What is the Esophagus?

The esophagus is a hollow tube that transfers food from the throat to the stomach,that is the "food tube". The tube starts just below the "epiglottis", the flap that keeps food from going into our trachea (air pipe) when we swallow. It ends at where it joins with the upper portion of the stomach, called the "cardia". The actual area of coinage is called the "gastroesophageal junction". The esophagus is muscular, to help propel food downward with swallowing. It has a complex array of nerves ("plexus") that work to coordinate the swallowing motion. The upper 2/3 of the esophagus has a inner lining ("mucosa") of a special type of cell, called "squamous" cells, which are also found in the mouth and anal region. These cells resist abrasion and heat and are able to heal quickly if damaged, say by the sharp edges on food. The lower 1/3 of the esophagus has an inner lining of a different type of mucosa called "columnar" cells.

This becomes important for considering the cancers that arise in the esophagus. If the lower portion of the esophagus becomes infiltrated with intestinal-like glands, as it is prone to do with prolonged irritation, then this is called "Barrett's" esophagus and is a risk factor to get cancer, as will be seen.

The esophagus has an outer lining, called the "adventitia", which surrounds the muscular layers and separates the esophagus from other nearby organs. The heart is directly behind the middle esophagus, while the windpipe ("trachea") is directly in front of it. The esophagus is also very close to the liver, lungs, and major blood vessels from the heart ("aorta and venal cava"). The esophagus receives most of it's blood from the aorta and drains it to the liver and venal cava. A system of "drainage channels" runs through the esophagus, between the mucosal and muscular layers. These are called "lymph channels" and act to purify the blood serum, by transporting it to nearby "lymph nodes" (glands) where the serum is filtered. Both the blood supply, and lymph channels and glands, can act as conduits to spread infection or cancer. This spread may be along the length of the esophagus, around it's diameter, to local lymph nodes or organs, or to distant body areas.

When a person has shrinkage ("cirrhosis") of the liver due to excessive alcohol or chronic infections, it places back pressure on the blood draining from the esophagus. This results in swelling of the blood vessels in the lower esophagus, called "varices". These may spontaneously bleed when there is a lot a pressure between the liver and esophagus ("portal hypertension") and is a medical emergency. Other common problems with the esophagus (besides cancer) are "rings" or "webs"-- areas of protrusion into the normally hollow interior ("lumen") of the esophagus where food can get caught. A "stricture" is an area of narrowing of the esophagus, often from scarring from ingested chemicals (i.e. lye). Achalasia is a disease where the nerves in the esophagus don't coordinate swallowing properly, so food gets caught there. An inflammation is "esophagitis", caused by bacteria, virus, or fungi, drugs or radiation.

What is Esophagus Cancer?

The esophagus, like all body tissues, is made up of individual cells. Normally, cells within the forming esophagus divide and grow very rapidly in the womb, in early childhood, and through puberty. In adulthood, new cells are only formed to replace those which have died from injury, old age or disease. The division of cells to produce new ones is under tight control by the "genes" within each cell. These genes are made up of DNA, and if it becomes damaged, the cell may start dividing out of control. Esophagus cancer starts in a single cell which has become abnormal. This cells produces millions, and eventually billions, of copies of itself. The copies are called "clones". These clones fail to function as normal esophagus tissue, but instead divert resources from healthy cells to fuel their own growth. When there are about 1 billion cells, they form a clump, or "tumor" 1/2 inch across. A "tumor" merely means a swelling, it can be caused by infection, inflammation, cancer or whatever. If a tumor only grows in it's local area (even very large) but does not have the capacity to spread to distant body areas, it is called "benign" and is not cancer. If, however, the tumor has the ability to spread to distant body areas, it is called"malignant" and this is cancer. The actual process of spread is called "metastasis", and can occur to any area of the body.

The most common type of benign esophageal tumor arises from the muscular layer, and is called a "leiomyoma". Unfortunately, a quickly growing tumor within the esophagus is most likely to be cancerous.

How Common is Esophagus Cancer?

There were 12,500 new cases of esophagus cancer and 11,000 deaths attributable to the disease in 1996. It Accounts for 5% of Gastrointestinal cancers and about 1% of all new cancers in the U.S.A. The overall number of cases each year is steadilyincreasing. In some places, like Northern China, it is 10 times more common than in North America. It is the 7th most common cancer worldwide. In the U.S.A. men are affected more than twice as commonly as women, and Black men 3 times as often as White men. The average patient is 60 years old.

What are the Types of Esophagus Cancer?

