What is Hypopharyngeal Cancer?
Hypopharyngeal cancer is a type of cancer that occurs in the area between the back of the mouth (oropharynx) and the beginning of the food pipe (esophageal inlet). More specifically, it develops between the hyoid bone in your neck and the lower end of the cricoid cartilage, another structure in your neck. There are different types of this cancer based on where exactly they pop up within this region. The three main areas are the part right after the cricoid (the pharyngoesophageal junction), the piriform sinus, and the back part of the throat (posterior pharyngeal wall). But, it’s important to note that hypopharyngeal cancers are not the same as larynx (voice box) cancers, even though they are close in location.
Almost all (95%) hypopharyngeal cancers are a type called squamous cell carcinoma, which starts in the mucosal layer (the moist membrane lining certain parts of the body). The remaining cases are other cancer types such as adenocarcinoma, sarcoma, and non-epidermoid carcinoma. These cancers usually invade locally and spread to the nearby lymph nodes, meaning that at the time of diagnosis, about 70% of patients already have cancer spread to these nodes.
Symptoms of hypopharyngeal cancer depend on the size and location of the tumor. Pain, bleeding, and difficulty swallowing (dysphagia) are the most common signs patients notice. Some people will also have malnutrition, which can be a sign that the disease is more severe. Besides, more advanced tumors may reach the larynx, causing issues with the breathing and swallowing routes. The surgery that’s needed for these cases, which can involve removing parts or all of the larynx, can lead to significant changes in function (like speaking or swallowing).
About 3,000 people in the United States are diagnosed with hypopharyngeal cancer each year, making up around 7% of cancers that develop in the upper digestive and respiratory tracts. It is often diagnosed at a late stage, which can make the prognosis worse than for laryngeal cancer, which is more common. At diagnosis, between 50% to 70% of patients already have cancer spread to surrounding lymph nodes. The chance of survival decreases as the disease progresses, with early stage tumors (T1-T2) having a 60% chance of survival over 5 years, in contrast to larger ones (T3-T4) or with multiple nodes affected, in which the survival rate drops to less than 25%.
What Causes Hypopharyngeal Cancer?
Hypopharyngeal cancer, which mainly involves a kind of cancer called squamous cell carcinoma, shares similar causes with other cancers of the head and neck. Long-term use of alcohol and tobacco are known to increase the risk of this cancer. The risk is even higher when alcohol and tobacco are used at the same time. This type of cancer is most common in men over 50 years old who have used alcohol and tobacco for a long time.
Other things that can make a person more likely to get hypopharyngeal cancer include:
* Plummer-Vinson syndrome: This is a condition that causes iron deficiency anemia, trouble swallowing (specifically after the voice box), and fleshy lumps in the food pipe. This condition usually affects women before menopause. It significantly increases the risk of developing cancers after the voice box and is a known cause of hypopharyngeal cancers in women under 50 who have iron deficiency anemia.
* Exposure to asbestos: While it’s unclear to what extent asbestos contributes to hypopharyngeal cancers, it is generally considered a risk factor for several types of cancer.
* While human papillomavirus (HPV) is a known risk factor for oral cavity and throat cancer, it is not really thought to increase the risk of hypopharyngeal cancers.
Recurrent irritation from stomach acid reflux, as seen with esophageal cancer, is also known to contribute to tumor formation in the area after the voice box. This is similar to the process seen in Barrett’s esophagus, a condition in which the tube that carries food from your mouth to your stomach changes to a type of tissue that is similar to the intestine.
Chewing areca (also known as betel) nut, a common habit in South and Southeast Asia and East Africa, is also strongly associated with tumors in the hypopharynx.
Risk Factors and Frequency for Hypopharyngeal Cancer
Hypopharyngeal cancer, which is a type of throat cancer, is relatively rare. It represents about 80,000 new cases per year or 0.4% of all new cancers globally. It’s also responsible for approximately 35,000 cancer-related deaths per year, accounting for around 0.4% of all cancer deaths. The incidence rate is quite low at 0.8 cases per 100,000 people, with men being affected more than women. Notably, the areas where this type of cancer occurs the most are South-Central Asia, Central and Eastern Europe, Western Europe, and North America.
