Cancer Types

Nasopharyngeal Cancer: What patients should know about

Materials of this image are taken from Vecteezy.com

May 16, 2024, 15:26

Nasopharyngeal Cancer: What patients should know about

What is Nasopharyngeal Cancer?

Nasopharyngeal Cancer (NPC) is a rare type of head and neck cancer that originates in the nasopharynx, the upper part of the throat behind the nose. It is a distinct subtype of head and neck cancer with unique epidemiological and clinical characteristics.

Nasopharyngeal carcinoma. This image is taken from newsnetwork.mayoclinic.org.

Prevalence

Nasopharyngeal cancer is rare in the United States, affecting only about 1 out of 100,000 people each year. However, it is more common in certain regions, particularly in Southeast Asia, North Africa, and the Middle East. In endemic areas like southern China, the incidence can be as high as 20-50 cases per 100,000 people per year. NPC is nearly twice as common among men as it is among women and is diagnosed more often among people over age 50 than it is among younger people. In the U.S., it typically affects people ages 15 to 24 and ages 65 to 79.

Risk Factors and Prevention

The exact cause of Nasopharyngeal cancer is not fully understood, but several risk factors have been identified:

Ethnic background – Having Chinese or Asian ancestry is a significant risk factor.
Epstein-Barr virus (EBV) infection – EBV is strongly associated with nasopharyngeal carcinoma, especially in endemic areas.
Genetic factors and family history – Genetic predisposition and having a family member with NPC increase the risk.
Environmental exposures – Consumption of preserved foods containing nitrosamines, such as salted fish, may be a risk factor.
Tobacco smoking and heavy alcohol consumption – These lifestyle factors may increase the risk in non-endemic areas.

To prevent Nasopharyngeal cancer, it is important to avoid known risk factors such as tobacco use and excessive alcohol consumption. Maintaining a healthy diet and regular physical activity may also help reduce the risk. Regular health check-ups and screenings are crucial for early detection, especially in high-risk populations. HPV vaccination may help prevent HPV-associated NPC cases, especially in younger non-smokers.

Types

There are three main types of nasopharyngeal cancer:

Keratinizing squamous cell carcinoma: This is the most common type in areas with low rates of NPC, such as the United States. It is typically found in older adults.
Non-keratinizing carcinoma: This type is further divided into:

- Non-keratinizing differentiated carcinoma
- Non-keratinizing undifferentiated carcinoma (also known as lymphoepithelioma)

Basaloid squamous cell carcinoma: This type is very rare.

The non-keratinizing types (types 2 and 3) are most commonly found among younger children and adolescents. Both non-keratinizing types have been associated with elevated levels of EBV titers, while the keratinizing type (type 1) has not.
Additionally, the non-keratinizing types may be followed by an influx of inflammatory cells, including lymphocytes, plasma cells, and eosinophils, a condition referred to as lymphoepithelioma. The treatment is generally the same for all types of Nasopharyngeal cancer, but the stage of the cancer (how far it has grown and spread) is often more important than the type in predicting a person’s outlook.

Symptoms

The most common symptom of nasopharyngeal cancer is a painless lump or mass on the side of the neck. This occurs when the cancer spreads to the lymph nodes in the neck, causing them to swell. The lump is usually not painful and may be the first noticeable sign of the disease. Other common symptoms include:

Hearing loss, usually only in one ear
Tinnitus (ringing in the ears) or a feeling of fullness in the ears
Blocked or stuffy nose, usually only on one side
Nosebleeds
Headaches
Blurred or double vision
Facial pain or numbness, especially in the lower part of the face
Difficulty swallowing
Hoarseness of the voice
Unexplained weight loss

Less common symptoms may include:

Chronic ear infections that keep coming back
Difficulty opening the mouth
Sore throat
Blood-stained sputum (phlegm)

It’s important to note that many of these symptoms are similar to those of other, less serious conditions. Having one or more of these symptoms does not necessarily mean that a person has NPC. However, if symptoms persist for more than two weeks or keep recurring, it is crucial to seek medical attention. Early-stage NPC may not cause any symptoms at all. Many people are not diagnosed until the cancer has reached an advanced stage. This highlights the importance of regular check-ups and screenings, especially for individuals at high risk, such as those with a family history or of Asian descent.

