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Tumours of Nasopharynx Dhingra
Tumours of Nasopharynx Dhingra
Tumours of Nasopharynx Dhingra
Tumours of Nasopharynx
279
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280 SECTION IV — Diseases of Pharynx
Figure 49.1. Angiofibroma. Section shows multiple dilated vessels surrounded by fibrous stroma. (A) H&E, ×100. (B) H&E, ×200.
Figure 49.2. (A) Specimen of an extensive angiofibroma in a 32-year-old male. (B&C) CT scans of the same. Note destruction of bone and
extension into pterygopalatine fossa.
Scan to play Nasopharyngeal Fibroma.
Diagnosis Investigations
It is mostly based on clinical picture. Biopsy of the tumour 1. Computed tomography (CT) scan of the head with
is attended with profuse bleeding and is, therefore, avoid- contrast enhancement is now the investigation of
ed. If it is essential to differentiate it from other tumours, choice (Figure 49.3). It has replaced conventional radi-
biopsy can be done under general anaesthesia with all ar- ographs. It shows the extent of tumour, bony destruc-
rangements to control bleeding and transfuse blood. tion or displacements. Anterior bowing of the poste-
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Chapter 49 — Tumours of Nasopharynx 281
Figure 49.3. Embolization of angiofibroma to decrease vascularity: (A) & (B) pre-embolization and (C) after embolization.
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282 SECTION IV — Diseases of Pharynx
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Chapter 49 — Tumours of Nasopharynx 283
growth but no significant regression has been observed born in America have lesser incidence than those born
in practice. in China. Burning of incense or wood (polycyclic hydro-
Chemotherapy. Very aggressive recurrent tumours carbon), use of preserved salted fish (nitrosamines) along
and residual lesions have been treated by chemotherapy. with vitamin C deficient diet (vitamin C blocks nitrosi-
Doxorubicin, vincristine and dacarbazine have been used fication of amines and is thus protective) may be other
in combination. factors operative in China.
Chemotherapy and radiotherapy can arrest the growth Nasopharyngeal cancer is uncommon in India and
and cause some tumour regression but not total tumour constitutes only 0.41% (0.66% in males and 0.17% in
eradication. females) of all cancers except in the North East region
where people are predominantly of Mongoloid origin.
People in Southern China, Taiwan and Indonesia are
OTHER BENIGN TUMOURS more prone to this cancer.
OF NASOPHARYNX Aetiology
They are very rare and arise from the roof or lateral wall The exact aetiology is not known. The factors responsible
of nasopharynx. They include: are:
1. Congenital tumours. They are seen at birth and are 1. Genetic. Chinese have a higher genetic susceptibility
six times more common in females than males. Vari- to nasopharyngeal cancer. Even after migration to oth-
ous types include: er countries they continue to have higher incidence.
(a) Hairy polyp. A dermoid with skin appendages. 2. Viral. Epstein–Barr (EB) virus is closely associated
(b) True teratoma. Having elements derived from all with nasopharyngeal cancer. Specific viral markers are
the three germ layers. being developed to screen people in high-incidence
(c) Epignathi. Having well-developed fetal parts. areas. EB virus has two important antigens: viral cap-
2. Pleomorphic adenoma. sid antigen (VCA) and early antigen (EA). IgA anti-
3. Chordoma. Derived from the notochord. bodies of EA are highly specific for nasopharyngeal
4. Hamartoma. Malformed normal tissue, e.g. haeman- cancer but have sensitivity of only 70–80% while IgA
gioma. antibodies of VCA are more sensitive but less specific.
5. Choristoma. Mass of normal tissues at an abnormal AgA antibodies against both EA and VCA should be
site. done for screening of patients for nasopharyngeal
6. Paraganglioma. cancer.
3. Environmental. Air pollution, smoking of tobacco
and opium, nitrosamines from dry salted fish, smoke
MALIGNANT TUMOURS from burning of incense and wood have all been
incriminated.
NASOPHARYNGEAL CANCER
Pathology
Epidemiology and Geographic Distribution Squamous cell carcinoma in various grades of its differ-
Nasopharyngeal cancer is a multifactorial disease. Its in- entiation or its variants such as transitional cell carcino-
cidence and geographic distribution depends on several ma and lymphoepithelioma is the most common (85%).
factors such as genetic susceptibility, environment, diet Lymphomas constitute 10% and the rest 5% are rhabdo-
and personal habits. myosarcoma, malignant mixed salivary tumour or malig-
Nasopharyngeal cancer is most common in China par- nant chordoma.
ticularly in southern states and Taiwan. On the basis of histology, as seen on light microscopy,
Its incidence in North American whites is 0.25% of all WHO has lately reclassified epithelial growths into three
cancers, while it is 18% in American Chinese. Chinese types (see Table 49.3).