- South-Central Asia has the highest percentage of hypopharyngeal cancer compared to other head and neck cancers at 17.3%.
- North America reports this cancer to make up 7.1% of the total head and neck cancers.
- In Europe, it represents 11.3% to 12.8%
- In Latin America, the rate is 7.3%.
In the United States specifically, around 2,500 instances of this cancer are diagnosed each year, making up 3 to 5% of all head and neck cancers. The international variation in the occurrence of this disease can largely be put down to social habits such as alcohol consumption, tobacco smoking, and chewing substances that can cause cancer.
Signs and Symptoms of Hypopharyngeal Cancer
Hypopharyngeal cancers are a type of head and neck cancer that typically do not present symptoms until the tumor has grown considerably large. This is due to the fact that these tumors develop in areas that are less critical for space. Early symptoms of this type of cancer can include an uncomfortable sensation of a lump in the throat, difficulty or pain while swallowing, or even pain that is felt in the middle ear. Since this cancer tends to spread to the lymph nodes early on, the initial sign may also be a new lump found on the neck. When the tumor grows larger, eating can become difficult, with problems swallowing solid foods before liquids. Additionally, a change in the patient’s voice may occur if the tumor reaches the larynx or causes vocal cord paralysis.
When checking for head and neck cancers, doctors usually perform the same routine exam to systematically review all areas of concern. This includes examining the oral cavity, where a primary hypopharyngeal tumor will not be visible. However, the doctor might be able to see other cancers in the mouth or throat, or signs of the cancer spreading to the palatopharyngeus muscle, often indicated by a difference in the tonsil pillars. Advanced tumors can cause a build-up of secretions or saliva, which can also be observed orally. It is also important to examine the neck, given the cancer’s tendency to spread early to the lymph nodes. Doctors will pay special attention to the lymph nodes in specific areas of the neck.
One of the most informative tests for this type of cancer is often a fiber-optic examination of the hypopharynx and larynx. This can help detect tumors found in the pyriform sinus posterior pharyngeal or post-cricoid areas. Doctors would be looking for pooling of secretions or asymmetry in the larynx. Other signs of this cancer could include ulcerated or erythematous mucosal lesions, and hyperkeratosis. They will also examine the symmetry and motion of the vocal cords. Any tumor spread to the glossopharyngeal or vagus nerves could be spotted by examining the cranial nerves. Lastly, a full exam of the patient can reveal signs of the cancer spreading to distant locations or changes in the patient’s overall physical condition.
Testing for Hypopharyngeal Cancer
If your doctor suspects that you might have hypopharyngeal cancer, which is a type of cancer found in the lower part of the throat, they will most likely order a range of tests to help determine the best treatment option for you. These tests will not only confirm the presence of cancer but also provide valuable information on your overall health status.
The first of these are laboratory blood tests that examine different aspects of your health. This includes things like your blood count, which can reveal if you’re currently facing conditions such as anemia that could complicate your treatment. It also involves looking at how well your kidneys are working, as some cancer treatments impact their functioning. Other tests may be used to examine your liver and nutritional status, which are important to understand before starting cancer treatment.
In addition to blood tests, your doctor will utilize various imaging tests to examine the extent and spread of the cancer. A chest X-ray might be used to check for other health problems, lung cancers, or a simultaneous lung tumor – conditions often found in heavy smokers which is a common risk group for this type of cancer. Another test, called a Barium or Water-Soluble Contrast Swallow, can help detect tumors that aren’t easily seen with the standard fiber-optic examination.
Other scan techniques, like a CT scan with contrast, are useful to determine the location and spread of the cancer, assessing things like local spread and distant metastases. An MRI, specifically designed to look at the oral cavity and neck areas, provides a detailed view of how far the disease has spread in these regions. A PET scan may be used if the doctor wants to track any likely spread of the disease to distant parts of the body that haven’t been picked up by other scans. This can also help find the primary source of cancer in cases where it’s not immediately clear.
