The clinical features commonly seen in the patients of esophageal carcinoma include the following:

• Progressive dysphagia: Dysphagia in esophageal carcinomas is at first intermittent and then becomes constant. Initially, there is difficulty in swallowing of solid foods only but later on when the tumor enlarges, there is difficulty in the swallowing of liquids as well.

• Discomfort: There is discomfort at the site of the obstruction which results from impaction of food. As expected, the discomfort increases with food intake.

• Weight loss: Weight loss in the patients of esophageal carcinoma is due to dysphagia and anorexia.

• Fistula formation: Fistula formation between the esophagus and the trachea results in coughing, pneumonia and pleural effusion.

Esophageal carcinoma frequently spreads by the following routes:

• Direct invasion of the surrounding structures and involvement of related lymph nodes is common as compared to widespread metastasis.

• Spread from the middle third of the esophagus is to the lymph nodes, larynx, trachea and recurrent laryngeal nerve.

• From the lower third of the esophagus, the spread is to the lymph nodes below the diaphragm and the mediastinal lymph nodes.

• Distant metastasis of esophageal carcinoma involve the lungs, liver and bones.

• Weight Loss

  The presence of esophageal cancer in these 3 patients years after the weight loss operation is worrisome. [ncbi.nlm.nih.gov]

  Weight loss: Weight loss in the patients of esophageal carcinoma is due to dysphagia and anorexia. Fistula formation: Fistula formation between the esophagus and the trachea results in coughing, pneumonia and pleural effusion. [symptoma.com]

  Both of our patients had common symptoms of progressive dysphagia and significant weight loss at presentation. [journals.lww.com]

• Anemia

  Carcinoma of the Esophagus • Predisposing factors • Men>women • Achalasia (polypoid mass in middle or distal third) • Asbestosis • Plummer-Vinson syndrome (iron deficiency anemia, webs) • Barrett esophagus (columnar metaplasia of the distal esophagus [learningradiology.com]

  […] poverty, caustic esophageal injury, achalasia, tylosis (a genetic disorder characterized by hyperkeratosis of the palms and soles and leukoplakia in the mouth), Plummer-Vinson syndrome (a condition occurring in patients with long-standing iron-deficiency anemia [pathologystudent.com]

  Esophageal Cancer Esophageal cancer can be associated with a variety of symptoms, including: Difficulty swallowing solid or liquid materials (dysphagia) Indigestion Heartburn Vomiting of blood Chest pain/discomfort Regurgitation of food Weight loss Anemia [nyp.org]

  Cancers may be discovered incidentally when patients are being evaluated for occult bleeding or iron deficiency anemia. [clinicaladvisor.com]

  Esophageal cancer may be associated with several genetic syndromes including Tylosis with non-epidermolytic Palmoplantar Keratoderma, Howel Evans’ syndrome, familial Barrett’s esophagus, Bloom syndrome and Fanconi anemia. [oncolink.org]

• Malnutrition

  It can lead to regurgitation of food, weight loss, physical wasting, and malnutrition. [medical-dictionary.thefreedictionary.com]

• Cough

  Cough and aspiration pneumonia in case of esophageal bronchial fistula. Accompanying B-symptoms (weight loss, night sweats and fever) may occur. [lecturio.com]

  In addition symptoms may include pain when swallowing, a hoarse voice, enlarged lymph nodes around the collarbone, a dry cough, and possibly coughing up or vomiting blood. [2] The two main sub-types of esophageal caecinoma are squamous-cell carcinoma [article.sapub.org]

  Fistula formation: Fistula formation between the esophagus and the trachea results in coughing, pneumonia and pleural effusion. [symptoma.com]

  Note any chronic coughing and increased oral secretions. Listen to the patient’s voice: Tumors in the upper esophagus can involve the larynx and cause hoarseness. [medical-dictionary.thefreedictionary.com]

  However, as the cancer grows, symptoms may include painful or difficult swallowing, weight loss and coughing up blood. [icd10data.com]

