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Esophageal cancer

 
Medical Encyclopedia: Esophageal Cancer

Definition

Esophageal cancer is a malignancy that develops in tissues of the hollow, muscular canal (esophagus) along which food and liquid travel from the throat to the stomach.

Description

Esophageal cancer usually originates in the inner layers of the lining of the esophagus and grows outward. In time, the tumor can obstruct the passage of food and liquid, making swallowing painful and difficult. Since most patients are not diagnosed until the late stages of the disease, esophageal cancer is associated with poor quality of life and low survival rates.

Squamous cell carcinoma is the most common type of esophageal cancer, accounting for 95% of all esophageal cancers worldwide. The esophagus is normally lined with thin, flat squamous cells that resemble tiny roof shingles. Squamous cell carcinoma can develop at any point along the esophagus but is most common in the middle portion.

Adenocarcinoma has surpassed squamous cell carcinoma as the most common type of esophageal cancer in the United States. Adenocarcinoma originates in glandular tissue not normally present in the lining of the esophagus. Before adenocarcinoma can develop, glandular cells must replace a section of squamous cells. This occurs in Barrett's esophagus, a precancerous condition in which chronic acid reflux from the stomach stimulates a transformation in cell type in the lower portion of the esophagus.

A very small fraction of esophageal cancers are melanomas, sarcomas, or lymphomas.

There is great variability in the incidence of esophageal cancer with regard to geography, ethnicity, and gender. The overall incidence is increasing. About 13, 000 new cases of esophageal cancer are diagnosed in the United States each year. During the same 12-month period, 12, 000 people die of this disease. It strikes between five and ten North Americans per 100, 000. In some areas of China the cancer is endemic.

Squamous cell carcinoma usually occurs in the sixth or seventh decade of life, with a greater incidence in African-Americans than in others. Adenocarcinoma develops earlier and is much more common in white patients. In general, esophageal cancer occurs more frequently in men than in women.

— Maureen Haggerty; Kevin O. Hwang, M.D.



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Oncology Encyclopedia: Esophageal Cancer
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Key Terms: Computed tomography, Endoscopic ultrasound, Laparoscopy, Positron emission tomography, Synergistic.

Definition

Esophageal cancer is a malignancy that develops in tissues of the hollow, muscular canal (esophagus) along which food and liquid travel from the throat to the stomach.

Description

Esophageal cancer usually originates in the inner layers of the lining of the esophagus and grows outward. In time, the tumor can obstruct the passage of food and liquid, making swallowing painful and difficult. Since most patients are not diagnosed until the late stages of the disease, esophageal cancer is associated with poor quality of life and low survival rates.

Squamous cell carcinoma is the most common type of esophageal cancer, accounting for 95% of all esophageal cancers worldwide. The esophagus is normally lined with thin, flat squamous cells that resemble tiny roof shingles. Squamous cell carcinoma can develop at any point along the esophagus but is most common in the middle portion.

Adenocarcinoma has been increasing, and, among white males in the U.S., incidence of adenocarcinoma is almost equal to that of squamous cell carcinoma. Adenocarcinoma originates in glandular tissue not normally present in the lining of the esophagus. Before adenocarcinoma can develop, glandular cells must replace a section of squamous cells. This occurs in Barrett's esophagus, a precancerous condition in which chronic acid reflux from the stomach stimulates a transformation in cell type in the lower portion of the esophagus.

A very small fraction of esophageal cancers are melanomas, sarcomas, or lymphomas.

Demographics

There is great variability in the incidence of esophageal cancer with regard to geography, ethnicity, and gender. The overall incidence is increasing. About 13,000 new cases of esophageal cancer are diagnosed in the United States each year. During the same 12-month period, 12,000 people die of this disease. It strikes between five and ten North Americans per 100,000. In some areas of China the cancer is endemic.

Squamous cell carcinoma usually occurs in the sixth or seventh decade of life, with a greater incidence in African-Americans than in others. Adenocarcinoma develops earlier and is much more common in white patients. In general, esophageal cancer occurs more frequently in men than in women.