The most common type had always been "squamous cell carcinoma" arising from the upper 2/3 of the esophagus. Now, however, there has been a dramatic increase in another type, called "adenocarcinoma" , which tends to arise in the lower 1/3 of the esophagus. Currently, the number of each of these two types of cancer cases is about equal, and together they make up nearly 100% of today's esophagus cancers. White men tend to get the disease more commonly in the lower esophagus, while Black men get it in the middle and upper esophagus.

There are occasional rare cancers found in the esophagus, such as "sarcomas" which arise from the muscular wall, "cylindroma" which begins from glands, and "lymphoma"that starts from the body's immune system cells within the esophagus.

What Causes, or Increases the Risk for Esophagus Cancer?

Like any cancer, the exact reason why one person gets esophagus cancer and another does not remains unknown . However, various "risk factors" have been noted to increase the risk for developing esophagus cancer:

Tobacco Smoking and Alcohol Consumption, especially both together over many years, markedly increases risk. The risk is 10 times higher if smokers drink beer, and 25 times higher if they drink whiskey (vs. non-drinking smokers).

Race and Sex -- Blacks get it 3 times more often; Men get it 2 times more often.

Diet -- eating lots of heavily seasoned, pickled, low protein, high fat, and low vitamin foods raises risk. By contrast, increased vitamins "A" and "E" lower risk.

Swallowing Lye as as child raises the risk 1000 times for esophagus cancer.

Rare Genetic Disease with increased risk include:
a) Tylosis- Excess skin formation on palms, soles of feet, and "warts" ("papilloma") of the esophagus-- 40% of these patients get cancer.
b) Plummer-Vinson shows anemia, tongue swelling, esophagus webs and increases risk for squamous cell upper esophagus cancer.
c) Achalasia can be genetic, it raises risk to 5% after 20 years.

Barrett's Esophagus is precancerous in at least 10% of patients. Doctor's often distinguish between "Barrett's and Non-Barrett's" esophagus cancer.

Other Aero-Digestive Tract Cancer means the risk for developing a different cancer in the lungs, esophagus, intestines or head and neck is about 20%.

How Does Esophagus Cancer Develop?

As mentioned, it starts in a single cell. There has been a gradual shift to more cancers in the lower esophagus. Overall, 15% of cases start in the upper ("cervical") esophagus, 45% in the middle ("thoracic") esophagus, and 40% in the lower esophagus. Only 2% of cases in the upper esophagus are "adenocarcinoma", but overall in the esophagus the number of cases of of adenocarcinoma and squamous cell carcinoma are now equal. The disturbed cell is "initiated" by Tobacco smoke, Pickled Vegetables, or some other chemical. Then it is "promoted" to develop cancer:

By Mouth: alcohol, hot beverages, low protein/vitamin diets-- squamous cell cancer.
By Gastro Intestinal tract: bile, stomach acid, intestinal juice-- adenocarcinoma.

Once cancer develops in a cell, this cell multiplies to form a malignant tumor. That tumor then grows upon and penetrates the inner lining of the esophagus, the spreads along the length of the organ. It gets into lymph channels to spread to local lymph glands ("lymphogenous dissemination") and then into blood vessels to spread to distant organs ("hematogenous dissemination"). It can erode through the wall of the esophagus, invading nearby organs. Most cases are already spread, at least to lymph nodes, when first diagnosed. The most common ultimate sites of spread are lymph nodes (80%), liver (55%), lung (35%), bone (11%) and brain (8%) if the disease is unchecked. If a patient succumbs to esophagus cancer, there is overall a 95% chance that it will have spread to distant areas.

What are the Symptoms of Esophagus Cancer?

Like any cancer, very early esophagus cancer will cause no symptoms since it is too small to affect organ function. When symptoms develop, the most common are:

Swallowing Problems ("dysphagia") is often present for months before the patient seeks medical attention. A subtle "hang up" feeling in the throat or chest is first seen with hard solid foods, and eventually with liquids. This causes the patient to shift toward a softer diet. Two-thirds of the inner circumference of the esophagus must be involved to get notable dysphagia. It is the most common presenting symptoms, seen in over 90% of patients.

Weight loss is seen in 80% of patients from poor nutrition, poor appetite ("anorexia") and more calories consumed by the growing cancer.

Pain ("odynophagia") is seen in 50% and indicates extension through the wall of the esophagus, and into the surrounding nerves. If the pain radiates to the back, it suggests spinal column involvement.

Pneumonia or other infections occur in 30% of patients and indicates that food is being forced into the lungs by the esophagus obstruction, or an actual communication channel has now developed between the esophagus and trachea ("tracheo-esophageal fistula"). An incessant cough may also develop.