• Hoarseness

  Herein, we present a rare case of a 44-year-old male patient with squamous cell carcinoma of the esophagus associated with the clinical symptoms of progressive dysphagia and hoarseness in the course of 2 days. [dovepress.com]

  Among the postoperative complications are: anastomotic leakage, interponate necrosis, stenosis, bleeding, chylothorax (injury of the thoracic duct) and hoarseness (injury of the recurrent laryngeal nerve). [lecturio.com]

  Fine-needle aspiration - of any palpable cervical lymph node; ± Bronchoscopy - in high oesophageal tumours or if hoarseness or haemoptysis is present. [patient.info]

  Symptoms Pain and difficulties with swallowing food, especially solid food Heartburn Hoarseness in voice Pain behind the breastbone Weight loss Indigestion and regurgitation of undigested food Vomiting blood or passing old blood with bowel movements Diagnosis [cancer-symptoms.com]

• Pneumonia

  Radiation pneumonia was evaluated according to Radiation Therapy Oncology Group's acute radiation pneumonia standards, with the endpoint being grade 2 or above radiation pneumonia. [ncbi.nlm.nih.gov]

  Cough and aspiration pneumonia in case of esophageal bronchial fistula. Accompanying B-symptoms (weight loss, night sweats and fever) may occur. [lecturio.com]

  Fistula formation: Fistula formation between the esophagus and the trachea results in coughing, pneumonia and pleural effusion. [symptoma.com]

• Dyspnea

  Malignant pleural effusions or pulmonary metastasis may cause dyspnea. Intraluminal tumor involvement may cause odynophagia, vomiting, hematemesis, melena, iron deficiency anemia, aspiration, and cough. [merckmanuals.com]

  Lung metastasis can cause, among other symptoms, impaired breathing due to excess fluid around the lungs ( pleural effusion ), and dyspnea (the feelings often associated with impaired breathing). [en.wikipedia.org]

• Stridor

  Double aortic arch (DAA) is an extremely rare vascular malformation which causes tracheal and esophageal compression, resulting in respiratory symptoms such as stridor and wheezing, or feeding problems such as dysphagia, usually during the first few months [ncbi.nlm.nih.gov]

• Dysphagia

  Changes in dysphagia scores and overall survival did not differ significantly in the brachytherapy-alone vs stents-alone comparison. [ncbi.nlm.nih.gov]

  When the lumen diameter is decreased to 13 mm, everyone has solid food or regular diet dysphagia. [gastrointestinalatlas.com]

• Dysphagia

  Changes in dysphagia scores and overall survival did not differ significantly in the brachytherapy-alone vs stents-alone comparison. [ncbi.nlm.nih.gov]

  When the lumen diameter is decreased to 13 mm, everyone has solid food or regular diet dysphagia. [gastrointestinalatlas.com]

• Vomiting

  The common adverse effects were mild to moderate neutropenia (84.2%), grade I-II hand and foot syndrome (51.3%), grade I-II nausea (48.7%), mild epistaxis (30.1%) and mild vomiting (14.5%). Esophageal bleeding occurred in 7.9% of patients. [ncbi.nlm.nih.gov]

  Mallory-Weiss tear A Mallory-Weiss tear results from prolonged and forceful vomiting, coughing or convulsions. [radiologyassistant.nl]

  Hematemesis (vomiting blood). Cough and aspiration pneumonia in case of esophageal bronchial fistula. Accompanying B-symptoms (weight loss, night sweats and fever) may occur. [lecturio.com]

  Weight loss Central chest pain and/or pain behind the breastbone Pain upon swallowing Vomiting Chronic cough and/or hoarseness Symptoms of gastrointestinal bleeding Black stools Vomiting blood or material that looks like coffee grounds What Types of Specialists [emedicinehealth.com]

  Side effects include edema, epithelial desquamation, esophagitis, odynophagia, anorexia, nausea, and vomiting. Although radiation by itself does not cure esophageal cancer, it eases symptoms such as pain, bleeding, and dysphagia. [medical-dictionary.thefreedictionary.com]

