Esophageal Cancer

CANCER OF THE ESOPHAGUS

 

  • In 2018, the estimated number is approximately 17, 290  new cases of esophageal carcinoma will occur in the United States, and 15.850  deaths will result from the disease
  • Esophageal cancer is the seventh leading cause of cancer death worldwide
  • Adenocarcinomas are mainly associated with gastroesophageal reflux which is more related to obesity.
  • Difficulty in swallowing (dysphagia), weight loss, hiccups, or food coming back up the esophagus may be symptoms of esophageal cancer
  • Patients who have adenocarcinoma located in the lowest part of the esophagus (near the stomach) can benefit from chemotherapy administered before and after surgery.
  • HER2 positive cancers predicts a better prognosis

 Cancer of the esophagus is a tumor that forms in the tissue lining the esophagus, the muscular tube through which food passes from the throat to the stomach. The two main types of esophageal cancer are squamous cell carcinoma and adenocarcinoma. Both types occur at about the same frequency. Squamous cell carcinoma begins in flat cells covering the esophagus, and adenocarcinoma begins in cells that produce and release mucus and other fluids.

 

How frequent is ESOPHAGEAL CANCER?

Cancer of the esophagus accounts for 7% of gastrointestinal tract cancers in the United States. In 2018, the estimated number is approximately 17, 290  new cases of esophageal carcinoma will occur in the United States, and 15.850  deaths will result from the disease. By the early 1990s, adenocarcinoma had become the most common cancerous cell type among white Americans, accounting for approximately one-half of esophageal malignancies in the United States and Europe. Squamous cell cancers still predominate among African American patients, Internationally: Esophageal cancer is the seventh leading cause of cancer death worldwide. Unlike in the United States, squamous cell carcinoma is responsible for 95% of all esophageal cancer worldwide. Esophageal Squamous Cell Carcinoma,Esophageal Adenocarcinoma
There are considerable differences between European countries. Esophageal cancer is more frequent
in France and the UK and less frequent in Greece.
Squamous cell carcinomas are most prevalent in Asia, whereas adenocarcinomas are more prevalent
and increasing rapidly in Western countries. Most esophageal cancers occur in people older than 65.
The discrepancies in the geographical distribution of the 2 main types, squamous cell carcinomas and
adenocarcinomas, are due to the differences in the factors involved in their development. Squamous
cell carcinomas are mainly associated with alcohol intake and smoking whereas adenocarcinomas are
mainly associated with gastroesophageal reflux which is more related to obesity. This also explains
the rapid increase of adenocarcinomas in western countries.

 

WHAT CAUSES ESOPHAGEAL CANCER?

It is not clear why esophageal cancer occurs. However, some risk factors have been identified. A risk factor increases the risk of cancer occurring but is neither necessary nor sufficient to cause cancer. A risk factor is not a cause in itself. Some people with these risks factors will never develop esophageal cancer and some people without any of these risk factors will nonetheless develop esophageal cancer.
The main risk factors for esophageal squamous cell carcinoma are:
– Tobacco use: Smoking as well as chewing tobacco increases
the risk of squamous cell carcinoma.
– Alcohol consumption: The probability of getting squamous cell
carcinoma is associated with the amount of alcohol consumed.
Combining drinking of alcohol and smoking increases the risk much
more than each of these two factors separately.
– Low intake of fresh fruits and vegetables
– Drinking maté: Maté is an infusion of a herb called yerba mate which
is commonly consumed in South America.
– Betel quid chewing: Betel quid is a mixture of plants that is chewed in many cultures in
Southeast Asia.
– Certain medical conditions:
o Achalasia increases the risk of developing squamous cell carcinoma. Achalasia is a disease in which the muscle which closes the lower esophagus cannot relax properly. Because swallowed food and liquids tend to collect in the esophagus, the lowest part dilates.
o Other rare diseases such as tylosis and Plummer-Vinson syndrome also increase
the risk of squamous cell carcinoma of the esophagus.

The main risk factors for esophageal adenocarcinoma are:
– Barrett’s esophagus: A Barrett’s esophagus is the name of a situation where normal cells lining its inner part are replaced by cells resembling another type of cells normally found in the intestines. Risk factors for Barrett’s esophagus are:
o Gastroesophageal reflux-disease: sometimes called acid reflux disease.

Obesity increases the risk of Barrett’s esophagus and esophageal adenocarcinoma.

– Likewise, tobacco use and alcohol consumption can both increase the risk of
adenocarcinoma, although the effect is smaller than in squamous cell carcinoma.
Other factors have been suspected to be associated with an increased risk of esophageal cancer likehigh intake of red meat or processed food, intake of high-temperature drinks, or exposure to certain chemicals.

