What is Esophageal Cancer?

Esophageal cancer is cancer that grows in the esophagus, the muscular tube going from the back of your mouth to your stomach. When you eat or drink, food goes down the esophagus when you swallow, moving from your mouth to your stomach. Esophageal cancer begins with the mutation of the cells that line the esophagus. There are two major types of esophageal cancer; these two kinds account for around 90% of all cases. These two types are esophageal squamous cell carcinoma and esophageal adenocarcinoma.

There are other cancers that more rarely affect the esophagus including lymphoma, malignant melanoma, small cell cancer, sarcoma, and choriocarcinoma. All of these other kinds combined account for about one-tenth of the cases of esophageal cancer.

Squamous cell carcinoma is so-called because it starts in the squamous cells. Squamous cells are the thin, flat cells that make up the outer layer of skin, the epithelium, and it covers our body’s surface inside and out. SCC can begin anywhere along the esophagus but is most often found in the upper and middle parts of the esophagus.

Adenocarcinoma begins in the gland cells, the cells that make and excrete mucous, in the esophagus. This cancer most often arises in the lower part of the esophagus, closer to the stomach than to the mouth, and is believed to be related to prolonged acid exposure to the cells, as in cases of gastroesophageal reflux disease (GERD).

History

Compared to other kinds of cancers, esophageal cancer is rare in the United States. Only about 1% of new cancer cases in the US are esophageal cancer. There were an estimated 16,910 cases in the United States in 2016. While it is not an incredibly common cancer in the US, it is the eighth most common cancer worldwide, and is the sixth or seventh most common cause of cancer-related deaths, causing 400,000 deaths throughout the world in 2012.

The prognosis (chance of survival) of cancer is affected by how far cancer has spread in the body, whether it can be removed by surgery, how it responds to available treatments, and the state of the overall health of the patient. Because the symptoms for esophageal cancer don’t show up until it is far advanced, it is often not caught until it has spread in the body.

When the cancer is not detected until it is already in the late-stage, the prognosis is very poor. 5-year survival rates are 5-30%, with most patients dying in the first year or two. The current treatments are far more effective than the treatments that were available forty years ago.

In 1980, the 5-year survival rate in the United States was only 4-6%; 30% of patients who had surgery died during surgery. Much more recently, about 18.4% of patients in the United States reach 5-year survival. This is still a rather low survival rate, but research is ongoing. If the cancer is found early, survival rates can be as high as 40%.

If cancer has metastasized, survival rates can be as low as 0-4%. The patient’s treatment team may be able to locate clinical trials going on and see whether the patient can participate. A clinical trial is a new treatment that made it through the first stages of research and is being tested for effectiveness on human patients now. Esophageal cancer is going through a significant change. In the 1970s, squamous cell carcinoma was the most common type of esophageal cancer in the United States and Western Europe, making up nearly 90% of cases.

That has been changing slowly, and adenocarcinoma is now the most common form of esophageal cancer in those regions, accounting for almost 70% of cases. The cause of this shift is not known. In general, ESCC is the most common type of esophageal cancer in the developing world, and adenocarcinoma is the most common type of esophageal cancer in the developed world.

Risk Factors

Esophageal cancers occur five times more frequently in men than in women and occur most often in older people. Esophageal cancer is 20 times more likely to be found in someone who is over 65.

There are some genetic and chromosomal abnormalities that can increase the risk of esophageal cancer, as well as some medical conditions. Tobacco use is a risk factor for both main types of esophageal cancer, though it is more strongly linked to squamous cell carcinoma. The two major forms of esophageal cancer also have some separate risk factors.

Risk factors more likely to increases the chances of adenocarcinoma include Gastroesophageal Reflux Disease, obesity (which increases the chances of GERD), and a condition called Barrett’s Esophagus, which is caused by long-term GERD. In Barrett’s Esophagus, exposure to stomach acid causes changes in the kind of cells in the lower esophagus from normal esophageal cells to the kind of cells more often found in the intestine.