Diagnosis

Initial Evaluation
The diagnostic process typically begins with a physical examination by the doctor. The doctor will examine the nose, mouth, throat and neck, feeling for any abnormal lumps or swelling. Blood tests may be ordered to check general health and look for signs of EBV, which is associated with nasopharyngeal cancer.
Imaging Tests
Imaging scans like CT, MRI and PET-CT are used to look for tumors and check if cancer has spread. MRI scans provide detailed images of the nasopharynx and surrounding structures. PET-CT scans can help detect small areas of cancer and determine if it has spread to lymph nodes or other organs.
Endoscopic Exams
A nasendoscopy uses a thin, flexible tube with a light and camera to examine the nasopharynx. The patient is usually awake but local anesthetic can be used to numb the nose and throat. A panendoscopy uses a more rigid endoscope to examine the upper airway, including the mouth, nose, throat, voice box and top of the esophagus.
Biopsy
A biopsy involves removing a small tissue sample so it can be examined under a microscope for cancer cells. It is usually done during a panendoscopy using special instruments passed through the endoscope. The biopsy is the only way to definitively diagnose nasopharyngeal cancer.
Staging
Once nasopharyngeal cancer is confirmed, further tests are done to determine the stage. Staging describes the size of the tumor and how far cancer has spread. This helps guide treatment planning and provides information about prognosis.
Waiting for Results
Waiting for test results can be an anxious time for patients. It usually takes 1-2 weeks to get results. Patients should contact their doctor if they haven’t heard anything after a couple weeks. Support is available from specialist nurses, close friends/family, and cancer support organizations.

In summary, diagnosing nasopharyngeal cancer involves a combination of physical exams, imaging tests, endoscopic procedures, and biopsy. Staging provides crucial information to guide treatment and determine prognosis. Support is important for patients coping with the stress of diagnosis and waiting for results.

In this video, you can find how EBV DNA screening can help detect NPC. Source of video is NEJM.COM

Treatment

Radiation Therapy

Radiation therapy is the primary treatment for nasopharyngeal carcinoma.
Intensity-modulated radiation therapy (IMRT) is the most commonly used technique, allowing for precise targeting of the tumor while minimizing radiation exposure to healthy tissues.
For early-stage nasopharyngeal carcinoma (stage I), radiation therapy alone can produce a 5-year survival rate of about 85%

Chemoradiation

Concurrent chemoradiation, using chemotherapy concurrently with radiation therapy, is often used for more advanced stages (stages II-IV).
Multiple randomized phase III trials have shown the benefit of chemoradiation compared to radiation therapy alone.
Cisplatin is the most commonly used chemotherapy drug in combination with radiation therapy.

Induction Chemotherapy

Induction chemotherapy, given before chemoradiation, is another approach for advanced-stage nasopharyngeal carcinoma. Induction chemotherapy followed by chemoradiation has shown promising results in some studies.
Commonly used induction chemotherapy regimens include PF (cisplatin and 5-fluorouracil) and GP (gemcitabine and cisplatin).

Adjuvant Chemotherapy

Adjuvant chemotherapy, given after chemoradiation, is also being investigated for advanced-stage nasopharyngeal carcinoma.

The combination of chemoradiation and adjuvant chemotherapy has demonstrated improved outcomes compared to chemoradiation alone in some studies.

Targeted Therapy and Immunotherapy

New treatment options, such as targeted monoclonal antibodies and small molecule tyrosine kinase inhibitors, are being studied for nasopharyngeal carcinoma.
Checkpoint inhibitors, like nivolumab and ipilimumab, have shown promising antitumor effects in treating recurrent and metastatic nasopharyngeal carcinoma.

However, the use of these newer therapies is still limited, and more research is needed to define their role in the treatment of nasopharyngeal carcinoma. Despite the advances in treatment, treatment failures are still common, with approximately 20% of patients with locally advanced disease developing distant metastases. Additionally, about 10-20% of patients experience local recurrence or distant metastasis after initial treatment. These patients have a poor prognosis, with a median survival of only about 10-15 months.

Prognosis

Factors Affecting Prognosis:

Stage at diagnosis: Early-stage disease (stage I-II) has a more favorable prognosis compared to advanced-stage (stage III-IV).
Tumor extent: Gross extension of the primary tumor involving the facial bones and skull base is associated with worse outcomes.
Lymph node involvement: Cervical nodal metastases at diagnosis are common (47.4%) and negatively impact prognosis.
Histological type: Non-keratinizing carcinomas have a better prognosis than keratinizing squamous cell carcinomas.
Treatment approach: Aggressive multimodality treatment with chemoradiation has improved outcomes for advanced disease.
Gender: Female patients have a more favorable prognosis, with 5-year survival rates exceeding 80%.
Body mass index (BMI): Overweight at diagnosis indicates a favorable long-term prognosis, while a thinner body shape is associated with worse outcomes.
EBV DNA: Detectable EBV DNA levels after treatment are a strong negative prognostic marker.

Recurrence and Metastasis
After initial treatment, 10-20% of patients experience local recurrence or distant metastasis. Patients with recurrent or metastatic disease have a poor prognosis, with a median survival of only about 10-15 months.