Speech Difficulty ("dysphonia") and hoarseness indicate the cancer has now spread to the voice-box nerve that runs through the upper chest ("recurrent laryngeal nerve). The voice may be of higher pitch on rapid exhalation.

Shortness of Breath --the diaphragm (muscle controlling the lung) may become immobile on one side if the phrenic nerve is involved. If both sides are paralyzed, the patient would be unable to breath at all.

Superior Vena Cava Syndrome means that the tumor in the chest is pressing upon and obstructive the main venous return to the heart. The face and upper limbs become very swollen, the patient is short of breath; it is an emergency.

Signs of Distant Spread to brain with neurological symptoms (forgetfulness, poor judgment, numbness, paralysis, balance problems, seizures), or to spinal cord (loss of bladder and bowel control, numbness and paralysis), or to bones (pain and possible fracture) are all possible and should be brought to medical attention immediately.

How is Esophagus Cancer Detected and Evaluated?

If a patient comes ("presents") to their doctor with signs or symptoms suggestive of esophagus cancer, the following are standardly done:

Complete History and Physical Examination with special attention to the head and neck and chest areas. Signs of hoarseness, lung infection, and recent weight loss are noted. The abdomen is examined for liver and spleen swelling (hepato-spleenomegaly") and the area above the clavicles and in the armpits ("axilla") for lymph node enlargement. Neurologic exam is done; the prostate is checked in males and the pelvis and breasts examined in females.

Endoscopic Examination means placing a visualization tube under light local anesthesia into the nose and down the throat. The preferred procedure is a "triple endoscopy", which looks at the nose, esophagus and larynx (voice-box). This procedure is usually performed by an Ear, Nose and Throat doctor ("ENT" or "otolaryngologist" ). Clear endoscopic visualization of the esophagus is "direct laryngoscopy" and is performed after spraying some mild numbing medicine (lidocaine) into the throat to help prevent irritation and gagging. A biopsy is taken of any suspicious areas, and sometimes "blind biopsies" are taken of areas most likely to develop cancer (such as the tonsil). This is done since 5% of patients will be found to have a "second primary" when they first come to medical attention-- that means another simultaneous cancer.

Endoscopy is a very safe procedure, and is the clearest way to actually look at tissues of the aero-digestive tract. A diagnosis of "Barrett's Esophagus" may be made through samples taken at endoscopy.

Blood and Urine Tests are standard pre-operative ones to assess general health; there are no special blood tests ("tumor markers") yet to detect spread of squamous cell cancer as there are for some other cancers. Routine tests include Complete Blood Count ("CBC") to look for anemia and infection. A Blood Chemistry Panel ("SMA") measures sodium, potassium, blood sugar, cholesterol and liver and kidney function. If a major surgery is contemplated, blood tests for clotting ability ( PT, PTT and bleeding time ) are standard. A Urinalysis (UA) to check for protein, blood or infection completes the lab tests.While Carcino-Embryonic Antigen (CEA) or CA-50 blood tests may be elevated in 40% of patients with esophagus cancer, they are not specific for this condition. Thus, they would not make good screening tests. However, if they are elevated, they may be used a marker for successful treatment.

Imaging Tests are done in the radiology department and standardly include a Chest X-ray to look for signs of infection or lung tumors. Also free air in the chest is seen if the tumor has penetrated the esophagus. Barium Swallow has been the conventional test to see if there is a tumor in the esophagus, and today is still often done prior to endoscopy. The patient drinks some thick barium contrast material, and a series of X-rays are taken. This outlines any noticeable abnormality in the hollow ("lumen") of the esophagus. It would also show if swallowing was compromised by achalasia or a stroke. If there is any problem swallowing, it is critical to do a "modified" (instead of "regular") barium swallow, since if a large quantity of barium is given to swallow, it may be shunted ("aspirated") into the lungs causing chemical pneumonia. CT scan in "thin slices" of the tumor area helps define the extent of the tumor, and is excellent for looking for spread to lymph nodes around the esophagus which cannot be detected on standard physical exam. Another excellent test to study the soft tissues in the chest is the more expensive Magnetic Resonance Imaging (MRI) scan. MRI, which uses magnetism instead of radiation, allows the area to be viewed in three dimensions. It is great for looking at the tumor as well as checking local muscle, cartilage and nerves for signs of invasion by cancer. Contrast material can be injected into the bloodstream prior to either CT scan or MRI, the contrast runs through and highlights blood vessels. If getting a CT with contrast, ask for "omnipaque" contrast; other types are less expensive but more likely to cause an allergic reaction. For MRI scans, a different type of contrast, called "gadolinium" is used. Other more exotic tests are only obtained in the presence of suspicious symptoms. For instance, a bone scan is gotten if there is new bone pain, a CT of the chest and Abdomen is ordered if the plain Chest X-ray appears to show tumor in the lung, and a CT of the Brain is gotten if new neurological symptoms occur. If major surgery is contemplated, Lung Function Tests ("FEV") are done in the pulmonology dept. to assess lung capacity. An EKG is gotten to rule-out recent heart attack, and if heart abnormali- ties are suspected astress-thallium and/or echocardiogram look at the heart. Tests can be ordered to look at any area of the body-- but only if necessary.