Lastly, your doctor might suggest a procedure called a panendoscopy, where, under general anesthesia, the doctor directly examines parts of your upper digestive tract. In addition to detecting hypopharyngeal cancer, this procedure can help find any other cancers that might have developed simultaneously, a situation that can occur in 10-15% of patients.
Treatment Options for Hypopharyngeal Cancer
Treating conditions like these often involves teamwork from different health professionals. These include surgeons who specialize in head and neck procedures, cancer doctors, radiation therapists, dietitians, speech therapists, and patient advocates. The goal is to control and get rid of the tumors while maintaining important functions like speaking, swallowing, and breathing as much as possible.
The treatment plan depends on the stage of the condition:
– For early-stage illnesses (T1/T2), therapy may involve either radiation therapy or conservative surgery, which may be followed by postoperative radiotherapy. Conservative surgery means surgically removing the tumor while preserving as much normal tissue as possible.
– For late-stage illnesses (T3/T4), tumors that can be removed are treated with either partial or complete laryngopharyngectomy, a surgery that involves removing part or all of the larynx and pharynx. This is often followed by chemotherapy and radiation therapy. If the tumor cannot be removed, the patient may receive either radiation treatment alone or combined with chemotherapy. Participation in clinical trials is also encouraged when possible.
Cancers within the pyriform sinus, an area in the throat, are treated differently depending on their location. Small tumors of the lateral wall can often be managed with surgery to remove part of the pharynx and thyroid cartilage. Medial wall tumors may also be suitable for larynx-sparing surgery, but the suitability is assessed based on certain criteria. However, for tumors spreading into the pyriform apex, posterior wall, or post-cricoid area, complete removal of the larynx and pharynx is required.
Tumors in the post-cricoid region are often more difficult to detect and are usually more advanced by the time of treatment, requiring more extensive surgery, reconstruction, and additional treatments. Considering the severity of these operations, the patient’s health status and ability to withstand surgery should be carefully assessed. In some cases, reconstructive flap surgery may be needed to replace the removed esophagus. Lastly, lymph nodes from specific areas of the neck might also need to be removed.
In cases of tumors on the posterior pharyngeal wall, which is at the back of the throat, treatment is decided based on how deep the tumor has spread. If it’s just on the surface, it can be removed and the area closed up. But if it’s spread deeper and invaded tissues in front of the spine, a slightly different surgery called a pharyngotomy may be necessary. However, the amount of tissue removed can lead to complications and even death while in hospital. The type of reconstruction after surgery also has a significant impact on outcomes. The in-hospital death rate can be as high as 11%, especially in cases where a gastric pull-up procedure is required to replace the esophagus.
As for advanced tumors that can be removed, chemoradiation therapy is often considered, and this is also the case for tumors that can’t be removed. This treatment involves giving chemotherapy to increase the effectiveness of radiation therapy. Research has proven that providing these two treatments together offers a significant survival advantage compared to giving radiation treatment alone. Commonly used drugs for this purpose include cisplatin and 5-fluorouracil.
What else can Hypopharyngeal Cancer be?
When a patient suddenly develops a lump in the neck, Doctors need to consider a wide range of possible causes which include:
- Enlarged lymph nodes due to various reasons like an acute infection
- Inflammatory conditions like sarcoidosis, which causes lumps of inflamed tissues in various organs
- Benign (non-cancerous) growths like a lipoma (fatty lump)
- An infection or flare-up of a birth-given lump like a branchial cyst that hasn’t been noticed before
Besides these causes, other cancers can also produce similar symptoms, like a lump in the neck and difficulty swallowing. These include:
- Lymphoma (cancer of the lymphatic system)
- High esophageal cancer (cancer located in the upper part of the swallowing tube)
- Other types of head and neck cancers like squamous cell carcinoma of the base of the tongue, tonsil, or other parts of the back of the throat
What to expect with Hypopharyngeal Cancer
Cancers of the hypopharynx, or the lower part of the throat, have a worse survival rate compared to other cancers in the head and neck. This is due to the fact they are often diagnosed late and spread quickly to the lymph nodes. For patients who are diagnosed early and do not have any lymph node involvement, the 5-year survival rate can be as high as 70%. However, as these cancers are not often detected soon enough, the average 5-year survival rate for all hypopharynx cancers can be as low as 20%.