Causes and Symptoms

Causes

The exact cause of esophageal cancer is unknown, although many investigators believe that chronic irritation of the esophagus is a major culprit. Most of the identified risk factors represent a form of chronic irritation. However, the wide variance in the distribution of esophageal cancer among different demographic groups raises the possibility that genetic factors also play a role.

Several risk factors are associated with esophageal cancer.

  • Tobacco and alcohol consumption are the major risk factors, especially for squamous cell carcinoma. Smoking and alcohol abuse each increase the risk of squamous cell carcinoma by five-fold. The effects of the two are synergistic, in that the combination of smoking and alchohol increases the risk by 25- to 100- fold. It is estimated that drinking about 13 ounces of alcohol every day for an extended period of time raises the risk of developing esophageal cancer by 18%. That likelihood increases to 44% in individuals who also smoke one or two packs of cigarettes a day. Smokeless tobacco also increases the risk for esophageal cancer.
  • Gastroesophageal reflux is a condition in which acid from the stomach refluxes backwards into the lower portion of the esophagus, sometimes causing symptoms of heartburn. In some cases of gastroesophageal reflux, the chronic exposure to acid causes the inner lining of the lower esophagus to change from squamous cells to glandular cells. This is called Barrett's esophagus. Patients with Barrett's esophagus are roughly 30 to 40 times more likely than the general population to develop adenocarcinoma of the esophagus.
  • A diet low in fruits, vegetables, zinc, riboflavin, and other vitamins can increase risk of developing to esophageal cancer.
  • Caustic injury to the esophagus inflicted by swallowing lye or other substances that damage esophageal cells can lead to the development of squamous cell esophageal cancer in later life.
  • Achalasia is a condition in which the lower esophageal sphincter (muscle) cannot relax enough to let food pass into the stomach. Squamous cell esophageal cancer develops in about 6% of patients with achalasia.
  • Tylosis is a rare inherited disease characterized by excess skin on the palms and soles. Affected patients have a much higher probability of developing esophageal cancer than the general population. They should have regular screenings to detect the disease in its early, most curable stages.
  • Esophageal webs, which are protrusions of tissue into the esophagus, and diverticula, which are outpouchings of the wall of the esophagus, are associated with a higher incidence of esophageal cancer.

Symptoms

Unfortunately, symptoms generally don't appear until the tumor has grown so large that the patient cannot be cured. Dysphagia (trouble swallowing or a sensation of having food stuck in the throat or chest) is the most common symptom. Swallowing problems may occur occasionally at first, and patients often react by eating more slowly and chewing their food more carefully and, as the tumor grows, switching to soft foods or a liquid diet. Without treatment, the tumor will eventually prevent even liquid from passing into the stomach. A sensation of burning or slight mid-chest pressure is a rare, often-disregarded symptom of esophageal cancer. Painful swallowing is usually a symptom of a large tumor obstructing the opening of the esophagus. It can lead to regurgitation of food, weight loss, physical wasting, and malnutrition. Anyone who has trouble swallowing, loses a significant amount of weight without dieting, or cannot eat solid food because it is too painful to swallow should see a doctor.

Diagnosis

A barium swallow is usually the first test performed on a patient whose symptoms suggest esophageal cancer. After the patient swallows a small amount of barium, a series of x rays can highlight any bumps or flat raised areas on the normally smooth surface of the esophageal wall. It can also detect large, irregular areas that narrow the esophagus in patients with advanced cancer, but it cannot provide information about disease that has spread beyond the esophagus. A double contrast study is a barium swallow with air blown into the esophagus to improve the way the barium coats the esophageal lining. Endoscopy is a diagnostic procedure in which a thin lighted tube (endoscope) is passed through the mouth, down the throat, and into the esophagus. Cells that appear abnormal are removed for biopsy. Once a diagnosis of esophageal cancer has been confirmed through biopsy, staging tests are performed to determine whether the disease has spread (metastasized) to tissues or organs near the original tumor or in other parts of the body. These tests may include computed tomography, endoscopic ultrasound, thoracoscopy, laparoscopy, and positron emission tomography.

