npc-170324145154.pdf

Introduction
 NPC is a squamous-cell carcinoma arising from epithelial lining of the
nasopharynx.
 Most common malignancy in the nasopharynx
 Nasopharyngeal malignancies
 SCCA (nasopharyngeal carcinoma)
 Lymphoma
 Salivary gland tumors
 Sarcomas

Race: More in Chinese & North African people
Sex: Male preponderance of 3:1
Age: -Its incidence rate starts to rise after the second decade of life.
-Median age is 50 years .
Gross: Proliferative, Ulcerative & Infiltrative types
The most common location is Fossa of Rosenmuller
Introduction

Lymphatic drainage
 Lateral Retropharyngeal L.N also called as
nodes of Röuviere, are the first nodes in the
lymphatic drainage of Nasopharynx.
 Extends from base of skull to C3 cervical
vertebra.

GENETIC
ENVIORMENT
VIRAL
Etiology

Etiology
Genetic:
 Commonest in Chinese population.
 Genomic studies have revealed 3 HLA locus.
 HLAA2; HLA B46; HLA B17 are associated with increased risk of NPC
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

Etiology
Environmental:
 salted fish food contain nitrosamines: carcinogen
 Lack of vit C in diet
 Burning of incense & woods: polyaromatic hydrocarbon:carcinogen
 Alcohol consumption & Cigarette smoking
 occupational exposure to dust, smoke, and chemical fumes

W.H.O. classification of NPC
 1- keratinizing squamous cell ( 25% )
 2. Type II is non-keratinizing squamous carcinomas 12 %
 3. Type III is the undifferentiated carcinomas 60 %

Clinical Features
Upper neck swelling 50%

Clinical Features
Nasal symptoms 30%
 Blood-stained post-nasal discharge
 progressive nasal obstruction,
 Epistaxis

Clinical Features
Otological symptoms:
 Hearing loss
 Otalgia
 Otorrhoea
 Tinnitus

Clinical Features
Ophthalmologic symptoms :
 Diplopia & ophthalmo-plegia (involvement of CN III, IV, VI),
 Proptosis (orbit invasion) &
 Blindness (involvement of CN II).

Clinical Features
Neurologic (25-40 %):
Headache: indicates skull base erosion
Facial pain - Trigeminal
Xerophthalmia - greater sup. petrosal n
Jugular foramen syndrome: CN IX, X, XI involved by lateral retropharyngeal lymph
node

Clinical Features
Other symptoms
 Weight loss
 Anorexia
 Trismus
 Distant metastasis: to bone, lung & liver

Diagnostic Evaluation
High index of suspicion required for early diagnosis
• Clinical evaluation
• Radiological evaluation
• Laboratory evaluation
• Histopathological evaluation

Clinical evaluation
 Detailed medical history
 Examination :
 General physical examination
 Complete head and neck examination

Clinical examination of nasophyrnx:
Indirect nasophayrngoscopy with mirror
Direct nasopharyngoscopy with fiber-optic scope
Rigid 0 and 30* Hopkins rod endoscope

Radiological Evaluation
 Help to make the correct diagnosis
 Help To know the disease stage
 Help to determine the target volume of radiotherapy
 Help to evaluate the treatment results
 Follow-up

 CT Scan
 Extent of tumor
 Neck node involvement
 Skull base erosion
 MRI – radiologic modality of choice
 Determine if any intracranial extension of the tumour 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.

 Positron emission tomography (PET) :
- Useful in diagnosing recurrent /residual lesion following RT.
-Useful to exclude distant metastasis before major salvage .
 Chest x-ray:
To identify lung metastasis
 Abdominal ultrasound:
To find liver metastasis.
 Bone scan.

Laboratory evaluation
 CBC,
 LFT’s
 Special diagnostic tests (for types II & III)
 IgA antibodies for viral capsid antigen (VCA)
 IgG antibodies for early antigen (EA)
 Antibody Dependent Cellular Cytotoxicity assay.

 Biopsy : first necessary investigation for NPC
 Endoscopic biopsy : Ideally it shuold be carried out
during the patient’s ist outpatient visit in suspected
cases.
 The most common sites are roof of nasopharynx and
fossae of Rosenmuller.
 FNA biopsy : should be done in suspicious neck
lump.
Histopathological Evaluation

T.N.M. staging
T1 = Tumour confined to the nasopharynx or extends to oropharynx and/or nasal cavity
T2 = Tumour with parapharyngeal extension
T3 = invasion of bony structures or P.N.S.
T4 = intracranial, involvement of orbit, cranial nerves, infratemporal fossa,
hypopharynx

T.N.M. staging
N0 = no evidence of regional lymph nodes
N1 = unilateral N2 = bilateral
(Both are above supraclavicular fossa & < 6 cm)
N3 = > 6 cm or in supraclavicular foss
N3a- greater than 6 cm in dimension
N3b- extension to the supraclavicular fossa
M0 = no evidence of distant metastasis
M1 = distant metastasis present

T.N.M. staging
 Stage I = T1 N0 M0
 Stage II = T2 or N1 M0
 Stage III = T3 or N2 M0
 Stage IV = T4 or N3 or M1

Treatment
 The management of NPC is unique for two reasons:
1-Tumor is in a relatively inaccessible location
2-Tumors is extremely radiosensitive