The presentation of lymph node involvement varies depending on the stage of the tumor:
* Stage T1 – 60%
* Stage T2 – 65 to 70%
* Stage T3 – 84%
* Stage T4 – 85%
Tumors located in the post-cricoid region (the area below the cartilage in the throat) and pyriform apex (the top of the pear-shaped hollow in the throat) have a worse survival rate than tumors at other locations. As expected, larger tumor size or cross-sectional radiological area is associated with worse outcomes.
Receiving radiation therapy after surgery has been shown to improve overall survival rate, as compared to surgery alone. A study from the United States showed the following 5-year survival rates based on different stages: Stage I had a survival rate of 63%, Stage II of 57.5%, Stage III of 42%, and Stage IV of 22%.
Possible Complications When Diagnosed with Hypopharyngeal Cancer
The treatment of hypopharyngeal cancer can have various complications, leading to significant health impacts.
Surgical Complications
Elements of the surgery can cause complications during the procedure and just after it’s over. These include heavy bleeding, injury to nerves that could lead to conditions like droopy shoulder, difficulty in tongue movement, and paralysis of the diaphragm. If the thyroid and parathyroids are removed, it can lead to low calcium and thyroid levels. Problems with grafts in the first week of surgery might require additional operations. Pharyngo-cutaneous fistulae – unusual connections between the pharynx and the skin – can happen frequently with larger resections or postoperative radiotherapy. The surgery can also make swallowing difficult in the long term, causing potential lung infections if the larynx was kept and leaking into the laryngectomy stoma if it wasn’t. Longer-term complications may require procedures to dilate a narrow pharynx or manage long-term fistulae formation.
Radiotherapy Complications
Although modern techniques of giving radiotherapy are more targeted and result in fewer complications, the process can still have immediate risks. These include painful mouth inflammation (mucositis) and loss of salivary gland function leading to symptoms like a dry mouth, taste disturbances, further inflammation, and skin inflammation. To avoid malnutrition and the risk of inhaling foods or liquids into the lungs (aspiration), patients often need a feeding tube inserted before the procedure. Later complications from radiotherapy might involve difficulty swallowing long-term, and potential harm to surrounding structures, causing conditions such as osteoradionecrosis of the jawbone or backbones, spinal cord injuries, and damage to the nerves of the arm.
Chemotherapy Complications
Chemotherapy complications can be severe and life-threatening, and typically last as long as the treatment does. These may include weakened immunity, thus increasing the risk of bacterial infection in the bloodstream. Other complications can involve hair loss, peripheral neuropathy – a nerve condition causing weakness, numbness, and pain usually in your hands and feet, heavy tiredness, nausea, vomiting, and severe injuries due to chemical leaking into the tissues. Chemotherapy can also amplify the immediate complications of radiotherapy, such as mouth inflammation.
Preventing Hypopharyngeal Cancer
Hypopharyngeal cancer, a type of throat cancer, is largely linked with lifestyle habits such as heavy drinking and using tobacco, both in chewed and smoked forms. Campaigns that aim to raise awareness about the harmful effects of alcohol and tobacco use are part of why we now see more cases of this type of cancer in developing countries worldwide, and less so in more financially developed regions.
In South and Southeast Asia and East Africa, efforts to decrease the use of areca nuts (a type of palm nut often chewed like tobacco) have been successful in reducing occurrences of various head and neck cancers, including those in the hypopharynx (the lower part of the throat).