Treatment Team

The treatment team includes the surgeon, radiologist, radiation therapist, and oncologist. Nutrition therapists are also vital in optimizing a diet that the patient can swallow easily.

Clinical Staging, Treatments, and Prognosis

Staging

Stage 0 is the earliest stage of the disease. Cancer cells are confined to the innermost lining of the esophagus. Stage I esophageal cancer has spread slightly deeper, but still has not extended to nearby tissues, lymph nodes, or other organs. In Stage IIA, cancer has invaded the thick, muscular layer of the esophagus that propels food into the stomach and may involve connective tissue covering the outside of the esophagus. In Stage IIB, cancer has spread to lymph nodes near the esophagus and may have invaded deeper layers of esophageal tissue. Stage III esophageal cancer has spread to tissues or lymph nodes near the esophagus or to the trachea (windpipe) or other organs near the esophagus. Stage IV cancer has spread to distant organs like the liver, bones, and brain. Recurrent esophageal cancer is disease that develops in the esophagus or another part of the body after initial treatment.

Treatment

Treatment for esophageal cancer is determined by the stage of the disease and the patient's general health. The most important distinction to make is whether the cancer is curable. If the cancer is in the early stages, cure may be possible. If the cancer is advanced or if the patient will not tolerate major surgery, treatment is usually directed at palliation (relief of symptoms only) instead of cure.

Surgery

The most common operations for the treatment of esophageal cancer are esophagectomy and esophagogastrectomy. Esophagectomy is the removal of the cancerous part of the esophagus and nearby lymph nodes. This procedure is performed only on patients with very early cancer that has not spread to the stomach. Esophagogastrectomy is the removal of the cancerous part of the esophagus, nearby lymph nodes, and the upper part of the stomach. The resected esophagus is replaced with the stomach or parts of intestine so the patient can swallow. These procedures can significantly relieve symptoms and improve the nutritional status of more than 80% of patients with dysphagia. Although surgery can cure some patients whose disease has not spread beyond the esophagus, but more than 75% of esophageal cancers have spread to other organs before being diagnosed. Less extensive surgical procedures can be used for palliation.

CHEMOTHERAPY Oral or intravenous chemotherapy alone will not cure esophageal cancer, but pre-operative treatments can shrink tumors and increase the probability that cancer can be surgically eradicated. Palliative chemotherapy can relieve symptoms of advanced cancer but will not alter the outcome of the disease.

Radiation

External beam or internal radiation, delivered by machine or implanted near cancer cells inside the body, is only rarely used as the primary form of treatment. Post-operative radiation is sometimes used to kill cancer cells that couldn't be surgically removed. Palliative radiation is effective in relieving dysphagia in patients who cannot be cured. However, radiation is most useful when combined with chemotherapy as either the definitive treatment or preoperative treatment.

Palliation

In addition to surgery, chemotherapy, and radiation, other palliative measures can provide symptomatic relief. Dilatation of the narrowed portion of the esophagus with soft tubes can provide short-term relief of dysphagia. Placement of a flexible, self-expanding stent within the narrowed portion is also useful in allowing more food intake.

Follow-Up Treatments

Regular barium swallows and other imaging studies are necessary to detect recurrence or spread of disease or new tumor development.

Prognosis

Since most patients are diagnosed when the cancer has spread to lymph nodes or other structures, the prognosis for esophageal cancer is poor. Generally, no more than half of all patients are candidates for curative treatment. Even if cure is attempted, the cancer can recur.

Alternative and Complementary Therapies

Photodynamic therapy (PDT) involves intravenously injecting a drug that is absorbed by cancer cells and kills them after they are exposed to specific laser beams. PDT can be used for palliation, but it also cured some early esophageal cancers during preliminary studies. Researchers are comparing its benefits with those of more established therapies.

Endoscopic laser therapy involves delivering short, powerful laser treatments to the tumor through an endoscope. It can improve dysphagia, but multiple treatments are required, and the benefit is seldom long-lasting.