Surveillance and Follow-up
Regular follow-ups with physical exams, nasal endoscopy, and EBV DNA testing are crucial for cancer surveillance. Frequent screening is recommended in the first 2 years post-treatment, every 3-6 months, and annually thereafter. Posttreatment PET-CT scans are often performed to assess response and detect recurrence.

In summary, the prognosis for nasopharyngeal carcinoma is influenced by multiple factors, with early-stage disease, female gender, higher BMI, and undetectable EBV DNA after treatment being favourable prognostic indicators. However, even with aggressive multimodality treatment, a significant proportion of patients develop recurrent or metastatic disease, highlighting the need for continued research to improve outcomes.

Patient’s Survivorship

Patients with nasopharyngeal carcinoma face several challenges during and after treatment that can impact their survival and quality of life. Here is a detailed overview of these challenges and how they can be managed:

Challenges During Treatment

Acute side effects of radiation therapy: Skin irritation, dry mouth, difficulty swallowing, and hearing loss.
Acute side effects of chemotherapy: Nausea, vomiting, fatigue, hair loss, and increased susceptibility to infections.
Difficulty tolerating the intensity and duration of treatment.
Logistical challenges, such as transportation to treatment centers and taking time off work.

These challenges can be managed through:

Proactive management of side effects with supportive care medications and interventions.
Nutritional support and counseling to maintain weight and nutrition status.
Psychosocial support and coping strategies to help patients tolerate treatment.
Coordination of care to address logistical barriers and ensure timely treatment delivery.

Long-Term Complications

Xerostomia (dry mouth) leading to dental problems and difficulty eating
Hearing loss
Cognitive issues and memory problems
Hypothyroidism
Increased risk of secondary cancers
Psychological distress and reduced quality of life

These long-term effects can be managed through:

Regular dental care and use of saliva substitutes for dry mouth
Hearing aids and assistive devices for hearing loss
Cognitive rehabilitation and memory strategies
Thyroid function monitoring and hormone replacement therapy
Screening for secondary cancers and early intervention
Psychosocial support, counseling, and support groups to address emotional challenges

Challenges After Treatment

Uncertainty about the future and fear of recurrence
Financial burden of ongoing care and lost productivity
Difficulty reintegrating into work and social life
Caregiver burden and strain on family relationships

These post-treatment challenges can be addressed through:

Regular follow-up care and surveillance for recurrence
Financial counseling and assistance programs to help manage costs
Vocational rehabilitation and support for returning to work
Family counseling and caregiver support programs
Support groups and peer-to-peer connections for patients and caregivers

Effective management of these challenges requires a multidisciplinary approach involving physicians, nurses, social workers, rehabilitation therapists, and mental health professionals. Regular communication, coordination of care, and patient education are key to helping patients with nasopharyngeal carcinoma navigate the challenges of treatment and survivorship.

Recommendations for Patients

Actively engage in your care – Ask questions and communicate openly with the healthcare team about concerns, symptoms, and treatment preferences. Attend all scheduled appointments and follow the prescribed treatment plan.

Utilize available support resources – Take advantage of patient support programs offered by the healthcare provider or pharmaceutical company. Engage with peer support groups and communities for emotional support and practical advice.

Prioritize Self-Care – Maintain a healthy lifestyle with proper nutrition, exercise, and stress management. Adhere to medication regimens and attend follow-up appointments. Communicate any side effects or difficulties with treatment to the healthcare team.

Advocate for your needs – Speak up about barriers to care, such as transportation, financial concerns, or language/cultural differences. Request accommodations or modifications to treatment plans if needed. Seek a second opinion if unsatisfied with the current treatment approach.

Maintain Open Communication – Keep the healthcare team informed about changes in symptoms, medications, or overall health status. Discuss any concerns or questions about treatment, side effects, or lifestyle changes. Provide feedback on the effectiveness of patient support resources and suggest areas for improvement.

Conclusion

While Nasopharyngeal cancer presents unique challenges, patients today have reason for hope. With early detection, advances in treatment, and a multidisciplinary approach to care, many patients are achieving excellent outcomes – for those diagnosed with localized disease, the 5-year survival rate is an encouraging 82%. By staying informed, adhering to treatment, managing side effects, seeking support, and maintaining a healthy lifestyle, patients can take an active role in their care and work towards the best possible outcomes.

Remember, you are not alone on this journey – you have a dedicated team of healthcare professionals and a community of support to help you every step of the way. With perseverance, resilience, and a positive outlook, patients with NPC can face the future with courage and optimism, knowing that every step forward brings them closer to a brighter tomorrow.