Treatment
Treatment Modalities
 Radiotherapy (modality of choice)
 Chemotherapy : combination with radiotherapy in advance
disease
 Surgery :To salvage local and regional failure

Radiotherapy
Modes of radiotherapy
 Teletherapy or External beam radiotherapy :Radiation beams projected to the
target area through skin
 Brachytherapy :uses radioactive material which are placed in close contact with
tumor tissue .
Interstitial: Radioactive source inserted into tumor tissue
Intracavitary: Radioactive source placed inside catheter or moulds & inserted into
nasopharynx

Radiotherapy
Modes of radiotherapy
 Intensity modulated radiation therapy (IMRT): recent development in
delivery of radiotherapy where maximum dose can be delivered to the tumor but
saving important normal structure
 Stereotactic radiosurgery
delivers radiation therapy precisely to the tumor using a machine called a gamma
knife. This can be used to treat tumors that have invaded the base of the skull, or
tumors that have recurred at the base of the brain or skull.

primary treatment
Radiotherapy
 External beam radiotherapy is most commonly delivered by opposed lateral fields
to encompass the primary tumor and upper neck
 Treatment field has to cover nasopharynx ,paraphryngeal space ,oropharynx
,skull base,sphenoid sinus ,posterior ethmoid ,posterior half of maxillary sinus
 Bilateral Cervical nodal irradiation is mandatory even in clinically node-negative
patients

primary treatment
 Radiotherapy
 65-70 GY for primary
 65-70 GY for positive L.N
 50-60 GY for negative L.N
 It should be delivered single fraction daily ,five per week without interruption .
 Proper shielding of all critical structures as well as surrounding normal tissue is
important.

primary treatment
Radiotherapy
 Radiation boosts in the form of intracavitary brachytherapy for T1 to T2 lesions
have been used to improve local control rates
 Stereotactic radiosurgery boosts may also be given for T3 and T4 lesions.

primary treatment
Chemotherapy
 Chemotherapy is believed to act as radiosensitizer.
 It helps to reduce the chance of distant metastasis.
 For locally advanced disease (stage III-IV ) chemotherapy in addition to
radiotherapy appears to improve overall results.
 Combination chemotherapy produces better responses
 combination cisplatin/5-flurouracil is the most widely used
 Indicates that concurrent chemoradiotherapy has a major role in advanced stage
NPC

primary treatment
Complications chemotharpy
 Bone marrow suppression
 Sensorineural hearing loss
 Renal impairment
 Hair loss
 Weight loss
 Nausea and vomiting

primary treatment
FOLLOW-UP PLAN:
 Close monitoring of the progress during and after treatment is necessary .
 Follow-up endoscopy at 6–8 weeks and imaging at 10–12 weeks after
completion of radiotherapy or chemoradiotherapy is recommended to document
tumour responses.
 malignancy detected after 10 weeks usually represents viable tumour and salvage
treatment is indicated

primary treatment
FOLLOW-UP PLAN:
 Close monitoring of the progress during and after treatment is necessary .
 The majority of relapses occur in first three years .
 After primary treatment, patient should be seen :
 Two-monthly for the first year
 Three –monthly for 2nd and third year
 Six –monthly thereafter.
 Lifelong follow-up is needed as very late recurrence may also occur

primary treatment
 FOLLOW-UP PLAN:
 The response of local disease is best followed up by repeated nasoendoscopy .
 Post-treatment biopsy indicated if there is any residual swelling at the primary
site .
 Imaging is often needed to evaluate regional disease .

Salvage treatment
Treatment of recurrence
 Recurrence at the primary site can be treated by surgery or re-irradiation.
 Further dose of ERT may be considered.
 Brachytherapy is preferred.
 Cervical nodal recurrences are best treated by surgery .

Salvage treatment
surgery
• Due to deep location of nasopharynx, and anatomic proximity to critical
structures, radical surgery is typically not used
• Limited to
 biopsy
 Neck dissections for persistently enlarged lymph nodes
 Nasopharyngectomy in persistent or recurrent disease

Salvage treatment
Nasopharyngectmy
 Surgical approaches to the nasopharynx:
 Anterior approaches
 Inferior approaches
 Lateral approaches

Salvage treatment
Anterior approaches :
 Lateral rhinotomy
 Transnasl transmaxillary
 Midfacial degloving
 Maxillary swing (most common approach )

Salvage treatment
Inferior approaches :
 Transplatal :For localized tumour in
the lower part of the posterior wall of the nasopharynx
 Mandibular swing

 lateral infratemporal fossa approach: When the main tumour
bulk is located in the paranasopharyngeal space close or
lateral to internal carotid artery

Salvage treatment
 Surgical salvage for neck disease:
 If a neck node persists in the absence of distant metastasis
,radical neck disection (RND)should be performed

Prognosis
 Approximate 5 years survival rates for NPC

Prognostic factors
 TNM
 EBV
 pathologic type
 Old age
 Cranial nerve palsy
 Level and Fixity of nodes


Complications of Radiotherapy
 xerostomia
 Oropharyngeal mucositis
 Alopecia
 Otitis media with effusion(OME)
 Otitis externa
 Rhinosinusitis
 Trismus
 Neck stiffness
 Dysphagia
 sensorineural deafness

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