• Odynophagia

  She was admitted to our hospital complaining of dysphagia and odynophagia. Upper gastrointestinal endoscopy revealed an ulcerovegetant lesion in the right wall of the esophagus suggesting esophageal carcinoma. [ncbi.nlm.nih.gov]

  Side effects include edema, epithelial desquamation, esophagitis, odynophagia, anorexia, nausea, and vomiting. Although radiation by itself does not cure esophageal cancer, it eases symptoms such as pain, bleeding, and dysphagia. [medical-dictionary.thefreedictionary.com]

  During the early stages of radiation therapy, edema may worsen esophageal obstruction, dysphagia, and odynophagia. This problem may require preradiation dilation and/or placement of a stent. [merckmanuals.com]

• Progressive Dysphagia

  Both of our patients had common symptoms of progressive dysphagia and significant weight loss at presentation. [ncbi.nlm.nih.gov]

  The clinical features commonly seen in the patients of esophageal carcinoma include the following: Progressive dysphagia: Dysphagia in esophageal carcinomas is at first intermittent and then becomes constant. [symptoma.com]

  Herein, we present a rare case of a 44-year-old male patient with squamous cell carcinoma of the esophagus associated with the clinical symptoms of progressive dysphagia and hoarseness in the course of 2 days. [dovepress.com]

  Early-stage cancer is typically asymptomatic; initial symptoms are usually progressive dysphagia, which results from significant encroachment on the lumen, and sometimes chest discomfort. [merckmanuals.com]

• Chest Pain

  A 50-year-old man with esophageal carcinoma developed severe, refractory, retrosternal chest pain. The diagnosis was made four days later when contrast studies showed an intramural dissecting hematoma of the esophagus. [ncbi.nlm.nih.gov]

  Symptoms may include any of the following: Backward movement of food through the esophagus and possibly mouth (regurgitation) Chest pain not related to eating Difficulty swallowing solids or liquids Heartburn Vomiting blood Weight loss Tests used to help [medlineplus.gov]

  The burning chest pain of bile reflux after distal esophagectomy can be more annoying than the original symptom of dysphagia and may require subsequent Roux-en-Y jejunostomy for bile diversion. [merckmanuals.com]

• Heart Disease

  A right aortic arch with mirror-image branching however is almost always associated with congenital heart disease. [radiologyassistant.nl]

• Retrosternal Chest Pain

  A 50-year-old man with esophageal carcinoma developed severe, refractory, retrosternal chest pain. The diagnosis was made four days later when contrast studies showed an intramural dissecting hematoma of the esophagus. [ncbi.nlm.nih.gov]

The following investigations are required when esophageal carcinoma is suspected.

• Esophagoscopic biopsy: Upper gastrointestinal endoscopy is the investigation of choice in mass lesions of the esophagus as it allows biopsy and cytology [7].

• Endoscopic ultrasound: This ultrasound is performed for the assessment of depth of tumor infiltration and for staging lymph node involvement [8] [9].

• Barium swallow: Barium swallow shows a characteristic rat tail appearance (i.e. gradual narrowing at the lower end close to the tumor). This appearance may also be seen in benign strictures, esophagitis and achalasia of the cardia.

• Chest X-ray: This is used to demonstrate metastasis in the lungs [10].

• Ultrasound of the abdomen: Ultrasound of the abdomen is useful in identifying the metastasis of the carcinoma to the liver.

• CT scan: CT scan of the thorax and abdomen are performed to assess local spread or metastasis for the staging of the tumor and also for assessing the possibility of surgery.

• Mediastinal Mass

  This examination proved to be useful in the identification of a paracardiac mediastinal mass. [ncbi.nlm.nih.gov]

• Leukocytosis

  This is the first report of esophageal carcinoma with humoral hypercalcemia and leukocytosis that was successfully treated by an operation. [link.springer.com]

Management of esophageal carcinoma depends upon the age and health of the patient and the stage of the tumor. The treatment consists of a combination of surgery, radiotherapy and chemotherapy.