Coping With Cancer Treatment

Many cancer patients have found it helpful to discuss cancer and treatment with other cancer patients and survivors in support groups. Guidance from a nutritionist may be helpful to maintain a balanced diet and to ensure that the patient is receiving adequate nutritional support. The hospital staff and treatment team may be valuable resources for locating support groups and other community resources.

Clinical Trials

Researchers are searching more effective chemotherapeutic agents and radiation treatment regimens. Many studies are aimed at defining the most beneficial combination of surgery, chemotherapy, and radiation in the treatment of esophageal cancer.

Prevention

There is no known way to prevent esophageal cancer.

Resources

Books

Heitmiller, Richard F., Arlene A. Forastiere, and Lawrence R. Kleinberg. "Esophagus." In Clinical Oncology, edited by Martin D. Abeloff, 2nd ed. New York: Churchill Livingstone, 2000, pp.1517–1539.

Zwischenberger, Joseph B., Scott K. Alpard, and Mark B. Orringer. "Esophageal Cancer." In Sabiston Textbook of Surgery, edited by Courtney Townsend Jr. 16th ed. Philadelphia: W.B. Saunders Company, 2001, pp.731–749.

Organizations

American Cancer Society. "Esophageal Cancer." [cited July 6, 2001]. .

National Coalition for Cancer Survivorship. 1010 Wayne Avenue, 5th Floor, Suite 300, Silver Spring, MD 20910. Telephone: 1-888-650-9127.

—Maureen Haggerty; Kevin O. Hwang, M.D.

Wikipedia: Esophageal cancer
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Esophageal cancer
Classification and external resources

Endoscopic image of patient with esophageal adenocarcinoma seen at gastro-esophageal junction.
ICD-10 C15.
ICD-9 150
MeSH D004938

Esophageal cancer is malignancy of the esophagus. There are various subtypes, primarily adenocarcinoma (approx. 50-80% of all Esophageal cancer) and squamous cell cancer. Squamous cell cancer arises from the cells that line the upper part of the esophagus. Adenocarcinoma arises from glandular cells that are present at the junction of the esophagus and stomach.[1] Esophageal tumors usually lead to dysphagia (difficulty swallowing), pain and other symptoms, and are diagnosed with biopsy. Small and localized tumors are treated surgically with curative intent. Larger tumors tend not to be operable and hence are treated with Palliative care; their growth can still be delayed with chemotherapy, radiotherapy or a combination of the two. In some cases chemo- and radiotherapy can render these larger tumors operable. Prognosis depends on the extent of the disease and other medical problems, but is fairly poor.[2]

Contents

Signs and symptoms

Dysphagia (difficulty swallowing) is the first symptom in most patients. Odynophagia (painful swallowing) may be present. Fluids and soft foods are usually tolerated, while hard or bulky substances (such as bread or meat) cause much more difficulty. Substantial weight loss is characteristic as a result of poor nutrition and the active cancer. Pain, often of a burning nature, may be severe and worsened by swallowing, and can be spasmodic in character. An early sign may be an unusually husky or raspy voice.

The presence of the tumor may disrupt normal peristalsis (the organised swallowing reflex), leading to nausea and vomiting, regurgitation of food, coughing and an increased risk of aspiration pneumonia. The tumor surface may be fragile and bleed, causing hematemesis (vomiting up blood). Compression of local structures occurs in advanced disease, leading to such problems as upper airway obstruction and superior vena cava syndrome. Fistulas may develop between the esophagus and the trachea, increasing the pneumonia risk; this condition is usually heralded by cough, fever or aspiration.[2]

If the disease has spread elsewhere, this may lead to symptoms related to this: liver metastasis could cause jaundice and ascites, lung metastasis could cause shortness of breath, pleural effusions, etc.

Causes and risk factors

Increased risk

Barrett's esophagus is considered to be a risk factor for esophageal adenocarcinoma.