Note: All the types are squamous cell carcinoma when seen under electron microscope. Special stains for epithelial and lymphoid markers are required to
differentiate them from lymphomas.
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284 SECTION IV — Diseases of Pharynx
Grossly, the tumour presents in three forms: nodes. Involvement of retropharyngeal nodes also causes
neck stiffness and torticollis.
1. Proliferative. When a polypoid tumour fills the na-
sopharynx, it causes obstructive nasal symptoms. Distant Metastases. Lung, bone and liver are the most
common sites involved.
2. Ulcerative. Epistaxis is the common symptom.
Clinical Features
3. Infiltrative. Growths infiltrate submucosally.
• Age. It is mostly seen in fifth to seventh decades but
Spread of nasopharyngeal carcinoma may involve younger age groups. It is not uncommon
(see Figure 49.5 ) to see cancer of nasopharynx in twenties and thirties.
• Sex. Males are three times more prone than females.
Local Spread. Commonest site of origin in the na-
sopharynx is the fossa of Rosenmüller. Anterior spread Symptomatology is divided into four main groups:
causes blockage of choana and nasal cavity and inferior
spread is towards oropharynx and hypopharynx, lateral 1. Nasal. Nasal obstruction, nasal discharge, denasal
spread involves parapharyngeal space and infratempo- speech (rhinolalia clausa) and epistaxis.
ral fossa through the sinus of Morgagni, upward spread
is towards intracranial structures. Foramen lacerum and 2. Otologic. Due to obstruction of eustachian tube,
foramen ovale provide direct routes of spread to middle there is conductive hearing loss, serous or suppurative
cranial fossa causing diplopia or ophthalmoplegia. VIth otitis media. Tinnitus and dizziness may occur. Presence
cranial nerve is the first to be involved. Spread along of unilateral serous otitis media in an adult should raise
the posterior skull base involves jugular foramen (CN suspicion of nasopharyngeal growth. Rarely, tumour
IX, X, XI), hypoglossal canal (CN XII) or sympathetic grows up the tube into the middle ear.
nerve (Horner syndrome). These structures can also
be involved secondary to involvement of parapharyn- 3. Ophthalmoneurologic. This occurs due to exten-
geal space. Involvement of pterygoid muscles causes sion of tumour to the surrounding regions. Nearly all the
trismus. cranial nerves may be involved.
Squint and diplopia due to involvement of CN VI,
Lymphatic Spread. Nasopharynx is rich in lymphatics ophthalmoplegia (CN III, IV and VI), facial pain and re-
and an early lymphatic spread is seen in cervical nodes. duced corneal reflex (invasion of CN V through foramen
Ipsilateral nodes are involved more often but contralat- lacerum) may occur. Tumours may directly invade the or-
eral or bilateral nodes can also get involved. Lymphatic bit leading to exophthalmos and blindness (CN II at the
spread may be direct to these nodes or indirectly through apex of the orbit). Involvement of IXth, Xth and XIth
involvement of retropharyngeal or parapharyngeal cranial nerves may occur, constituting jugular foramen
Figure 49.5. Routes of spread (green area) and clinical features (blue area) of nasopharyngeal cancer.
Scan to play Malignant Tumours of Nasopharynx.
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Chapter 49 — Tumours of Nasopharynx 285
syndrome. Usually, this is due to pressure of enlarged lat- also important for side effects of radiation and chemo-
eral retropharyngeal lymph nodes on these nerves in the therapy which can cause sensorineural hearing loss.
neck. CN XII may be involved due to extension of growth
to hypoglossal canal. Horner syndrome may occur due to Classification (see Table 49.4)
involvement of cervical sympathetic chain. WHO classified nasopharyngeal carcinoma on histo-
Nasopharyngeal cancer can cause conductive deafness pathological basis into three types (Table 49.3). Type III
(eustachian tube blockage), ipsilateral temporoparietal is the most common in North America. However, the
neuralgia (involvement of CN V) and palatal paralysis frequency of different histopathological types may differ
(CN X)—collectively called Trotter’s triad. from country to country. These types have also been cor-
related to titres of EB virus and also in their response to
4. Cervical Nodal Metastases. This may be the only radiotherapy. It is observed that type II and type III are
manifestation of nasopharyngeal cancer. A lump of nodes associated with higher titres of EB virus and have higher
is found between the angle of jaw and the mastoid and local control rates with radiotherapy.