 

HOW IS ESOPHAGEAL CANCER DIAGNOSED?

 

Esophageal cancer can be suspected in different situations. For people with Barrett’s esophagus, regular screening should be performed to detect any evolution
to adenocarcinoma as early as possible.
For others, some symptoms can possibly indicate the presence of an esophageal cancer.
 Difficulty in swallowing (dysphagia), hiccups, or food coming back up the esophagus.
 Unexplainable weight loss
 Pain or discomfort in the throat or in the back
 Hoarseness
 Long-lasting cough
 Vomiting, or coughing up blood

Cancer of the esophagus remains a devastating disease because it is usually not detected until it has progressed to an advanced incurable stage. Modern imaging techniques, including: 1. barium esophagography. 2. contrast-enhanced computed tomography (CT) 3. magnetic resonance imaging (MRI) 4. endoscopic ultrasonography (EUS) 5. positron-emission tomography (PET) are powerful tools in the detection, diagnosis, and staging of this malignancy. Only surgical resection at a very early stage has been shown to improve survival rates in patients with this disease.

The diagnosis of esophageal cancer is based on the following examinations

1. Clinical examination.

2. Endoscopic examination. During an endoscopic examination of the upper digestive tract or esophagogastroscopy, the doctor passes a thin, flexible, lighted tube
called an endoscope down the patient’s throat. This enables the doctor to see the lining of the esophagus, stomach, and the first part of the small intestine. The doctor can also check the upper part of the trachea (airway). If abnormal areas are noted, biopsies (tissue samples) can be taken using instruments passed through the endoscope. These tissue samples are examined by a pathologist.

During the endoscopy, an endoscopic ultrasound can be performed at the same time.  This technique is used to see how far cancer has spread in the esophageal wall, into nearby tissues or to nearby lymph nodes, which is very important to know in more detail and in advance for patients who will be operated. This reveals useful information for surgery and can also guide the doctor to remove a small sample
(biopsy) of a suspicious lesion during the endoscopy. Performing an endoscopic ultrasound is, therefore, particularly useful before surgery

 

Radiological examination.  CT-scan of the chest and abdomen is usually performed. A barium swallow may also be performed to indicate precisely where the tumor is located in the esophagus. A barium swallow or barium meal involves taking X-ray* pictures while the person drinks a special fluid. Because this fluid is very bright on the X-ray picture, the inner lining of the esophagus is clearly outlined on the X-ray.

A PET-scan can be used to see how far cancer has spread outside the esophagus.

An endoscopy examining the airways (pharynx, larynx, trachea and bronchi) can also be performed.
Histopathological examination. The biopsy specimen which is the tissue sample
that has been taken during the endoscopy, will be examined in the laboratory by a pathologist

 

What are the stages of Esophageal Cancer?

Staging to assess the extent of cancer (i.e. how far it has spread in the patient’s body) and the prognosis of the patient. The TNM staging system is commonly used. The combination of T, size of the tumor and invasion of nearby tissue, N, the involvement of lymph nodes, and M, metastasis or spread of cancer to other organs of the body. Knowing the exact stage of the cancer is fundamental in order to make the right decision about the treatment. The lower the stage is, the better the prognosis. Staging is usually performed twice: after a clinical and radiological examination and after surgery. If surgery is performed, staging may also
take into account the laboratory examination of the removed tumor. this diagram explains the stages.

HOW IS ESOPHAGEAL CANCER TREATED?

Planning of the treatment involves an inter-disciplinary team of medical professionals. This usually implies a meeting of different specialists, called
multidisciplinary opinion or tumor board review. In this meeting, the planning of treatment will be discussed according to the relevant information
mentioned before.
The treatment will usually combine intervention methods that:
 Act on the cancer locally, such as surgery or radiotherapy*
 Act on cancer cells all over the body by systemic therapy* such as chemotherapy
The type and extent of the treatment will depend on the stage of the cancer, on the characteristics of the tumor and on the risks involved.