There are a number of risk factors for esophageal cancer.[2] Some subtypes of cancer are linked to particular risk factors:

Decreased risk

  • Risk appears to be less in patients using aspirin or related drugs (NSAIDs).[10]
  • The role of Helicobacter pylori in progression to esophageal adenocarcinoma is still uncertain, but, on the basis of population data, it may carry a protective effect.[11][12] It is postulated that H. pylori prevents chronic gastritis, which is a risk factor for reflux, which in turn is a risk factor for esophageal adenocarcinoma.[13]
  • According to the National Cancer Institute, "diets high in cruciferous (cabbage, broccoli, cauliflower) and green and yellow vegetables and fruits are associated with a decreased risk of esophageal cancer."[14]
  • Moderate coffee consumption is associated with a decreased risk.[15]
  • According to one Italian study of "diet surveys completed by 5,500 Italians"—a study which has raised debates questioning its claims among cancer researchers cited in news reports about it—eating pizza more than once a week appears "to be a favorable indicator of risk for digestive tract neoplasms in this population."[16]

Diagnosis

Endoscopy and radial endoscopic ultrasound images of submucosal tumour in mid-esophagus.

Clinical evaluation

Although an occlusive tumor may be suspected on a barium swallow or barium meal, the diagnosis is best made with esophagogastroduodenoscopy (EGD, endoscopy); this involves the passing of a flexible tube down the esophagus and visualising the wall. Biopsies taken of suspicious lesions are then examined histologically for signs of malignancy.

Additional testing is usually performed to estimate the tumor stage. Computed tomography (CT) of the chest, abdomen and pelvis, can evaluate whether the cancer has spread to adjacent tissues or distant organs (especially liver and lymph nodes). The sensitivity of CT scan is limited by its ability to detect masses (e.g. enlarged lymph nodes or involved organs) generally larger than 1 cm. FDG-PET (positron emission tomography) scan is also being used to estimate whether enlarged masses are metabolically active, indicating faster-growing cells that might be expected in cancer. Esophageal endoscopic ultrasound (EUS) can provide staging information regarding the level of tumor invasion, and possible spread to regional lymph nodes.

The location of the tumor is generally measured by the distance from the teeth. The esophagus (25 cm or 10 inches long) is commonly divided into three parts for purposes of determining the location. Adenocarcinomas tend to occur distally and squamous cell carcinomas proximally, but the converse may also be the case.

Histopathology

Most tumors of the esophagus are malignant, only about 0.5% are benign. A very small proportion (under 10%) is leiomyoma (smooth muscle tumor) or gastrointestinal stromal tumor (GIST). Malignant tumors are generally adenocarcinomas, squamous cell carcinomas, and occasionally small-cell carcinomas. The latter share many properties with small-cell lung cancer, and are relatively sensitive to chemotherapy compared to the other types.

Classification

Esophageal cancers are typically carcinomas which arise from the epithelium, or surface lining, of the esophagus. Most esophageal cancers fall into one of two classes: squamous cell carcinomas, which are similar to head and neck cancer in their appearance and association with tobacco and alcohol consumption, and adenocarcinomas, which are often associated with a history of gastroesophageal reflux disease and Barrett's esophagus.

Treatment

Self-expandable metallic stents are used for the palliation of esophageal cancer.

General approaches

Esophageal cancer affecting the lower esophageus. Insets show the tumor in more detail both before and after placement of a stent.

The treatment is determined by the cellular type of cancer (adenocarcinoma or squamous cell carcinoma vs other types), the stage of the disease, the general condition of the patient and other diseases present. On the whole, adequate nutrition needs to be assured, and adequate dental care is vital.

If the patient cannot swallow at all, a stent may be inserted to keep the esophagus patent; stents may also assist in occluding fistulas. A nasogastric tube may be necessary to continue feeding while treatment for the tumor is given, and some patients require a gastrostomy (feeding hole in the skin that gives direct access to the stomach). The latter two are especially important if the patient tends to aspirate food or saliva into the airways, predisposing for aspiration pneumonia.

Tumor treatments

Surgery is possible if the disease is localised, which is the case in 20–30% of all patients. If the tumor is larger but localised, chemotherapy and/or radiotherapy may occasionally shrink the tumor to the extent that it becomes "operable"; however, this combination of treatments (referred to as neoadjuvant chemoradiation) is still somewhat controversial in most medical circles. Esophagectomy is the removal of a segment of the esophagus; as this shortens the length of the remaining esophagus, some other segment of the digestive tract (typically the stomach or part of the colon) is pulled up to the chest cavity and interposed.[17] If the tumor is unresectable or the patient is not fit for surgery, palliative esophageal stenting can allow the patient to tolerate soft diet.

