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Esophageal cancer is malignancy of the esophagus. There are various subtypes. Esophageal tumors usually lead to dysphagia (difficulty swallowing), pain and other symptoms, and are diagnosed with biopsy. Small and localized tumors are treated with surgery, and advanced tumors are treated with chemotherapy, radiotherapy or combinations. Prognosis depends on the extent of the disease and other medical problems, but is fairly poor.
Esophageal cancers are typically carcinomas, which arise from the epithelium, or surface lining of the esophagus. Most esophageal cancer 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.
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 superior vena cava syndrome. Fistulas may develop between the esophagus and the trachea, increasing the pneumonia risk; this symptom is usually heralded by cough, fever or aspiration.
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
There are a number of risk factors for esophageal cancer. Some subtypes of cancer are linked to particular risk factors:
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.
Most tumors of the esophagus are malignant. 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.
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.
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 distance between the throat and the stomach, some other segment of the digestive tract (typically the stomach or part of the colon) is placed in the chest cavity and interposed. If the tumor is metastatic, surgical resection is not considered worthwhile, but palliative surgery may offer some benefit.
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. 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.
Patients are followed up frequently after a treatment regimen has been completed. Frequently, other treatments are necessary to improve symptoms and maximize nutrition.
The prognosis of esophageal cancer is quite poor. Even in patients who undergo surgery with curative intent, the five year survival rate is only 25%. The prognosis is even more dismal in those who are not fit for surgery.
Esophageal cancer is a relatively rare form of cancer, but some world areas have a markedly higher incidence than others: China, India and Japan, as well as the United Kingdom, appear to have a higher incidence, as well as the region around the Caspian Sea.
The American Cancer Society estimates that during 2007, approximately 15,560 new esophageal cancer cases will be diagnosed in the United States.
The esophageal cancer incidence and mortality rates for people of African-Americans have been higher than the rate for Caucasians. 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 yr, 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.
|Other/general||Appendicitis - Peritonitis (Spontaneous bacterial peritonitis)
postprocedural: Gastric dumping syndrome - Postcholecystectomy syndromebleeding: Hematemesis - Melena - Gastrointestinal bleeding (Upper, Lower)
|See also congenital|
Pathology: Cancer, Tumors, neoplasia, and oncology (C00-D48, 140-239)
|Benign tumors||Hyperplasia - Cyst - Pseudocyst - Hamartoma - Benign tumor|
|Malignant progression||Dysplasia - Carcinoma in situ - Cancer - Metastasis|
|Topography||lip, oral cavity and pharynx: Oral - Head/Neck - Nasopharyngeal Thyroid (Papillary, Follicular, Medullary, Anaplastic) - Adrenal tumor (Adrenocortical carcinoma, Pheochromocytoma) - Pituitary|
|Misc.||Tumor suppressor genes/oncogenes - Staging/grading - Carcinogenesis - Carcinogen - Research - Paraneoplastic syndrome - List of oncology-related terms|