Causes of Hypopharyngeal Cancer

As with most cancers, doctors can’t tell you with certainty what causes hypopharyngeal cancer. It’s a combination of genetic factors and factors in your environment.

By far the most common factor contributing to hypopharyngeal cancer is using tobacco, particularly smoking it. Drinking excessive amounts of alcohol also contributes to the risk of developing cancer of the hypopharynx.

• Tobacco:Smoking cigarettes, cigars or pipes and using chewing tobacco greatly increase your chance of getting a hypopharynx cancer.
• Alcohol:Drinking excessive amounts of alcohol is also very strongly related to getting hypopharyngeal cancer. Moreover, if you both smoke and drink heavily, the risk more than doubles.

Other factors that can increase your chance of getting hypopharyngeal cancer include:

• Exposure to radiation in the past:Being exposed to radiation as part of a natural disaster, treatment for another disease a long time ago or even through work can increase the chances of some hypopharynx cancers.
• Plummer-Vinson Syndrome:This is a very rare disease seen in non-smoking women between 30 and 50 years old. It is called a syndrome because it includes a pattern of symptoms, including difficulty with swallowing, a web of tissue that can partially block off the hypopharynx and low iron counts leading to low blood counts, along with weight loss.

Other factors that can increase the risk of developing cancer of the hypopharynx include:

• A history of drinking poisons such as lye
• Certain viruses
• Asbestos exposure
• Genetic factors

Signs and Symptoms of Hypopharyngeal Cancer

In many cases, hypopharyngeal cancers get quite large before patients become aware of symptoms.

The most common symptom is increasing difficulty with swallowing, but many other symptoms can also be present:

• Pain or difficulty with swallowing in the throat:This can occur because a tumour is in the way of swallowing, and so it becomes difficult or painful to swallow. Also, there can be ulceration and bleeding as the tumour grows, causing pain.
• A lump in the neck:This will be a symptom of hypopharynx cancer if it has spread to lymph nodes in the neck. This can be the first symptom that brings a patient to the doctor. If you have a neck mass, and your doctor is concerned that it represents cancer that has spread from somewhere else, one of the first places he or she will look is your hypopharynx.
• Ear pain (particularly on one side, with no other ear problems):Ear pain, also known as otalgia, happens because the nerves of the throat reach the brain through the same pathway as one of the nerves in the ear. Therefore, your brain might interpret a pain in the throat as coming from the ear. This is called referred pain. Consequently, unexplained ear pain that doesn’t go away should be evaluated by a specialist. It is important to understand that most causes of ear pain are due to simple problems such as middle ear infection or dysfunction of the Eustachian tube. TMJ pain due to a problem in the joint located in front of the ear may also present as otalgia.

Other possible symptoms might include:

• A hoarse voice
• Weight loss
• Feeling like there’s something stuck in your throat
• Bleeding from the mouth
• Coughing every time, you drink liquids
• Difficult or noisy breathing

But don’t jump to any conclusions. You could have one or more of these symptoms but NOT have hypopharyngeal cancer. There are several non-cancerous causes of the same symptoms. That’s why you need to see a specialist.

What to Expect at Your Doctor’s Visit?
 Step 1: History
First, your head and neck specialist will take a thorough history of your health and address any specific concerns you may have.
Your doctor might ask questions such as:

• How long has the problem been there?
• Is it getting worse, better or staying the same?
• Does it come and go?
• Have you tried anything to make it better?
• Is it painful?
• Do you have numbness or tingling anywhere in your face or mouth?
• Do you have any lumps or bumps in your neck?
• Are you losing weight?
• Do you have any other medical conditions?
• Have you had any surgeries in the past?
• What medications do you take? And do you have any allergies?
• Have you ever been exposed to radiation in the head and neck?
• What do you (or did you) do for a living?
• Do you have a family history of cancer?