Endoscopic Therapy for Localized Disease There is accumulating data that endoscopic therapy is a safe, less invasive, and effective therapy for very early esophageal cancer. The candidates for endoscopic therapy are Stage 1 patients with tumors invading into the lamina propria (T1 mucosal) or submucosa (T1 submucosal) that do not have regional or distant metastasis. Patients with carcinoma in-situ or high-grade dysplasia can also be treated with endoscopic therapy. Submucosa cancers with increased risk of nodal metastases may not be as amenable to curative therapy.

The two forms of endoscopic therapy that have been used for Stage 0 and I disease are endoscopic mucosal resection (EMR) and mucosal ablation using photodynamic therapy , Nd-YAG laser, or argon plasma coagulation.

EMR Endoscopic Mucosal Resection has been advocated for early cancers (that is, those that are superficial and confined to the mucosa only) and has been shown to be a less invasive, safe, and highly effective nonsurgical therapy for early squamous cell esophageal cancer. Preliminary reports also suggest its safety and efficacy for early adenocarcinoma arising in Barrett’s esophagus. The prognosis after treatment with endoscopic mucosal resection is comparable to surgical resection. This technique can be attempted in patients, without evidence of nodal or distant metastases, with differentiated tumors that are slightly raised and less than 2 cm in diameter, or in differentiated tumors that are ulcerated and less than 1 cm in diameter. The most commonly employed modalities of endoscopic mucosal resection include strip biopsy, double-snare polypectomy, resection with combined use of highly concentrated saline and epinephrine, and resection using a cap.

The strip biopsy method for endoscopic mucosal resection of esophageal cancer is performed with a double-channel endoscope equipped with grasping forceps and snare. After marking the lesion border with an electric coagulator, saline is injected into the submucosa below the lesion to separate the lesion from the muscle layer and to force its protrusion. The grasping forceps are passed through the snare loop. The mucosa surrounding the lesion is grasped, lifted, and strangulated and resected by electrocautery. The endoscopic double-snare polypectomy method is indicated for protruding lesions. Using a double-channel scope, the lesion is grasped and lifted by the first snare and strangulated with the second snare for complete resection.

Endoscopic resection with injection of concentrated saline and epinephrine is carried out using a double-channel scope. The lesion borders are marked with a coagulator. Highly concentrated saline and epinephrine are injected (15–20 ml) into the submucosal layer to swell the area containing the lesion and elucidate the markings. The mucosa outside the demarcated border is excised using a high-frequency scalpel to the depth of the submucosal layer. The resected mucosa is lifted and grasped with forceps, trapping and strangulating the lesion with a snare, and then resected by electrocautery.

A fourth method of endoscopic mucosal resection employs the use of a clear cap and prelooped snare inside the cap. After insertion, the cap is placed on the lesion and the mucosa containing the lesion is drawn up inside the cap by aspiration. The mucosa is caught by the snare and strangulated, and finally resected by electrocautery. This is called the "band and snare" or "suck and cut" technique. The resected specimen is retrieved and submitted for microscopic examination for determination of tumor invasion depth, resection margin, and possible vascular involvement. The resulting "ulcer" heals within 3 weeks.

Although most lesions treated in the esophagus have been early squamous cell cancers, endoscopic snare resection can also be used to debulk or completely treat polypoid dysplastic or malignant lesions in Barrett’s esophagus. In a preliminary report from Germany, EMR was performed as primary treatment or adjunctive therapy following photodynamic therapy for early adenocarcinomas in Barrett's esophagus. The "suck and cut" technique (with and without prior saline injection) was used as well as the "band and cut" technique. Although all tumors were resected without difficulty, 12.5% developed bleeding (which was managed successfully by endoscopic therapy). Eighty-one percent of the lesions were completely resected. The other lesions were also treated with other endoscopic techniques. While this report suggests it is feasible to completely resect local, circumscribed, early adenocarcinomas arising in Barrett's esophagus, the relative safety and efficacy of EMR in conjunction with photodynamic therapy is unknown.

