16 Questions
What is the most common site of origin for nasopharyngeal carcinoma?
The fossa of Rosenmüller
What percentage of patients present with a painless enlarging upper neck mass?
70%
What is a common symptom related to the presence of a mass in the nasopharynx?
Epistaxis
What is a common site of distant metastasis at the time of diagnosis?
All of the above
What is a useful diagnostic tool for non-keratinizing NPC?
All of the above
What percentage of patients with NPC present with dermatomyositis in endemic areas?
1%
What is the detection rate of elevated levels of circulating EBV DNA or RNA in patients with NPC?
85-96%
What is the significance of the posttreatment response level of EBV DNA or RNA?
It is valuable for adapting subsequent treatment
What is the primary role of circulating EBV DNA assay in NPC management?
Surveillance and early detection of disease relapse
What is the imaging modality of choice for assessing and staging NPC?
MRI
What is the sensitivity of FDG PET-CT in detecting distant metastases?
Highly sensitive
What is the estimated global cancer incidence burden of NPC in 2020?
0.7%
What is the typical age-adjusted incidence rate of NPC in most parts of the world?
1-2 cases per 100 000 person-years
In which regions is NPC incidence rate higher than in most parts of the world?
Southern China and South-eastern Asia
What is the role of EBV in NPC etiology?
Critical step in disease progression
What is the outcome of people who migrate from high- to low-risk countries in terms of NPC incidence?
They develop NPC at an intermediate rate between their native and new country of residence
Study Notes
Origin and Presentation of Nasopharyngeal Carcinoma (NPC)
- The commonest site of origin of NPC is the lateral wall of the nasopharynx, especially the fossa of Rosenmüller, followed by the superior posterior wall.
- Most patients present with locoregionally advanced disease.
- The commonest presentation is a painless enlarging upper neck mass (in 70% of cases).
- Other symptoms include blood-stained postnasal drip, epistaxis, nasal obstruction, tinnitus, hearing impairment, and serous otitis media due to Eustachian tube blockage.
Advanced Presentation and Distant Metastasis
- More advanced cases present with headache due to skull base infiltration, and facial numbness and diplopia due to fifth and sixth cranial nerve involvement.
- Approximately 5% of patients have distant metastasis at the time of diagnosis, with common sites being bone, lung, liver, and distant lymph nodes.
- In endemic areas, 1% of patients with NPC present with dermatomyositis.
Serology and Diagnosis
- EBV serology is positive in most patients with non-keratinizing NPC (NK-NPC).
- IgA antibody against EBV VCA and IgG/IgA against EBV early antigens are commonly used diagnostic tools.
- Elevated levels of circulating EBV DNA or RNA can be demonstrated in 85-96% of patients.
- Pretreatment and posttreatment levels of circulating EBV DNA are useful for prognosis and surveillance.
Imaging and Staging
- MRI is the preferred imaging modality for assessing and staging NPC due to its superior soft tissue resolution and ability to visualize perineural extension, bone marrow involvement, and intracranial spread.
- FDG PET-CT is highly sensitive in detecting distant metastases, small lymph node metastases, and local residual/recurrent disease.
Epidemiology
- NPC is a rare malignancy with an estimated 133 500 new cases and 80 000 deaths in 2020, accounting for 0.7% and 0.8% of the global cancer incidence and mortality burden.
- Age-adjusted incidence rates are typically < 1-2 cases per 100 000 person-years, but are higher in certain regions, such as southern China, northern Africa, and south-eastern Asia.
- The etiology of NPC is complex, involving viral, environmental, and genetic components.
This quiz covers the clinical features and presentation of nasopharyngeal carcinoma, including the commonest site of origin and symptoms.
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