 Step 2: Physical Exam
Next, your doctor will examine you. Typically, if you’re seeing a specialist in head and neck disorders, you will get a thorough physical examination focused on the area of concern.
You should also expect the doctor to:

• Feel your neck to carefully check for any lumps or bumps
• Look inside your ears
• Look inside the front of your nose
• Check your cranial nerves by asking you to move your face, stick your tongue out, lift your shoulders, follow his or her fingers around with your eyes, do some simple hearing tests and test your sense of touch all over your face

A few special diagnostic tests might be required as part of your physical exam.
 Pharyngo-laryngoscopy (looking at your oropharynx, hypopharynx and larynx): This can be done in a few ways, including with a headlight and mirror placed on the roof of your mouth to look down or with a flexible camera placed through your nose. Don’t be surprised if you are required to puff out your cheeks while the camera is in; this opens up the hypopharynx and will allow your doctor to get a better view.
Flexible pharyngo-laryngoscopy: Your doctor may spray your nose with some medications and then slowly and carefully place a flexible tube with a camera through your nose down into your throat. Just sit still, breathe slowly and listen to your doctor’s instructions.
Indirect mirror examination: Your doctor will distract you while placing a small mirror into the back of your throat through your mouth. The doctor will ask you to breathe through your mouth and make sounds as he or she examines your throat.
Indirect Transnasal Flexible Endoscopy (Laryngoscopy)
 Your doctor may spray your nose with some medications and then slowly and carefully place a flexible tube with a camera through your nose down into your throat. Just sit still, breathe slowly and listen to your doctor’s instructions.
Indirect Mirror Examination (Laryngoscopy)
 Your doctor will distract you while placing a small mirror into the back of your throat through your mouth. The doctor will ask you to breathe through your mouth and make sounds as he or she examines your throat.
 Step 3: Reviewing Tests
After getting your history and performing a physical exam, your doctor will review any imaging, laboratory work and pathology results you may have already had. Be sure to bring all of these with you to your appointment. Bring actual discs of any scans you’ve had, as well as any reports of those scans. If you are seeing a head and neck cancer specialist after a lesion was removed by a non-cancer specialist, you need a thorough review of the pathology to discuss whether additional treatment is necessary. Try to obtain the actual glass slides that were prepared by the pathologist with the specimen taken during your biopsy procedure so your doctor can conduct a complete review. You might need more tissue removed or further treatment.
Step 4: Recommendations
Finally, your doctor will make recommendations about your next steps. This will likely include reviewing some of the studies you’ve already had done or ordering more tests. Once your doctor has all the necessary information, you should be given a preliminary stage and discuss treatment plans.
If the tumour is very large and is putting your breathing at risk, your doctor might recommend you undergo a tracheotomy, which is a breathing tube placed into the front part of your neck directly into your windpipe.
Also, if you are just not able to get enough nutrition by mouth because of the tumour, your doctor might recommend that you receive a feeding tube. This will help make sure you are in good shape to undergo the treatment that you will need to beat the cancer. If you have lost weight, your doctor may give you a choice of getting enough nutrition by mouth by increasing the number of calories in your diet or by undergoing placement of a feeding tube. There are a variety of nutritional supplements that you can eat or drink that can help to achieve that goal. You may want to meet with a nutrition expert early during your treatment. Note that for esophageal cancers, your doctor will probably recommend a special type of feeding tube called a J-tube (or jejunal feeding tube). This is different from a G-tube (gastric tube). This is important because if surgical resection is a possible treatment for your cancer, sometimes your stomach is used as a new esophagus, and it is better if the stomach doesn’t have a feeding tube in it. The J-tube is placed in the jejunum, which is the organ further down the GI tract from the stomach.

Determining the Type of Hypopharyngeal Cancer

Only after a pathologist analyses some cells or actual pieces of tissue from the lesion will your doctor be able to tell you if you have cancer. Your doctor and pathologist should specialise in head and neck cancers because some benign (non-cancerous) lesions can look like cancer on a small biopsy.

Squamous cell carcinoma: These are by far the most common hypopharyngeal cancers (95 percent). They arise from cells lining the hypopharynx.

Squamous cell cancers of the hypopharynx are typically given a grade by a pathologist after looking at the cells under a microscope. Grade means that the tumour falls on a scale from well differentiated (Grade I) to poorly differentiated (Grade IV). It is generally felt that the prognosis for a more well-differentiated cancer is more favourable.