The major complications of endoscopic mucosal resection include postoperative bleeding and perforation and stricture formation. During the procedure, an injection of 100,000 times diluted epinephrine into the muscular wall, along with high frequency coagulation or clipping can be applied to the bleeding point for hemostasis. It is important to administer acid-reducing medications to prevent postoperative hemorrhage. Perforation may be prevented with sufficient saline injection to raise the mucosa containing the lesion. The "non-lifting sign" and complaints of pain when the snare strangulates the lesion are contrainidications of EMR. When perforation is recognized immediately after a procedure, the perforation should be closed by clips. Surgery should be considered in cases of endoscopic closure failure. The incidence of complication range from 0–50% and squamous cell recurrence rates range from 0–8%.

Laser therapy is the use of high-intensity light to destroy tumor cells; it affects only the treated area. This is typically done if the cancer cannot be removed by surgery. The relief of a blockage can help to reduce dysphagia and pain. Photodynamic therapy (PDT), a type of laser therapy, involves the use of drugs that are absorbed by cancer cells; when exposed to a special light, the drugs become active and destroy the cancer cells.

Chemotherapy depends on the tumor type, but tends to be cisplatin-based (or carboplatin or oxaliplatin) every three weeks with fluorouracil (5-FU) either continuously or every three weeks. In more recent studies, addition of epirubicin (ECF) was better than other comparable regimens in advanced nonresectable cancer.[18] Chemotherapy may be given after surgery (adjuvant, i.e. to reduce risk of recurrence), before surgery (neoadjuvant) or if surgery is not possible; in this case, cisplatin and 5-FU are used. Ongoing trials compare various combinations of chemotherapy; the phase II/III REAL-2 trial – for example – compares four regimens containing epirubicin and either cisplatin or oxaliplatin and either continuously infused fluorouracil or capecitabine.

Radiotherapy is given before, during or after chemotherapy or surgery, and sometimes on its own to control symptoms. In patients with localised disease but contraindications to surgery, "radical radiotherapy" may be used with curative intent.

Follow-up

Patients are followed up frequently after a treatment regimen has been completed. Frequently, other treatments are necessary to improve symptoms and maximize nutrition.

Prognosis

In general, the prognosis of esophageal cancer is quite poor, because so many patients present with advanced disease: The overall five-year survival rate (5YSR) is less than 5%. Individualized prognosis depends largely on stage. Those with cancer restricted entirely to the esophageal mucosa have about an 80% 5YSR, but submucosal involvement brings this down to less than 50%. Extension into the muscularis propria (muscular layer of the esophageus) has meant a 20% 5YSR and extension to the structures adjacent to the esophagus results in a 7% 5YSR. Patients with distant metastases (who are not candidates for curative surgery) have a less than 3% 5YSR. Of all patients undergoing surgery with curative intent, the 5YSR is only about 25%.[citation needed] But these statistics are getting better, as more patients are getting diagnosis earlier because of the awareness of Barrett's Esophagus.

Epidemiology

Esophageal cancer is a relatively rare form of cancer, but some world areas have a markedly higher incidence than others: China, Iceland, India and Japan, as well as the United Kingdom, appear to have a higher incidence, as well as the region around the Caspian Sea.[19]

The American Cancer Society estimates that during 2007, approximately 15,560 new esophageal cancer cases will be diagnosed in the United States.[20]

The esophageal cancer incidence and mortality rates for people of African-American descent have been higher than the rate for Caucasians.[21] According to the NCI, incidence of adenocarcinoma of the esophagus, which is associated with Barrett's esophagus, is rising in the United States. This type is more common in Caucasian men over the age of 60.