Biopsy (sampling) is crucial, since only by examining an actual piece of the tumor under the microscope can a diagnosis of cancer be made, and then the particular type known with certainty. For a very small tumor, the whole of it may be removed, along with a "safety margin" of surrounding normal tissue, and sent for evaluation ("Excisional Biopsy"). For a larger tumor, a cut is made into it so some tissue can be removed for examination ("Incisional Biopsy"). It usually takes several days (of anxious waiting) for the pathology report to come back. A pathologist is a physician who specializes in diagnosing disease from tissue samples. The pathologist will confirm or deny cancer in the "biopsy specimen", and name the particular type and "grade" if cancer is found. "Grades" are given as I, II or III and indicate the probable aggressiveness of the cancer. "Grade III" is "high grade", shows rapid cell divisions, and doesn't look much like normal esophagus tissue. It is likely to be aggressive. On the other hand, "Grade I" will look a lot like the normal tissue it arose from, have relatively few cell divisions, and tend to be more "indolent" (slower growing and spreading). "Grade II" is intermediate in behavior. The pathologist may need special stains and even an electron microscope to characterize the cancer; there may be more than one type of cancer or grade within a given tumor ("mixed tumor").

How is the Extent of Esophagus Cancer Guaged?

The extent is given by the American Joint Cancer Committee "Stages":
Stage I The cancer is confined to the esophagus' superficial inner lining.
Stage II The cancer invades into the muscular lining of the esophagus.
Stage III The cancer invades the outer lining ("adventitia") and/or lymph nodes
Stage IV The cancer has distant spread, i.e. lung, liver, bone or brain.

What is the Survival from Esophagus Cancer?

This depends upon the stage, type, general condition of the patient and treatment selected. From standard textbooks, historical survivals are:

It is important to realize that the above numbers reflect death from all causes in these patients, such as heart attack and accidents, and not just esophageal cancer. Many patients with esophagus cancer have been long term smokers and drinkers and have other "concomitant" health problems leading to their demise. No one can say how long an individual with esophagus cancer will live. Furthermore, the results are better than those quoted above when considering the latest effective treatments. Moreover, many patients lead high quality and fulfilling lives with their cancer, if symptoms are properly managed ("palliation").

What Determines Outcome in Esophagus Cancer?

Determinants of outcome, gleaned from studies, are called "prognostic factors" :

• Females do better than Males
• Whites do better than Blacks
• Younger than age 65 do better
• Tumors less than 5 cm. (2 inches) do better
• Non-circumferential tumors do better than those that surround entire lumen
• Protruding ("exophytic") tumors do better than Ulcerating ("endophytic") ones.
• Upper esophagus tumors do better than lower esophagus tumors.

In the Clinic, the most important factors determining how well a patient will do are:

• How well the person can take care of themselves ("Karnofsky score")
• Whether or not the tumor penetrates the esophagus wall.
• Whether lymph nodes are involved.
• How much weight the patient has lost.

Unfortunately, most (85%) of patients have disease already spread outside of the esophagus when they first come to medical attention. This accounts for the poor historic overall survival of 15% at 5 years for esophagus cancer patients as a group.

What is the Conventional Treatment for Esophagus Cancer?

Surgery Alone has been, and remains, the treatment of choice for early esophagus
cancer (confined to the esophagus). If the entire cancer can be removed, then survival will exceed 80%. However, only about 25% of patients can be operated upon for "cure"
when they first present, since the cancer will have obviously spread outside of the esophagus in the remainder.

Patients potentially curable by surgery include:

1) No extension beyond the esophagus wall
2) No lymph node involvement
3) No signs of distant disease spread.

For the very smallest, superficial tumors, laparoscopic removal with a safety margin of normal tissue may be appropriate, especially for older, debilitated patients or those with severe concomitant medical problems. However, in almost all cases the actual operation performed is called a "Total or Subtotal" Esophagectomy", meaning that at least a large portion, if not the entire esophagus, is removed. Recall that cancers tend to spread in the submucosal lymphatics up and down the length of the esophagus. For an Esophagectomy operation, a midline incision is made in the chest (called a "thoracotomy" ) and the cancer is cut out with at least a 3 inch safety margin on all sides. The remaining esophageal ends are rejoined ("re-anastomosed") surgically if possible. If the gap is too large to stretch the remaining esophagus for rejoining, then the stomach can be pulled up though the "crus" of the diaphragm to rejoin the lower end of the esophagus. Alternatively, a piece of the left colon can substitute.

