Nasopharyngeal Carcinoma Dr. Vishal Sharma

Introduction 85% adult nasopharyngeal malignancies are carcinoma Common pediatric malignancies of naso-pharynx are rhabdomyosarcoma & lymphoma 30% pediatric nasopharyngeal malignancies are carcinoma

Introduction Race: More in Chinese & North African people Sex: Male preponderance of 3:1 Age: Small peak: 12-18 yrs; large peak: 50-60 yrs Gross: Proliferative, Ulcerative & Infiltrative types Histology: 85% Squamous cell carcinoma, 10% Lymphomas, 5% Mixed

Aetiology 1. Genetic: Commonest in Chinese population. HLA-A2 & HLA-B-Sin 2 histocompatibility locus 2. Viral: Epstein-Barr Virus 3. Environmental: Exposure to nitrosamines (dry salted fish), polycyclic hydrocarbons (smoke from incense & wood), smoking, chronic nasal infection, poor ventilation of nasopharynx

W.H.O. classification Type 1: keratinizing squamous cell carcinoma Type 2: non-keratinizing (transitional) carcinoma  Without lymphoid stroma (intermediate cell)  With lymphoid stroma (lympho-epithelial) Type 3: undifferentiated (anaplastic) carcinoma  Without lymphoid stroma (clear cell)

Clinical Features 1. Neck swelling (60-90%): B/L, enlarged upper & middle deep cervical nodes + posterior triangle nodes (Rouviere's sign) 2. Nasal (40-75%): epistaxis, nose block, nasal discharge 3. Otologic (40-70%): Conductive deafness, tinnitus

Clinical Features 4. Ophthalmologic (25-40%): Diplopia & ophthalmo-plegia (involvement of CN III, IV, VI), Proptosis (orbit invasion) & blindness (involvement of CN II). 5. Neurologic (25-40 %): Jugular foramen syndrome: CN IX, X, XI involved by lateral retropharyngeal lymph node Horner's syndrome: sympathetic chain involvement

Clinical Features 6. Severe Headache: indicates skull base erosion 7. Trotter's triad: Conductive deafness: Eustachian Tube block + I/L temporo-parietal neuralgia: Trigeminal damage + I/L palatal paralysis: Vagus damage 8. Distant metastasis: to bone, lung & liver

Neck swelling

Ptosis & adduction palsy

Left proptosis

Investigations 1. Nasopharyngoscopy & Diagnostic Nasal Endoscopy: Tumor mass seen in nasopharynx Commonest site is fossa of Rosenmüller 2. Nasopharyngeal tumor biopsy: seen or blind 3. F.N.A.C. of neck node: done in occult primary 4. C.T. scan head & neck: for tumor extent, skull base erosion & cervical lymph node metastasis

Investigations 5. M.R.I. head & neck: for intracranial extension. 6. Tests for metastases: C.T. chest + abdomen, bone scan, P.E.T. scan, liver function tests. 7. Serologic tests: Immuno-fluorescence for IgA antibodies to Viral Capsid Antigen, IgG antibodies to Early Antigen, Antibody Dependent Cellular Cytotoxicity assay.

Nasopharyngoscopy

Diagnostic Nasal Endoscopy

Computerized Tomogram

CT scan: retropharyngeal node

CT scan: Infratemporal fossa & orbit involvement

CT scan: sella involvement

Magnetic Resonance Imaging

MRI: parapharyngeal mass

MRI: neck node metastasis

M.R.I.: intracranial extension

Endoscopic biopsy

CT scan: liver metastasis

Whole body bone scan

Positron Emission Tomography

T.N.M. staging T1 = confined to nasopharynx T2 = soft tissue involvement in oropharynx or nasal cavity or parapharyngeal space 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 fossa M0 = no evidence of distant metastasis M1 = distant metastasis present

Supraclavicular fossa Synonym: Ho’s triangle A = medial end of clavicle B = Lateral end of C = junction between neck & shoulder

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

Differential Diagnosis 1. Juvenile angiofibroma 2. Rhabdomyosarcoma 3. Lymphoma

Treatment modalities 2. Brachytherapy 3. Chemotherapy 4. Surgery 1. Teletherapy or External beam radiotherapy 2. Brachytherapy 3. Chemotherapy 4. Surgery 5. Immunotherapy against E.B.V. 6. Vaccination against EBV: experimental

Cobalt Teletherapy

External beam irradiation 2 lateral fields: nasopharynx, skull base & upper neck; sparing temporal lobe, pituitary & spinal cord. 1 anterior field: lower neck; sparing spinal cord & larynx

Brachytherapy Used for small tumor, residual or recurrent tumor Interstitial: Radioactive source (Radium, Iridium, Iodine, Gold) inserted into tumor tissue Intracavitary: Radioactive source placed inside catheter or moulds & inserted into nasopharynx High dose rate (HDR): High intensity radiation delivered with precision under computer guidance

Interstitial Brachytherapy

Intracavitary Brachytherapy

High Dose Rate Brachytherapy

Chemotherapy Drugs used: 1. Cisplatin 2. 5-Fluorouracil Indications: 1. Radiation failure 2. Palliation in distant metastasis

Surgery 1. Nasopharyngectomy, Cryosurgery: for residual or recurrent tumor 2. Radical neck dissection: for radio-resistant lymph node metastasis 3. Palliative debulking: for T4 tumors 4. Myringotomy & grommet insertion: for persistent otitis media with effusion

Radical neck dissection & Interstitial Brachytherapy

Treatment Protocol T1 = External Radiotherapy (6500 cGy) T3 & T4 = Radiotherapy + Chemotherapy  Brachytherapy / Salvage surgery if required N0 = External Radiotherapy (5000 cGy) N1, N2, N3 = External Radiotherapy (6000 cGy) + Chemotherapy

Prognosis W.H.O. Type 2 & 3 carcinomas have good response to radiotherapy & better survival rates. 5 year survival rates for treated patients: Stage I = 95 – 100 % Stage II = 60 – 80 % Stage III = 30 – 60 % Stage IV = 20 – 30 %

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