These can develop into precancerous cells, which can then grow into cancer. Adenocarcinoma is more strongly male-linked than SCC, occurring seven to ten times more often in men; when it does occur in women, it occurs on an average twenty years later in life. There are suggestions that hormonal factors can provide some protection from esophageal cancer.

Obesity is strongly linked with this kind of cancer, with one of the strongest links that have been found between obesity and cancer. Interestingly, the bacteria that causes ulcers, H. Pylori, is NOT linked with adenocarcinoma, and may even provide some protection from it. If a patient has had radiation therapy for some other condition in the chest, it may increase his chances of adenocarcinoma.

Squamous cell carcinoma is more likely to be related to lifestyle factors, like excessive alcohol use, being habitually in environments or situations that cause irritation and inflammation of the lining of the esophagus (called the esophageal mucosa), drinking a caustic substance, habitually drinking very, very hot drinks, and poor oral hygiene.

A poor diet – lots of processed foods, and few fresh foods – can increase your risk of squamous cell carcinoma, as can general nutritional deficiencies. Eating a diet with a lot of cruciferous vegetables (cabbage, broccoli) and yellow and green vegetables can provide some protection.

SCC is also linked to a variety of other medical conditions like esophageal diverticula (small pockets of stretched tissue forming a pocket or pockets along the esophagus), unusual fungal infections, achalasia (which weakens the muscle response of the esophagus), and tylosis (a rare condition that greatly increases the lifetime risk of SCC).

There is a possible link being explored between squamous cell carcinoma and HPV (human papillomavirus).

Screening

Screening is the process of checking to see if someone may have cancer before there are any symptoms.

There has been researching trying to find an effective way to screen for esophageal cancer because the earlier cancer is caught, the more likely it is to respond to treatment; however, there is not yet a simple test.

Esophageal cancer is not usually screened for currently, because there isn’t a people group that can be identified as particularly high-risk, and the primary tests used to locate it (endoscopy and biopsy) are very invasive.

If a patient has significant GERD or had developed Barrett’s Esophagus, he or she may want to talk to a doctor about the possible advantages or disadvantages of regular screening for them.

Since drinking in excess and smoking (or other use of tobacco products) are linked to esophageal cancer, to lower risk, moderate alcohol use and avoid tobacco. If a patient has GERD, follow the doctor’s directions to control it. A patient with GERD may need an endoscopy to check for Barrett’s Esophagus, which has a strong enough association with esophageal cancer that it is considered a precancerous condition.

If it is present, Barrett’s esophagus needs to be monitored to see if the cell damage progresses, and the patient may receive cell ablation (destruction of abnormal tissue).

Symptoms

Esophageal cancer is usually not detected until the tumors are large enough to affect swallowing; thus the first sign that most patients have that something is wrong is dysphagia. Dysphagia can be summarized as difficulty swallowing, but it is more than just that.

It includes pain while swallowing, a feeling of food being stuck in the throat or chest, drooling, and gagging or coughing while swallowing. This can get bad enough that the person can’t swallow at all, or vomits. The patient may start avoiding certain foods because they are hard to eat and eating less. It can get severe enough that even liquids are avoided. Patients often have unexplained weight loss; this weight loss is linked with worse outcomes for the cancer patient. They may have a persistent cough.

By the time dysphagia occurs, the tumor is large enough to narrow the tube of the esophagus, and the cancer is well advanced and possibly has spread. Because adenocarcinoma is strongly linked to GERD, indigestion, heartburn, and symptoms of GERD are also very common. The patient may have hoarseness, caused by stomach acid damaging the vocal cords. There may also be difficulty speaking because the tumor has grown large enough to affect the laryngeal nerve, which helps control the vocal cords. The surface of the tumor may be fragile and bleed. This can cause the patient to vomit blood, and when the blood goes the other direction and passes through the digestive system it can come out as black, tarry stools. The bleeding may lead to low red blood cell counts and iron-deficiency anemia, causing weakness.

Sometimes bleeding starts slowly, and sometimes it happens suddenly. Because esophageal cancer is usually detected very late, other symptoms a patient may have related to how cancer has grown in that person’s body. If it has metastasized, there are additional symptoms based on where the extra tumors are.