More rarely, other cancers can be found in the hypopharynx as well. Some of them include:

Salivary gland cancers: There are minor salivary glands located under the lining of the throat. Therefore, cancers that we typically see in salivary glands can arise in this region. They include diagnoses such as mucoepidermoid carcinomas, adenocarcinomas and adenoid cystic carcinomas, to name a few. See Salivary Gland Cancer for more information.

 Lymphoma: Lymphoma is cancer of the lymphatic system, which includes lymph nodes, lymph channels, lymphatic fluid and lymphoid tissue. The throat is lined with lymphoid cells. Some major sites of lymphoid tissue include the adenoids in the nasopharynx and palatine tonsils and lingual tonsils in the oropharynx. Therefore, lymphoma might appear as a lump in the throat area.

Mucosal melanoma: These cancers come from skin cells that give skin its colour. In rare cases, melanoma can be found in the lining of the mouth, nose and/or throat.

Other extremely rare cancers of the hypopharynx include:

• Sarcomas such as chondsarcoma, liposarcoma and synovial sarcoma
• Malignant fibrous histiocytoma
• Peripheral Neuroectodermal Tumour (PNET)
• Cancer spread from another site

Determining the Grade of the Tumour

Pathologists will typically report on the grade of the tumour. This is a qualitative interpretation by the pathologist of how much the cancerous cells resemble normal tissue from that site. There are several different grading systems that might be used. The most common are as follows:

• GX:Grade cannot be assessed
• G1:Well differentiated
• G2:Moderately differentiated
• G3:Poorly differentiated
• G4:Undifferentiated

Differentiation refers to how closely the cells taken from a tumour or lesion resemble normal cells from the healthy tissue surrounding the tumour. “Well differentiated” means that the cells look like normal cells in that area. “Undifferentiated” means the cells look nothing like normal cells in that area.

While it is important to report the tumour’s grade, few doctors use this information to make decisions regarding treatment or prognosis for this cancer type.

Determining the Stage of the Cancer The final step before discussing treatment options is a determination of the stage of the cancer. Like with all cancers of the head and neck, in Australia it is mandatory for doctors to use the AJCC Cancer Staging Manual (7th Ed) to determine the stage based on three factors.  

At first, you will be given a clinical stage based on all the available information.  Clinical staging (cTNM) is determined from any information your doctor might have about how extensive the cancer is BEFORE starting any treatment. Stage is determined based on your doctor’s physical exam, imaging studies, laboratory work and biopsies. Classification of clinical stage is described using the lowercase prefix c (e.g., cT, cN, cM). If there is surgical removal of the cancer as part of your treatment, a pathologist will analyse the tumour and any lymph nodes that may have been removed. You will then be assigned a pathologic stage. Pathologic staging (pTNM) provides more data. Classification of pathology stage is described using the lowercase prefix p (e.g., pT, pN, pM). This may or may not differ from the clinical stage. There are also several other lowercase prefixes that might be used in the staging of your cancer.

• The subscript y(yTNM) is used to assign a cancer stage after some sort of medical, systemic or radiation treatment is given (Posttherapy or Postneoadjuvant Stage). It is typically combined with either a clinical or pathologic stage. For example, ycT2N0M0 indicates that after some sort of non-surgical therapy, the new clinical stage is T2N0M0.
• The subscript r(rTNM) is used when the tumour has recurred after some period in which it was gone. This is called Retreatment Classification Stage. Your doctor will use all the available information to assign you a retreatment stage.

 T stage: the main tumour mass Based on a physical examination and review of any imaging, your doctor should be able to give you a T stage that falls within one of the following categories.  

 N stage: spread of cancer to the lymph nodes in the neck Next, your doctor will use all the available information and assign you an N stage. This is based on the assessment as to whether the cancer has spread to lymph nodes in the neck.  

 M stage: spread of cancer outside the head and neck Finally, based on an assessment on the entire body, you will be assigned an M stage.  

