Nasopharyngeal cancer is a head and neck squamous cell carcinoma (SCC) that commonly arises in the fossa of Rosenmuller. It is strongly associated with the Epstein-Barr virus (especially in endemic regions).
It is the second most common head and neck cancer in Kenya. It peaks between 35 and 50 years, and affects men more than women.
WHO histological classification of nasopharyngeal cancer
| WHO Type | Histology | Subtypes |
|---|---|---|
| WHO Type I | Keratinizing SCC | Less associated with EBV and has a worse prognosis |
| WHO Type II | Non-keratinizing SCC | Associated with EBV and has a better prognosis |
| WHO Type III | Non-keratinizing Undifferentiated SCC | This is the most common type. It is strongly associated with EBV and has the best response to treatment. |
Clinical features of nasopharyngeal carcinoma
| Category | Clinical features |
|---|---|
| Nose | Nasal obstruction, epistaxis, rhinorrhoea, and nasal mass |
| Neck | Painless neck mass |
| Ear | Ear blockage, fullness, conductive hearing loss, otitis media with effusion, and otalgia |
| Throat | Oropharyngeal mass, displaced palate, post-nasal drip, hyponasal speech, dysphagia, odynophagia, and airway obstruction |
| Eye | Proptosis, visual disturbance, and impaired eye movement |
| Central Nervous System (CNS) | Xerophthalmia (greater superior petrosal nerve), facial pain (trigeminal nerve), diplopia (CN VI), ophthalmoplegia (CN II, IV, VI), horner syndrome (sympathetic trunk), headache, and convulsions |
- Risk factors
- Male sex
- East Asian or Northern African heritage
- Epstein-Barr Virus (EBV)
- Genetic predisposition (HLA/MHC variants)
- Family history of nasopharyngeal cancer
- Nitrosamines in salted fish and other preserved foods
- Burning of an incense stick and wood
- Cigarette smoking
- Lack of vitamin C
- Alcohol consumption
- Occupational exposure to wood, dust, or smoke
- Polycyclic hydrocarbons
- Chronic nasal infection
- Poor hygiene
- Poor ventilation
- Smoke
- Pathophysiology
- EBV infects nasopharyngeal epithelial cells → viral oncogenes (LMP1) activate pathways that force the cells to proliferate uncontrollably → mutations and evasion of the immune system → malignant transformation from a single infected cell (clonal origin)
- Signs and symptoms
- Neck mass (70%)
- Painless
- This is the most common presentation
- Otitis media with effusion (50%)
- Secondary to eustachian tube obstruction
- This is the second most common presentation
- Nasal obstruction
- Unilateral in early stages
- Cranial nerve palsies
- Abducens nerve palsy is the most common
- Eye symptoms occur in advanced disease
- Recurrent epistaxis
- Trismus
- Headache
- Neck mass (70%)
- Differentials
- Benign tumors
- Angiofibroma
- Choanal polyp
- Squamous papilloma
- Craniopharyngioma
- Hamartoma
- Malignant
- Lymphoma
- Rhabdmyosarcoma
- Chordoma
- Plasmacytoma
- Malignant salivary gland tumor
- Benign tumors
- investigations
- Nasal endoscopy with biopsy for definitive diagnosis
- FNA of enlarged lymph nodes
- CT scan for bone involvement
- PET-CT for metastases
- Treatment
- Treatment is mainly non-surgical
- Radiotherapy for stage I-II
- Radiotherapy to the primary site and bilateral necks, regardless of nodal status
- Chemoradiation for advanced disease
- Neck dissection for post-radiation salvage surgery
- Tracheotomy if there is airway compromise
AJCC Staging of nasopharyngeal cancer
Tumor
| Stage | Definition |
|---|---|
| Tx | Primary tumor not assessed |
| T0 | No primary tumor |
| Tis | Carcinoma in situ |
| T1 | Tumor confined to the nasopharynx, or extension to the oropharynx/nasal cavity w/o parapharyngeal involvement |
| T2 | Tumor extends to the parapharyngeal space and/or adjacent soft tissue (pterygoid mm, prevertebral muscles) |
| T3 | Tumor involves bony structures of skull base/cervical vertebrae/pterygoid and/or paranasal sinus |
| T4 | Tumor invades intracranially/cranial nerves/hypopharynx/orbit/parotid gland and/or extensive soft tissue infiltration beyond the lateral surface of the lateral pterygoid |
Node
| Stage | Definition |
|---|---|
| Nx | Nodes cannot be assessed |
| N0 | No regional LN metastasis |
| N1 | Unilateral metastasis in cervical LN and/or unilateral or bilateral metastasis in retropharyngeal LNs, ≤ 6 cm above the caudal border of cricoid cartilage |
| N2 | Bilateral metastasis in Cervical LNs, ≤ 6 cm above the caudal border of the cricoid cartilage |
| N3 | Unilateral or bilateral metastasis in cervical LNs >6cm and/or extension below the caudal border of the cricoid cartilage |
Metastasis
| Stage | Definition |
|---|---|
| M0 | No distant metastasis |
| M1 | Distant metastasis |