Esophageal cancer often spreads to the heart, stomach, liver, bones, and lymph nodes.

For instance, the patient can have a greater risk of aspiration pneumonia – pneumonia from substances gone into the lungs – because if the tumor has spread from the esophagus to the nearby trachea (windpipe) a fistula (opening) can form between the two and then things swallowed may end up in the lungs and not the stomach.

If it spreads to the liver, there can be jaundice; if to the lungs, fluid retention around the lungs leading to difficulty breathing.

Diagnosis and Stages

There are usually no symptoms of esophageal cancer is until it is causing problems with swallowing.

Because dysphagia is linked with serious medical conditions like esophageal cancer, if it occurs, it should be checked out by a doctor immediately.

When you go to the doctor, the first step will be a physical and taking a health history, checking for the current state of health, any ongoing issues, and checking for any unusual lumps.

However, the esophagus is deep inside the chest, so esophageal cancer is most likely not detectable externally; any lumps related to esophageal cancer detectable externally are probably metastases. After a medical history and symptoms are evaluated, the tests involved checking the patient internally for tumors. There may be a chest x-ray. There may be a test called a barium swallow, where the patient drinks a liquid that contains a metallic substance called barium, which lines the throat and stomach.

Then they take a series of x-rays called an upper GI series, focusing on the esophagus and stomach. This can show whether there is a narrowing or blockage of the esophagus, but is not used as often now due to the development of better tools. The best diagnostic tool is actually an examination with an endoscope.

This examination of the esophagus with an endoscope is called an esophagogastroduodenoscopy (and can be referred to by several other names, such as esophagoscopy, or simply an endoscopy). The patient is sedated, and the endoscope is threaded through the nose or mouth and into the esophagus. An endoscope is a small tube that includes a light and a small camera lens. The technician or doctor guides the tube through the body to look at what is going on in the esophagus and upper stomach. There will also also be a small tool attached to the endoscope to collect tissue samples of any suspicious-looking areas for biopsy. The samples that are taken are looked at under a microscope to see whether there are malignant cells present. If cancer is found, the patient will need to have more tests done.

A computed tomography (CT) scan of the chest and abdomen will be used to look for signs cancer has spread to other parts of the body, such as lymph nodes or other organs. Positron emission tomography (PET) scans combined with CT scans are more precise than CT scans alone. CT scans are less stressful on the patient – they take about 5 minutes, use x-rays, and don’t require anything to be injected into the patient.

CT scans show clear 2-D images of bones and can show abnormalities in soft tissues. A CT scan is sensitive down to things about a centimeter large.

PET scans take over half an hour, not counting setup time, require a radiotracer (a very small amount of a radioactive substance) to be injected into the patient, and show 3-D images of biological processes. It is possible to combine these two images to correlate the data and pinpoint what is going on in the patient’s body with a very high degree of accuracy. Endoscopic ultrasonography uses sound waves to take a picture of the digestive tract. It involves a small ultrasound probe being attached to an endoscope and sent down the patient’s esophagus.

Endoscopic ultrasonography is best able to see how deep the tumor goes and whether it is in the lymph nodes that line the esophagus too. Depending on how far it appears cancer has spread, there may be other scopes done to look for cancer. Bronchoscopy is when a scope is sent into the lungs to look for cancer.

Laparoscopy is when the abdomen is checked with a scope; it involves making a small incision through which to thread the scope, as the abdomen does not have any openings. Thoracoscopy similarly checks out the chest cavity. Then the medical team needs to find what stage the cancer is in; this makes a huge difference in how to go about treatment. Around half of patients are inoperable and/or have metastatic disease at diagnosis.

The doctors will look at the primary tumor, at nearby lymph nodes, at spread further in the body, and at how these things combine in this particular patient. This is referred to as T (tumor) N (lymph nodes) M (metastases) staging. Each of those elements will be evaluated for severity in order to determine how advanced the cancer is.

T is scaled from TX (unable to be evaluated) to T4 – the higher the number, the larger and deeper the tumor.