 Your cancer stage After TNM staging, your doctor can assign a cancer stage based on the following chart.  

 Your clinical stage Once the diagnostic tests are completed, before deciding what type of treatment you are going to undergo, you should be given a clinical stage that will look like the example below.  

 * The lowercase subscript c indicates that this is a CLINICAL STAGE, the stage assigned based on all information available to your doctor before starting treatment. After surgery, you should get a pathologic stage of your tumour. It will look almost like the clinical stage you received before starting treatment, but notice the “p” that indicates the stage group is based on an analysis of the entire tumour, with or without lymph nodes, under a microscope by a pathologist. In many cases, the pathologic stage will be the same as the clinical stage, but sometimes it will change. After surgery, and after the pathologist has evaluated all of the tumour that was removed, you should be given a pathologic stage that looks something like this:  

 The lowercase subscript p indicates that this is a PATHOLOGIC STAGE, the stage assigned after tumour removal and confirmation of cancer by a pathologist.  Note also that the M stage is usually clinical, based on all available data without actually analysing any tissue.

Deciding on a Treatment Plan 

Before starting treatment, your doctor will make sure that the following steps are completed:

 Pre-treatment Evaluation

A full history and physical examination, including a complete head and neck exam (mirror and fibreoptic exam if needed)

• An evaluation by the members of a head and neck cancer team
• A biopsy of the primary site or FNA of the neck to confirm a diagnosis of cancer
• Imaging of the lungs to check for spread, if needed
• Imaging of the primary tumour and the neck with CT and/or MRI
• Maybe a PET-CT for advanced cancers
• Examination under anaesthesia with endoscopy
• A dental evaluation
• Nutrition, speech, and swallowing evaluation and maybe even a hearing evaluation
• Pulmonary function tests may be considered if a candidate for conservation surgery
• Pre-treatment medical clearance and evaluation of the risks of anaesthesia

Then your doctor will recommend a course of treatment for you, depending on several factors.

As with all cancers in the head and neck, there are three general options to consider:

• Surgical removal(with or without reconstruction)
• Radiation(a few different types)
• Medications(chemotherapy and biologic medications)

For hypopharynx cancer, there is not one clear treatment method. You should have an extensive discussion with your cancer team to decide upon the best treatment course for you personally. The options that your doctor will recommend will likely be as follows, based on the stage of cancer that you have:

Determining Your Prognosis

Your prognosis is a prediction of the outcome of your disease. What is the risk of succumbing to the cancer or the risk of it coming back? These are the big questions on most people’s minds after receiving a diagnosis of throat cancer. Prognosis is based on many factors, and a survival rate is an estimate based on large populations of patients who have been given a similar stage of their throat cancer. There are many specific factors that are unique to each patient that may influence treatment success.

The following aspects of the cancer may affect your prognosis.

It is very difficult to discuss prognosis without understanding all the details of your cancer, and this is a conversation you’re better off having in person with your doctor.

What to Expect After Treatment is Completed?

Once you have made it through treatment, you need to have close follow-up with your doctor. This follow-up plan is recommended after being treated for a throat cancer:

• Visit your head and neck specialist on a regular schedule (or earlier if you have any concerning symptoms). This allows your doctor to examine you for any signs that the cancer has come back.
  • For the first year, you should go every one to three months.
  • For the second year, you should go every two to six months.
  • For the third to fifth year, you should go every four to eight months.
  • After five years, you can start going every year.
• Your doctor should select a scan to be performed in the first six months after treatment. The first scan serves as a “baseline” study for the purpose of comparing future studies. This will depend on the type, stage and location of your cancer. Imaging may include CT scans, MRI scans and PET scans. If something suspicious comes up, you might need another biopsy.
• Consider chest imaging to check for any signs of lung cancer if you have an extensive smoking history.
• Check your thyroid function every six to twelve months if you have had radiation to the neck area.
• Get help with a therapist as needed for difficulties with speaking, hearing and swallowing.
• See a specialist about appropriate nutrition and diet.
• Alert your doctor if you experience any signs of depression.
• Stop smoking and stop drinking.
• See a dentist.