• Surgery: Surgery is considered in patients in whom the tumor is confined to the esophagus and has not infiltrated outside the esophageal wall. Unfortunately, a large percentage of the tumors have extensive spread and are inoperable.

• Radiotherapy: Squamous cell carcinoma of the upper and middle thirds of the esophagus are treated with high voltage radiotherapy. Adenocarcinoma is less radiosensitive.

• Chemotherapy: Cisplatinum and 5-fluorouracil are being used before surgical resection. Some prolongation of survival has been noted.

• Palliative therapy: Extensive tumors that are unsuitable for radical surgery or intensive radiotherapy are not treated. Instead, palliative therapy given which involves the repeated dilation of the esophagus with insertion of a plastic or metallic tube (stent) with the help of an endoscope. This allows liquids and soft foods to be taken.

The survival of the patient depends upon the stage of the tumor. The five year survival rate for the patients with stage 1 tumor is 80% whereas for stage 2 patients, it is as low as 30%. The 5 year survival rates for patients with stage 3 and 4 tumor are 18% and 4% respectively.

There is no single cause for the development of esophageal carcinoma; however, certain risk factors have been recognized. The risk factors differ for squamous cell carcinoma and adenocarcinoma of the esophagus.

Squamous cell carcinoma

The most notable risk factors that lead to the development of squamous cell carcinoma of the esophagus are listed below.

• Tobacco smoking: An overwhelming amount of evidence has established tobacco smoking to be one of the leading risk factors for the development of squamous cell carcinoma [1].

• Alcohol intake: Heavy alcohol intake has also been linked to an increased incidence of squamous cell carcinoma of the esophagus.

It should be noted that the risk for the development of squamous cell carcinoma of the esophagus in the people who are both smokers and alcoholics is exponentially higher.

• Plummer Vinson syndrome: Plummer Vinson syndrome – a triad of esophageal webs or stenosis, large beefy tongue and cracks at the corners of the mouth – is also associated with an increased risk for the development of esophageal squamous cell carcinoma.

• Achalasia: Long standing achalasia also has the potential to cause squamous cell carcinoma of the esophagus.

• Tylosis: Tylosis is an autosomal disorder characterized by hyperkeratosis of the palms and soles. It has also been linked to an increased risk of squamous cell carcinoma of the esophagus.

• Celiac disease: There is also an increased risk for the development of esophageal squamous cell carcinoma in the people suffering from celiac disease.

• Vitamin deficiency: The deficiency of certain vitamins is also known to be associated with an increased risk for the development of squamous cell carcinoma of the esophagus. Fruits and vegetables (which naturally contain vitamins) decrease the risk for the development of esophageal carcinoma [2].

Adenocarcinoma

The risk factors that are associated with the development of adenocarcinoma of the esophagus include the following:

• Long standing reflux esophagitis: Repeated reflux of the contents of the stomach into the esophagus causes inflammation in the latter and is known as reflux esophagitis. The risk for the development of esophageal adenocarcinoma is higher in the people who suffer from long standing reflux esophagitis.

• Barrett’s esophagus: Barrett’s esophagus refers to the replacement of the normal squamous epithelium of the esophagus with intestinal type columnar epithelium. It is the consequence of gastro-esophageal reflux disease. Barrett’s esophagus poses a very high risk for the development of adenocarcinoma of the esophagus [3].

n the United States, esophageal carcinoma is the seventh most common fatal cancer in males. Two kinds of esophageal carcinomas are commonly seen.

Squamous cell carcinoma is by far the more common of the two. It has a peak incidence over the age of 50 years. It is much more prevalent in males as compared to females - the male to female ratio being 3:1. It is most common in China and Iran.

The incidence of adenocarcinoma is increasing nowadays because of the high prevalence of gastroesophageal reflux disease.

Various risk factors can cause esophageal carcinoma individually or in association with each other. Chronic irritation of the esophagus is the underlying mechanism by which esophageal carcinoma develops.