Previously, surgery was quite dangerous with a death rate of up to 45%; now it has been reduced to as low as 5% . Mortality is caused by heart attack, stroke, blood clots in the lungs and infection in the peri-operative (around the time of the surgery) period.

Side effects of even successful surgery may include: Stomach emptying problems, narrowing of the esophagus ("stricture") that will require periodic dilatation, and developing pneumonia from regurgitation of food into the lungs ("aspiration"). It is important for the patient to exercise the breathing muscles after surgery by forcefully breathing into bedside "spirometer" and coughing, even if it hurts, to reduce the chance for getting pneumonia. The head of the bed should be somewhat elevated, especially at night, for food may creep up the esophagus and into the airway when the patient is asleep or heavily medicated. Pressure stockings should be used around the patients legs, if they have a prolonged stay in bed, to decrease the risk of leg blood clots forming and traveling to the lungs ("pulmonary embolism"). The symptoms of this are shortness of breath and anxiety, and it must be quickly managed with blood thinners, which is tricky soon after major surgery. The best course of action is to get the patient up out of bed walking with assistance as soon as possible. Normally, surgically sewn ("sutured") areas return to 75% of their normal strength within 3 weeks after surgery, and this is when heavy weight can be lifted.

The overall survival with surgery alone is 15%, which well corresponds to the fraction of patients with disease localized to the esophagus at the time it is first detected. Even the best surgery is only a local therapy, it does nothing for disease that has spread away from the surgical area. Nonetheless, it may assist the success of other therapies, and certainly give the patient immediate relief from swallowing obstruction caused by a large tumor.

Radiation Therapy Alone has historically been used for inoperable tumors and those of the upper ("thoracic") esophagus. It has been considered more symptom relieving ("palliative") than curative, but new therapies with it mandate full description.

Prior to getting radiation therapy, the patient is seen in consultation by a "Radiation Oncologist" , a cancer physician who specializes in administering radiation. There are 2 standard methods of giving radiation-- "External Beam" and "Brachytherapy". External Beam is the more common type and shines a beam of photons or electrons onto a predesignated area of the patient's chest. Thus, it can cover a large area of possible cancer spread. Brachytherapy, also called "intracavitary", means putting an actual radiation source into the esophagus,, either temporarily or permanently, to treat a limited area of tumor. Both techniques may be used in a given patient.

Radiation kills cancer cells by damaging their DNA, they die when they try to divide. Thus, damaged cancer cells die even after the treatment is complete. Radiation will also kill normal cells, which limits the amount that can be given. However, it usually takes more radiation to kill normal cells than cancer cells, and normal cells can often repair the radiation damage, while cancer cells can not. Nevertheless, it is important to be as exacting as possible in the administration and dose of radiation, so as to minimize the injury to adjacent normal cells. Particular areas of concern when radiating the esophagus include the liver, heart, lung and spinal cord. The doses to each of these areas must be carefully limited to avoid unnecessary injury.

To receive therapy, a patient is first seen in "consult" by the radiation oncologist, who reviews the patient's medical record, complaints, and radiology films. After explaining the possible benefits and side-effects of radiation, the patient is scheduled for a "simulation". This means the area to be treated is marked out on a replica treatment machine, and films are taken. Watercolor marks are painted on the patient to denote the treatment area, and eventually small, permanent tattoos are placed on the skin. Sometimes the patient is sent for a CT scan along with the simulation, the whole process takes less than 2 hours, and is painless. Information from the simulation and relevant scans is placed into a"treatment planning computer", which generates a "plan". This plan tells how much radiation is going to the tumor area, and how much to adjoining normal tissues. The plan is reviewed by the radiation oncologist and also by a specially licensed Radiation Physicist prior to starting therapy. The patient then comes in for their "treatment start". They are placed on a hard, flat table in a specially shielded room and aligned with laser lights. The actual treatments are given by "Radiation Therapists", or "R.T.T's", who are first certified for diagnostic X-rays and then get additional training to deliver therapy. For the first treatment, "verification films" are taken to ensure proper positioning; they do not tell anything about the cancer. The actual treatment only takes a couple of minutes and is given with a "Linear Accelerator" (aka "LINAC" ), or older Cobalt-60 technology, which precisely aims a beam of photons at the treatment area. The head of the machine can swivel about the patient, to give the treatment from several angles. The patient needs only to lie still. Areas that are not to be treated can be "blocked" with special lead-type blocks in the head of the treatment machine. Normally, patients are treated 5 days a week, Monday through Friday, taking only several minutes each day.