N is scaled from NX (regional lymph nodes unable to be evaluated) to N0 to N3.

N0 means there is no cancer in the lymph nodes; N3 means it has spread throughout the lymph nodes.

M is scaled from M0 to M1 – metastases absent, or present in more distant parts of the body.

These elements are combined to give a stage, as seen below.

  • Stage IA: T1, N0, M0 – the tumor is entirely in the esophagus and is not large.
  • Stage IB: T2, N0, M0 – larger tumor, still contained
  • Stage IIA: T3, N0, M0 – large tumor, contained
  • Stage IIB: T1, T2, N1, M0 – small tumor, but some lymph node involvement
  • Stage IIIA: T4a, N0, M0 – very large, deep tumor, but still operable
  •  T3, N1, M0 – large tumor, a little lymph node involvement
  • T1, T2, N2, M0 – smallish tumor, but more lymph nodes involved
  • Stage IIIB: T3, N2, M0 – larger tumor, more lymph nodes involved
  • Stage IIIC: T4a, N1, N2, M0 – very large tumor, lymph node involvement
  • T4b, any N, M0 – very large tumor, inoperable, with lymph node involvement
  •  Any T, N3, M0 – 7 or more local lymph nodes involved
  • Stage IV: any T, any N, M1 – Cancer has spread very far in the body.

Stage 1 has the best prognosis.

Stage 4 has the poorest prognosis, and treatments may focus on palliative care (reducing pain and discomfort) if cancer has reached Stage IV.

Treatments and Drugs

Treatments include surgery, radiation, and chemotherapy. Radiation and chemotherapy are often done before the surgery so that they have a chance to shrink the tumors.

Surgery may be put off for a month to six weeks after diagnosis to give time for the radiation and chemotherapy to be administered. Post-surgery radiation and chemotherapy are also sometimes given to try to ward off a recurrence.

Chemotherapy and radiation may also be given if surgery is not possible. The patient and treatment team also need to consider the patient’s quality of life when making a treatment plan.

Radiation involves focusing on specific kinds of concentrated energy rays tightly on the tumors to kill cancer cells. It may be done externally, using a machine that aims beams of radiation at the tumors (external beam radiation), or it may be done by placing radioactive implants near the tumor (brachytherapy).

Chemotherapy involves giving strong drugs to destroy cancer cells. The kind of chemotherapy drug used depends on the kind of cancer that is involved. Some drugs that are often used include carboplatin and paclitaxel, cisplatin and 5-fluorouracil ECF: epirubicin, and cisplatin. Patients will often receive a mixture of different chemotherapy drugs to increase the overall effectiveness of the treatment. Chemotherapy alone rarely cures esophageal cancer; used together with radiation, it can improve outcomes.

Surgery removes as much cancer as possible. There are different ways to do surgery, depending on what is going on in that patient’s body. If the cancer is caught early and is completely confined to the walls of the esophagus, with no spread anywhere else in the body, it may be possible to do an endoscopic surgery. If it is not spread far, it may be possible to do a laparoscopic surgery (done with small tools through tiny incisions).

It may also require larger incisions in an open procedure if the surgery is more extensive. Surgery may involve removing part or all of the esophagus, the affected lymph nodes, or parts of other organs where cancer has spread, such as the stomach. If the esophagus is removed, sometimes it is possible for it to be replaced with a length of the bowel, and sometimes a tube is placed for feedings directly to the stomach instead. Which one is done depends on the particulars of that patient’s case.

Esophageal cancer has a risk of serious complications, such as bleeding or leaking from the area where the cancer was removed or infection. Not all patients are eligible for surgical treatment. If cancer has spread throughout the body, to certain lymph nodes, to solid organs such as the liver, or to areas like the aorta, pericardium, or bronchial tree, surgery will not help and will not be recommended.

If there is another serious health issue, like diabetes or heart disease, that would mean surgery itself is too dangerous, then the medical team will not recommend surgery. In those cases, the patient may still receive radiation and/or chemotherapy, if they are evaluated as being likely to help.