some nodes along the spinal accessory in the posterior
triangle of neck. Nodal metastases are seen in 75% of the Treatment
patients, when first seen, about half of them with bilat- 1. Radiotherapy. It is the treatment of choice for na-
eral nodes. sopharyngeal cancer. Stage I and II are treated by
radiotherapy alone while stage III and IV require con-
5. Distant Metastases. involve bone, lung, liver and comitant radiation and chemotherapy or radiation fol-
other sites. Distant metastases may be present at the time lowed by chemotherapy. External beam radiation of
of diagnosis. 6000–7000 cGy can be delivered by linear accelerator
Presenting symptoms and signs of nasopharyngeal to the primary and both sides of neck. More advanced
cancer in order of frequency are: techniques of radiotherapy such as three-dimensional
conformal radiotherapy and intensity modulated radi-
• Cervical lymphadenopathy (most common) (60–90%)
otherapy (IMRT) are now being used more and more.
• Hearing loss
They allow higher dose delivery to the tumour with re-
• Nasal obstruction
duced damage to the adjacent normal structures such
• Epistaxis
as spinal cord, brainstem and parotid glands. IMRT has
• Cranial nerve palsies. CN VI paralysis is the most com-
also been used for recurrent disease where convention-
mon of these
al radiotherapy produces more serious side effects such
• Headache
as transverse myelitis.
• Earache
2. Chemotherapy. Some stage III and IV cancers of naso-
• Neck pain
pharynx can be cured by radiotherapy alone but cure
• Weight loss
rate is doubled when chemotherapy is combined with
radiotherapy. Chemotherapy can be given concomi-
Diagnosis tantly or postradiotherapy. Cisplatin or cisplatin with
1. Endoscopic evaluation. This can be done under local 5-FU have been used. Chemotherapy has also been
anaesthesia using rigid or flexible endoscopes. Growth found useful to control metastases from lymphoepi-
may be proliferative, ulcerative or infiltrative submu- thelioma and undifferentiated carcinoma of nasophar-
cosal. Biopsy can be taken. ynx. Goal of chemoradiotherapy in nasopharyngeal
2. Imaging studies carcinoma is to improve local control of tumour and
(a) CT scan/MRI nasopharynx and neck. High-reso- to treat distant metastases.
lution, contrast-enhanced CT of neck and naso- 3. Treatment of recurrent and residual (persistent) dis-
pharynx is the study of choice. It reveals primary ease. This can occur in neck nodes or in the nasopharynx.
growth, erosion of skull base and clivus, exten- (a) Positive nodes in the neck. They require radical neck
sions to parapharyngeal, retropharyngeal and in- dissection with removal of sternocleidomastoid
tracranial regions. Neck nodes can also be seen. muscle, CN XI and internal jugular vein. Modified
MRI is better for soft-tissue extension. neck dissection is not preferred as extensive dis-
(b) X-ray/CT chest for secondaries lung. ease has been seen on histopathology even when
(c) CT abdomen or ultrasound abdomen for secondar- only a single node was present. Bilateral neck
ies liver. disease may require bilateral neck dissection but
(d) Positron emission tomography scan. It is getting with preservation of internal jugular vein to avoid
popular to show metastases anywhere in the body. cerebral and facial oedema.
3. Biopsy. It can be done under local or general anaes- (b) Recurrent or residual (persistent) disease in the naso-
thesia using endoscopes. In case growth is not visible, pharynx. First it should be evaluated by CT and
but highly suspected because of metastatic nodes, MRI to see the size, location and regional extent or
blind biopsies from multiple sites in nasopharynx can infiltration. More recently, PET-CT has been used
be taken. A strip of mucosa from fossa of Rosenmüller to find any regional or systemic metastases. It can
or posterior wall of nasopharynx can be taken. It may be treated by:
require transpalatal exposure of nasopharynx. General (i) Second course of external radiation. IMRT has
anaesthesia is preferred if occult primary is suspected. been used. Second course of radiation is more
4. Audiogram. A baseline audiogram is important. It not hazardous and causes injury to brainstem, eye,
only establishes diagnosis of serous otitis media but is ear, pituitary gland and temporal lobe.
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286 SECTION IV — Diseases of Pharynx
Note: In nasopharyngeal carcinoma, N. classification is different from that of other mucosal cancers of the head and neck. Enlarged nodes in the lower
neck (supraclavicular fossa) places them in N3 category. Less weightage is given to nodes in upper neck. Nodes even up to 6 cm size are still catego-
rized as N1 as against N2 at other sites.
Supraclavicular fossa or Ho’s triangle is defined as area of neck lying between three points: (i) medial end of clavicle, (ii) lateral end of clavicle and (iii) the
point where neck meets the shoulder (Figure 49.6).
Enlarged node(s) in this triangle, irrespective of the size, are categorized as N3.
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