopharynx, evaluated by CT and MRI to
see the size, location and regional extent or infiltration. Can be treated by; Second course of
external radiation, Brachytherapy-can deliver high dose to the tumour with less radiation to
the surrounding structures, Gold grains (Gold 198) have been used or Nasopharyngectomy
21.
22. • Before undertaking nasopharyngectomy exclude extension of growth
intracranially, to parapharyngeal space, or around the internal carotid
artery.
Concurrent cisplatin, 5-fluorouracil, and radiotherapy have been shown to improve survival.
Sequential chemoradiotherapy with gemcitabine and cisplatin has been shown to improve survival in locoregionally advanced nasopharyngeal carcinoma.
Many pediatric studies have used neoadjuvant chemotherapy followed by radiation therapy with improvement in local control