A newer treatment is immunotherapy. If certain specific genes are present, such as HER2, a targeted medication is able to bind to particular proteins that are present in those specific cases. This medication then tries to kill only the cancer cells, without killing other healthy cells as regular chemotherapy does.This is not available in all cases, as it is in the early stages with much research ongoing.

Another new treatment is sometimes available in cases where the cancer cells are very small with no spread throughout the body. This is photodynamic therapy; a drug that causes sensitivity to light (photo-sensitivity) is injected into the body. It lasts for a couple of days; however, cancer cells retain the drug longer than healthy cells. Then when they are exposed to a laser, they may die. Light does not penetrate far into the body, so this only works for very small spots of cancer that are close to the surface.

Treatments for esophageal cancer often have side effects that include nausea, vomiting, hair loss, weight loss. There can be significant physical discomfort.

Depression and grief are common. The patient’s immune system is down, making them more vulnerable to other infections like pneumonia.

Depending on how much of the esophagus and stomach was removed, and how much scarring there is, they may have difficulty swallowing or need a feeding tube. They may need nutritional supports because of the difficulty they have eating, because of difficulty keeping food down or to teach them how to manage a feeding tube. A patient with esophageal cancer will need a whole team of medical professionals.

There will a gastroenterologist to monitor the esophagus. An oncologist will plan out the cancer treatments including radiation and chemotherapy.

There will be surgeons if the cancer is operable; there could be several kinds of surgeons depending on the spread of cancer. A primary care doctor may help to manage the side effects and overall health of the patient in the middle of all this focused treatment.

A radiation oncologist will be involved in monitoring and administering the radiation. A dietitian will help the patient maintain nutrition and adapt to any changes needed as a result of cancer and treatments. There will be nurses and aides, and medical professionals trained in specific tests – the CT scan, the PET scan. If cancer becomes untreatable or symptom control is needed, palliative care may also be involved.

Supportive therapies, such as massage, guided imagery, relaxation techniques may help a patient deal with the effects of cancer and treatment. Patients should prepare for appointments ahead of time to make the most of having the doctors there and able to answer questions, and so that they have a clear picture of what is going on with them specifically. They should bring lists of the medications and supplements may take and of any other medical issues or symptoms they’ve noticed.

A patient should feel free to bring a list of questions he may have about his cancer, the treatments, and what to expect and bring something to write down the answers. He should consider bringing a family member or friend for support, and for them to also hear and remember what the medical team advises.

Coping and Support

Getting a cancer diagnosis is incredibly stressful. Learning that it is a kind of cancer that often has a bad prognosis is even harder, and esophageal cancer has long been known as one of the most deadly malignancies. The treatment team should consider the stress and grief about the changes in life, fears, and possible end of life issues for both the patient and their family. These issues should be treated by the team, and particularly by social work, palliative care, chaplains, or counseling. There are a lot of things a patient can do to cope with this diagnosis.

The patient can start out by learning as much as he is comfortable with his cancer so that he can make informed decisions about care. This will decrease the sense of everything being completely out of control. Some people want to know everything, and some only want to know what they have to. Both choices are okay.

Learn enough to be prepared for any side effects or longer-term effects. Get connected to social support. Connect with friends and family. Let them know what is going on, and when they ask how to help, don’t brush them off.

Have something they can do – help cook, drive to appointments, help with housework. Have someone to talk to. This might be a family member, a chaplain or member of the clergy, a counselor or social worker, or a support group. Do not try to go it alone; that only makes it much harder and if the patient is trying to clean house, grocery shop, and keep a stiff upper lip, that is energy taken away from healing.

Live healthfully. Eat nutritious food, get rest, accept support from others, drink plenty of water, exercise if you are able when the patient is able, and the patient should do things that they enjoy.

A diagnosis is not the end of life. Treatments are improving all the time, and life can be enjoyed even while living with esophageal cancer.

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The information on this website has not been evaluated by the Food & Drug Administration or any other medical body. We do not aim to diagnose, treat, cure or prevent any illness or disease. Information is shared for educational purposes only. You must consult your doctor before acting on any content on this website, especially if you are pregnant, nursing, taking medication, or have a medical condition.

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