The usual dose of radiation for esophagus cancer is ~60 Gray (units of radiation) given at about 2 Gray per day over 5 to 7 weeks. Often, a larger area of the chest is treated initially, and then a "cone down" or "boost" is used to narrow the high dose treatment to the specific tumor area as seen on X-ray. Treatment itself is painless, the patient doesnot become sick, "radioactive", or lose their scalp hair from radiation to the chest. The side effects from chest radiation for this cancer are divided into two general categories,"acute" and "late" effects. "Acute" effects occur during the treatment period, and commonly resolve afterward. "Late" effects may occur months to years after treatment, and may improve very slowly or never resolve. Typical "acute" effects are skin redness within the treatment area, difficulty or pain on swallowing as the esophagus is treated, and general fatigue. It is more likely to get infections of the esophagus, particularly fungal "esophageal candidiasis" (aka thrush). This can be treated with antifungal medications like oral nystatin or fluconozole pills. Swallowing discomfort can be mostly alleviated with numbing "viscous lidocaine", often mixed into a soothing solution with Benadryl and Maalox. Mild codeine preparations may also be helpful. Possible "late" effects include damage to normal lung ("radiation pneumonitis") which causes cough, fever and shortness of breath. It only occurs in about 10% of patients and is often treatable with steroids. However, it is occasionally fatal. Treating large areas of lung, and/or giving chemotherapy along with chest radiation, increases the chance for radiation pneumonitis. Another feared complication of chest radiation is spinal cord damage, since the spinal cord is located in the spinal column behind the esophagus. Spinal cord damage may be reversible, such as a temporary sensation of "electric shocks" shooting down down the body with neck flexion ("Lhermitte's sign") which is scary but commonly resolves. Worse but very rare with modern techniques is "transverse myelopathy", which means severing the spinal cord from too much radiation-- this will cause permanent paralysis below the injury. The spinal cord tolerates about 45 Gray with less than 1% chance of damage, and this is a reason why careful "simulation" and review by a radiation physicist is crucial to ensure that the "cord" is not being overdosed. As mentioned, this is very rare with today's technology and training. Radiation can also cause heart damage, such as irritation of the fluid-filled sac around the heart ("pericarditis") which may cause chest pain and fever, and need a surgical procedure to drain excess fluid. This is also very rare. Overall, External Beam chest radiation is well tolerated and increases survival in esophagus cancer, compared to no therapy.

Results of Radiation Alone show a survival of 15% at 5 years (similar to surgery.) There is no question that local radiation is very valuable in relieving symptoms of esophageal cancer, whether from pain from spread to ribs (or other bone) or helping reduce the coughing up of blood (hemoptysis) from the cancer. It can be used to relieve neurological symptoms from spread to the brain, or dangerous pressure upon the spinal cord from metastasis. Thus, radiation therapy is almost always used in advanced cancer to relieve symptoms, with up to 90% effectiveness.

Brachytherapy is being used more commonly today, since it gives a high dose of radiation to a local tumor area with minimal side effects to surrounding normal tissues. In practice, it is usually given in conjunction with external beam therapy, since we are worried about cancer cells that may have escaped around the periphery of the tumor, and into local lymph nodes, which would not be adequately treated with any brachytherapy alone. Giving brachytherapy, or "intracavitary" therapy, can be done in several ways. One is surgical placement of permanent "iodine -125" seeds in the area of the tumor, often at surgery. These seeds have an effective life of about 90 days during which they give potent radiation to their immediate area, but nearly none just a couple of centimeters away. Another option, especially for patients not getting surgery, is treatment with high-dose radioactive sources contained in a small tube ("catheter")
placed with an endoscopy tube through the mouth, down the throat and into the esophagus. Brachytherapy is well suited for tumors within the hollow esophagus, since we can place our radiation source there without surgery. An "endoscope" is used to guide the radiation catheter(s), using topical viscous lidocaine or mild ("twilight") anesthesia to prevent gagging. Multiple treatments may be given, 2 to 4 weeks apart, with high dose brachytherapy (HDR) ; the actual time of treatment is only a few minutes. If the esophagus gets re-obstructed after other therapies, there is a good chance (up to 70%) that it can be re-opened using this "endoesophageal brachytherapy". It is available at most Academic University Radiation Oncology departments.