Multiple reports indicate that esophageal adenocarcinoma incidence has increased during the past 20 years, especially in non-Hispanic white men. Esophageal adenocarcinoma age-adjusted incidence increased in New Mexico from 1973 to 1997. This increase was found in non-Hispanic whites and Hispanics and became predominant in non-Hispanic whites.[22]

References

  1. ^ Esophageal cancer at Mount Sinai Hospital
  2. ^ a b c d e Enzinger PC, Mayer RJ (2003). "Esophageal cancer". N. Engl. J. Med. 349 (23): 2241–52. doi:10.1056/NEJMra035010. PMID 14657432. 
  3. ^ a b Lagergren J, Bergström R, Lindgren A, Nyrén O (1999). "Symptomatic gastroesophageal reflux as a risk factor for esophageal adenocarcinoma". N. Engl. J. Med. 340 (11): 825–31. doi:10.1056/NEJM199903183401101. PMID 10080844. 
  4. ^ Syrjänen KJ (2002). "HPV infections and oesophageal cancer". J. Clin. Pathol. 55 (10): 721–8. doi:10.1136/jcp.55.10.721. PMID 12354793. http://jcp.bmj.com/cgi/content/full/55/10/721.  Full text at PMC: 1769774
  5. ^ Green PH, Fleischauer AT, Bhagat G, Goyal R, Jabri B, Neugut AI (2003). "Risk of malignancy in patients with celiac disease". Am. J. Med. 115 (3): 191–5. doi:10.1016/S0002-9343(03)00302-4. PMID 12935825. 
  6. ^ Merry AH, Schouten LJ, Goldbohm RA, van den Brandt PA (2007). "Body Mass Index, height and risk of adenocarcinoma of the oesophagus and gastric cardia: a prospective cohort study". Gut 56: 1503. doi:10.1136/gut.2006.116665. PMID 17337464. 
  7. ^ Layke JC, Lopez PP (2006). "Esophageal cancer: a review and update". American family physician 73 (12): 2187–94. PMID 16836035. 
  8. ^ Brooks PJ, Enoch MA, Goldman D, Li TK, Yokoyama A (2009). "The alcohol flushing response: An unrecognized risk factor for esophageal cancer from alcohol consumption". PLOS Medicine 6 (3): 191–5. doi:10.1371/journal.pmed.1000050. 
  9. ^ Park W, Vaezi M (2005). "Etiology and pathogenesis of achalasia: the current understanding". Am J Gastroenterol 100 (6): 1404–14. doi:10.1111/j.1572-0241.2005.41775.x. PMID 15929777. 
  10. ^ Corley DA, Kerlikowske K, Verma R, Buffler P. Protective association of aspirin/NSAIDs and esophageal cancer: a systematic review and meta-analysis. Gastroenterology 2003;124:47–56. PMID 12512029. See also NCI - "Esophageal Cancer (PDQ): Prevention".
  11. ^ Wong A, Fitzgerald RC (2005). "Epidemiologic risk factors for Barrett's esophagus and associated adenocarcinoma". Clin. Gastroenterol. Hepatol. 3 (1): 1–10. doi:10.1016/S1542-3565(04)00602-0. PMID 15645398. 
  12. ^ Ye W, Held M, Lagergren J, et al. (2004). "Helicobacter pylori infection and gastric atrophy: risk of adenocarcinoma and squamous-cell carcinoma of the esophagus and adenocarcinoma of the gastric cardia". J. Natl. Cancer Inst. 96 (5): 388–96. doi:10.1093/jnci/djh057. PMID 14996860. http://jnci.oxfordjournals.org/cgi/content/full/96/5/388. 
  13. ^ Nakajima S, Hattori T (2004). "Oesophageal adenocarcinoma or gastric cancer with or without eradication of Helicobacter pylori infection in chronic atrophic gastritis patients: a hypothetical opinion from a systematic review". Aliment. Pharmacol. Ther. 20 Suppl 1: 54–61. doi:10.1111/j.1365-2036.2004.01975.x. PMID 15298606. http://www.blackwell-synergy.com/doi/full/10.1111/j.1365-2036.2004.01975.x. 
  14. ^ NCI Prevention: Dietary Factors, based on Chainani-Wu N. Diet and oral, pharyngeal, and esophageal cancer. Nutr Cancer 2002;44:104–26. PMID 12734057.
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