Tobacco smoking has a highly irritating effect on the esophageal epithelium and so does heavy alcohol intake. The repeated cycles of damage and repair of the esophagus ultimately progress to cancer development.

Similarly, in achalasia, the food stays in contact with the esophageal mucosa for an extended period of time. This causes chronic inflammation and irritation, ultimately leading to the development of squamous cell carcinoma.

Vitamin deficiency is associated with ineffective repair of the damaged epithelium which then leads to an increased risk for the development of esophageal squamous cell carcinoma.

The reflux of the acid contents from the stomach has a highly damaging and irritating effect on the esophagus. Barrett’s esophagus which results from long standing reflux disease is a metaplasia characterized by the replacement of the normal squamous epithelium of the esophagus by columnar epithelium of the intestinal type.

All metaplasias have a tendency to progress to cancer development. Further damage by the reflux of the stomach contents accelerates the development of adenocarcinoma in these patients [4].

Furthermore, mutations in the oncosuppressor gene TP53 also occur which are associated with adenocarcinoma [5] [6].

There are no guidelines for prevention of esophageal carcinoma.

Two types of carcinomas can occur in the esophagus; squamous cell carcinoma and adenocarcinoma. Tobacco smoking and alcohol intake are the commonest causes of the former whereas gastroesophageal reflux disease is the commonest cause of the latter.

Progressive dysphagia and weight loss are the most recognizable symptoms. The prognosis depends upon the stage of the tumor.

Esophageal carcinoma usually occurs in smokers and alcoholics. A variant is also common in the people who suffer from heartburn.

Esophageal carcinoma is characterized by progressive difficulty in swallowing along with discomfort in the chest and weight loss. If detected early and treated properly, the survival rate is very high.

1. Steevens J, Schouten LJ, Goldbohm RA, van den Brandt PA. Alcohol consumption, cigarette smoking and risk of subtypes of oesophageal and gastric cancer: a prospective cohort study. Gut. Jan 2010;59(1):39-48.
2. Steevens J, Schouten LJ, Goldbohm RA, van den Brandt PA. Vegetables and fruits consumption and risk of esophageal and gastric cancer subtypes in the Netherlands Cohort Study. International journal of cancer. Journal international du cancer. Dec 1 2011;129(11):2681-2693.
3. Lagergren J, Bergstrom, R, Lindgren, A, et al. Symptomatic gastroesophageal reflux as a risk factor for esophageal adenocarcinoma. N Engl J Med. 1999;340(11):825-831.
4. Koppert LB, Wijnhoven BP, van Dekken H, Tilanus HW, Dinjens WN. The molecular biology of esophageal adenocarcinoma. Journal of surgical oncology. Dec 1 2005;92(3):169-190.
5. Jankowski JA, Wright NA, Meltzer SJ, et al. Molecular evolution of the metaplasia-dysplasia-adenocarcinoma sequence in the esophagus. The American journal of pathology. Apr 1999;154(4):965-973.
6. Tilanus HW. Changing patterns in the treatment of carcinoma of the esophagus. Scandinavian journal of gastroenterology. Supplement. 1995;212:38-42.
7. Varghese TK Jr, Hofstetter WL, Rizk NP, et al. The society of thoracic surgeons guidelines on the diagnosis and staging of patients with esophageal cancer. The Annals of thoracic surgery. Jul 2013;96(1):346-356.
8. Yasuda K, Kamaguchi M, Morikawa J, Honda M, Nakajima M. Role of endoscopic ultrasonography in the diagnosis of early esophageal carcinoma. Gastrointestinal endoscopy clinics of North America. Jan 2005;15(1):93-99, ix.
9. Dittler HJ, Siewert JR. Role of endoscopic ultrasonography in esophageal carcinoma. Endoscopy. Feb 1993;25(2):156-161.
10. O'Donovan PB. The radiographic evaluation of the patient with esophageal carcinoma. Chest surgery clinics of North America. May 1994;4(2):241-256.