When radiation is used for esophageal cancer, we may successfully control the disease in the chest, but lose the war to spread of the disease elsewhere in the body. This is the same as for surgery, since ultimately both radiation and surgery are local therapies that do nothing about disease which has escaped to other body areas. Thus, to address this, chemotherapy has been looked to treat the entire body.

Chemotherapy is rarely used alone, but almost always in conjunction with surgery, radiation or both. This is because chemotherapy alone has been disappointing with the current agents available. Active chemicals include cis - Platinum, 5-Fluorouracil ("5-FU"), and Mitomycin-C . The response to agents alone, or combined with each other, can be as high as 80%. Unfortunately, this "response" tends to be short lived, for an average of 6 months. Then the cancer starts growing again. Cancers become resistant to chemotherapy much as bacteria do toward antibiotics. Furthermore, the chemotherapy can be quite toxic, and must be administered under the watchful eye of a vigilant "Medical Oncologist". Side-effects include anemia and decreased white blood cell count leading to infections (called "Bone-Marrow Suppression" and "Febrile Neutropenia") which can be treated with red and white cell forming stimulants (Erythropoetin and Neupogen). Mouth sores and nausea are common (5-FU). Permanent nerve damage, kidney damage and hearing loss may occur (Platinum). The results of Chemotherapy Alone show no overall improvement in Survival.

Combination Therapy "Pre-Operative" Radiation: Giving Radiation treatments prior to surgically removing the cancer was tried to decrease tumor size, kill tumor in local lymph nodes, and reduce the spread of cancerous cells("seeding") during surgery. After all, a larger "field" can be treated with radiation than surgery, possibly "sterilizing" tumor cells that have escaped from the periphery of the main tumor. A less dramatic operation may be needed if the cancer shrinks when radiated, reducing surgical complications. Radiation has been given as External Beam therapy. One option is a low dose "short course" (20 Gray in one week with surgery to follow), or alternatively "long course" (60 Gray over 6 weeks with surgery about 5 weeks after completion of radiation). While the idea seems good in theory, it did not translate out into increased survival. The death rate at surgery, and ultimately from esophageal cancer, is about the same whether or not pre-operative radiation was given. It can increase surgical complications, like wound separation ("dehiscence"), without confering proven benefit. Thus, there is no overall benefit to giving pre-operative radiation alone so it's use has been abandoned.

Post-Operative Radiation Therapy has also been tried to "mop up" any remaining cancer, and so help prevent relapse and improve survival in esophagus cancer. The theory is that the true extent of chest disease is discovered at surgery, and so the radiation fields can be "tailored" to treat remaining disease. Although most Radiation Oncologists would advocate giving radiation as "adjuvant" (extra) therapy if cancer remains after surgery, this approach has found absolutely no increase in survivalwhen radiation is administered after curative surgery.Once the area has been surgically disturbed, it does not get as much oxygen, which is necessary for radiation to be effective. Thus, higher doses of radiation are theoretically needed, with more potential for radiation side-effects. Nonetheless, it remains very common to treat patients after surgery, since it is then that the disease is found to be larger than originally thought. A "margin" of at least 5 cm. on each side of the remaining esophagus is recommended to kill cancer cells which have traveled along the esophageal length. At least 3 weeks should be waited between surgery and the commencement of radiation therapy, to allow the sutured areas to heal. The patient should be carefully monitored for complications, which are always more likely when more than one treatment ("modality") is used.

What is the Latest, Most Effective Treatment for Esophagus Cancer?

Survival has been shown to be increased over conventional therapy with the latest combination of therapies, called "Multi-Modal" therapy:

Chemotherapy with Radiation is called "Chemo-Radiation" and was found to increase survival in esophageal cancer. A landmark study by Dr. Herskovic at Wayne State University in Michigan, published in the New England Journal of Medicine in 1992, used chemotherapy with 5-FU and Platinum, along with radiation to 50 Gray given at the same time ("concomitantly"). These results were compared to a group of patients getting radiation alone. Survival was improved in the group getting Chemo-Radiation. Specifically, at 2 years the survival was 40% in the group getting both chemotherapy and radiation, compared to just 10% in the group getting radiation only. While the side-effects (particularly radiation esophagitis with swallowing pain, and fatigue) are greater with chemo-radiation, most patients can complete this treatment. If the patient is getting malnourished from swallowing difficulty, it may be necessary to place a temporary feeding tube ("PEG") through the abdominal wall, and into the stomach to ensure proper nutrition. This tube is removed when the acute affects subside after treatment. While the therapy is not pleasant, it can be done on many who could not tolerate radical surgery. These were the best results seen yet for patients with inoperable esophageal cancer.