Resectability
The surgeons will either judge the tumor as operable (or resectable), meaning that it is possible to remove the complete tumor in an operation, or as inoperable(or unresectable), meaning that this is not possible. A tumor can be unresectable because it has grown too far into nearby tissues or lymph nodes, because it is too close to major blood vessels, or because it has spread to distant parts of the body. There is no distinct dividing line between resectable and unresectable in terms of the TNM stage of the cancer, but earlier stage cancers are more likely to be resectable. The decision will also depend on whether the person is fit enough to undergo the operation.
 Location of the tumor in the esophagus
To make the best treatment choice it is important to know the location of the tumor. According to their vertical location in the esophagus, tumors are usually categorized as:
o cervical, which corresponds to the upper region, located in the neck;
o intrathoracic, which corresponds to the middle region, located in the chest;
o esophago-gastric junction, which corresponds to the lowest part, connecting to the
stomach.
 Results of the biopsy
Results of the examination of the biopsy should include:
o Histological type.  If the tumor consists of flat cells lining the esophagus, it is squamous cell carcinoma. If it consists of cells that make and release mucus and other fluids, it is an adenocarcinoma. If IS as a small cell carcinoma, a very rare type of esophageal cancer, it will be treated accordingly. However, the information provided in this Guide for Patients does not apply to small cell carcinomas.
o Grade. The grade is based on how different from normal esophageal cells tumor cells look and on how quickly they multiply. The lower the grade, the better the prognosis. Besides investigating the biopsy under the microscope, the pathologist will perform certain tests that give information about the genes of the tumor cells.
o HER2-status. This test should be done for adenocarcinoma of the lowest part of the esophagus, near the junction with the stomach.  The HER2 gene is  responsible for the production of a protein that may make a cell more malignant by influencing its growth and migration. Moreover, it is an important element in defining the treatment options. When there are too many copies of HER2 and too much content of the corresponding protein in the tumor cells, we speak of a HER2-positive cancer. Otherwise, the HER2 status is negative. HER2 positive cancers are aggressive in nature.

 

 

The treatment plan for stage O to stage III adenocarcinoma.

 

Surgery is the treatment of choice in fit patients. When nearby lymph nodes are affected, surgery is still the best treatment

Adjuvant therapy  is a therapy given in addition to the main treatment, which, in this case, is the removal of the tumor by surgery. Chemotherapy used before surgery and, if possible, after surgery is the standard treatment. The combination of both radiotherapy and chemotherapy before surgery is another option. The benefits and risks of the different strategies are explained below.

Chemotherapy is the use of drugs that aim to kill tumor cells or limit their growth. When it is administered before surgery, the intention is to reduce
the size of the tumor and make ittherapy isy surgery easier. This strategy is called preoperative or neo-adjuvant chemotherapy. It gives a benefit in all types of esophageal cancer, but the advantage is greatest in adenocarcinoma. Patients who have adenocarcinoma located in the lowest part of the
esophagus (near the stomach) can benefit from chemotherapy administered before and after surgery. Chemotherapy that is given both before and after surgery is called perioperative chemotherapy. It is currently recommended for patients with locally-advanced adenocarcinoma. The drugs used to treat adenocarcinoma are cisplatin, 5-fluorouracil and possibly epirubicin. This depends on the decision of your doctor.

Radiotherapy combined with chemotherapy administered before surgery (preoperative chemoradiation) is another treatment option. However, it is still unknown which patients benefit from this intensive therapy. Chemoradiation is the combination of chemotherapy and radiotherapy within the same timeframe and following a specific schedule.

 

The tumor is judged inoperable when a tumor is judged to be inoperable or the patient is not fit enough to undergo surgery, the combination of chemotherapy and radiotherapy (chemoradiation) is preferred since it has been proven to be more effective than radiotherapy alone.

 

The treatment plan for metastatic disease (stage IV) the tumor is either of the squamous cell or of the adenocarcinoma type and it has spread to other
parts of the body like to the lungs or to the liver, regardless of the local invasion of the tumor and the affection of lymph nodes.
Patients with metastatic esophageal cancer can be considered for different treatment options to relieve their symptoms. The choice made will depend on their specific situation.
Local treatment include:
Brachytherapy is a type of radiotherapy in which radioactive material is placed directly into or near
the tumor.
Chemotherapy is the main type of systemic therapy.
Chemotherapy can help to reduce symptoms and should be considered particularly for patients who
are fit and have good general health.
Patients with adenocarcinoma of the lowest part of the esophagus should be screened for HER2
status. When there are too many copies of HER2-gene or too much protein of the corresponding
HER2-protein, we speak about a HER2-positive cancer. In case of a HER2 positive cancer, a drug
called trastuzumab could be added to the chemotherapy. Trastuzumab is a drug that specifically
targets the HER2 protein. This type of treatment is called a targeted therapy.

 

When life throws questions and decisions about you or family, what you need – quite simply – is information, answers, and direction. This online guide has been developed to provide you with clear and practical information about cancer.

The information in this document does not replace a medical consultation. It is for personal guidance use only. We recommend that patients ask their doctors about what tests or types of treatments are needed for their type and stage of the disease.

Sources:

American Cancer Society

The National Cancer Institute

National Comprehensive Cancer Network

American Academy of Gastroenterology

National Institute of Health

MD Anderson Cancer Center

Memorial Sloan Kettering Cancer Center

 

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