Chemo-Radiation followed by Surgery makes use of all three of our conventional modalities, and is currently the most aggressive approach. If a couple of doses of chemotherapy are given before the radiation is started, this is called "neo-adjuvant" chemo-radiation, and theoretically sensitizes the cancer cells by dousing them with chemicals, making them more susceptible to radiation damage. Recall that cancer cells cannot repair radiation damage, while adjacent normal cells usually can. When just Chemo-Radiation was used (as described above) there was still a local relapse rate of almost 50% in the chest. Therefore, Dr. Arlene Forestiere at MD Anderson Cancer Center in Texas tried a new approach using all 3 ways of standard therapy-- Chemotherapy, Radiation, and Surgery. She used 3 agents for chemotherapy-- 5-FU, Platinum , andVinblastine ("Velban"). She concomitantly gave radiation to 45 Gray and then did surgery. Her results showed that over 80%2%! The overall survival rate at 5 years was 35%, and in those who had no cancer left in the esophagus at the time of surgery (that is a "complete response" to chemo-radiation) survival was 60% at 5 years! These were the best results ever seen for esophagus cancer, but not every study of such an aggressive approach has shown equal benefit. A study called RTOG 95-15 is attempting to clarify this. Overall, however, there is no doubt that multi-modality therapy , properly sequenced and administered, improves results for cancer spread outside of the esophagus.

What About for Advanced Esophagus Cancer?

When esophagus cancer is stage IV, the most advanced type with distant disease spread through the body, the objective is no longer cure but palliation (meaning relief of pain and other symptoms). The patient should be made as comfortable as possible, and narcotic medicines like morphine (which Osler called "G-d's own medicine) should never be withheld for fear of causing "addiction". Importantly, Radiation Treatment can help relieve chest pain, bone pain, esophagus obstruction and vomiting up of blood in over 80% of patients. It is also useful for reducing the symptoms, and even extending survival, in patients with spread to the brain. Sometimes radiation therapy is used as an emergency measure when the cancer spreads to the spinal column and threatens to cause paralysis by pressing upon the spinal cord. Any patient with esophagus cancer who experiences new weakness of the extremities, numbness, or loss of bowel or bladder function must be brought into the Emergency Room immediately to see whether the tumor is compressing the spinal cord causing these symptoms.

Up to 60% of new back pain in a cancer patient is caused by spread of cancer there. The patient is given a painless Magnetic Resonance Imaging (MRI) scan to check for "epidural spinal cord compression". If this is caught early, and treatment is given, permanent paralysis may be prevented. It is unfortunately uncommon to reverse symptoms of paralysis once they have set it, however, so quick recognition is essential. Another syndrome that esophagus cancer patients may get is called "Superior Vena Cava" or "SVC" syndrome; this involves swelling of the face and arms, with shortness of breath. This can be relieved rapidly by local radiation treatments.

As mentioned, radiation treatment can be very helpful for metastatic esophagus cancer. A relatively new method of radiation for spread to the brain is "Stereotactic Radiosurgery", where multiple beams of convergent radiation are aimed onto the area(s) of spread in brain, in a single painless session of one afternoon. This is usually followed by conventional External Beam Radiation. The advantage of Stereotactic Radiosurgery is that it can give a very high dose of radiation to areas of brain metastasis, and possibly enhance survival for these patients, without the risk of an open brain surgery from a neurosurgeon.

If the esophagus has had maximal radiation, and is still obstructed, a "stent" tube may be put in to bypass the tumor and still allow for mouth feedings. Also, the YAG-laser can reduce the tumor size in 1 or 2 sessions for palliation. Placement of a permanent feeding tube in the face or tracheo-esophageal fistula or unrelievable obstruction is a choice to be made by the patient, family, clergy and physician.

The patient with newly diagnosed esophagus cancer should not rely on any one therapy, such as a pill or ray, but instead should use a combination approach to maximize the chance for success. Specifically, besides the conventional medical therapies mentioned above, consider the use of a non-toxic, not over-expensive alternative therapy that you believe in, a program of spiritual renewal, "mind over cancer', nutritional therapy and exercise. Keep the most positive attitude possible-- research has shown this to be an important factor in survival. Using a true "multi-modality" approach will give the confidence that you have done everything possible for a happy outcome, and certainly improve the current quality of life. If cure is the objective, consider getting treatments at a University Academic Center and joining ongoing research trials which offer the latest therapies. The future has never looked brighter for esophagus cancer patients!

This is the full transcript, offered freely in the spirit of internet sharing, of CancerAnswers' report on Esophagus Cancer. Much more, including latest additional treatments for Esophagus Cancer can be found on our order page. Thank you for using CancerAnswers as your information resource.