Oesophageal Tumours

Original article by Tom Leach | Last updated on 27/5/2014
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Summary

There are 4 types of oesophageal tumour; adenocarcinoma, squamous cell carcinoma, leiomyoma and squamous papilloma. Squamous cell and adenocarcinoma are by far the most common.
 

Squamous cell carcinoma

Epidemiology

  • 2-3 per 100 000 in the UK and USA. Much higher in Asia and the Middle East
  • Male:Female = 2:1
  • Peak age of incidence is 60-70
 

Aetiology

  • Eating certain foods, including; cereals, foods containing nitrous amides
  • Not eating enough of other foods; vitamins A and C, iron
  • Achalasia
  • Chewing tobacco
  • HPV
Most commonly seen in the upper 2/3 of the oesophagus
 

Adenocarcinoma

This is only found in columnar epithelium and thus is strongly associated with Barrett’s Oesophagus.
 

Epidemiology

  • 5 per 100 000 – thus in these regions it is more common than squamous cell carcinoma
  • Incidence has risen greatly in the last few decades – thought to be related to the increase in obesity (and thus the increase in reflux disease)
  • Most common presentation is in white, middle-aged males
 

Aetiology

  • Barrett’s Oesophagus. Risk increases with
    • Length of oesophagus effected
    • Duration of condition
  • Obesity
  • Smoking
  • Alcohol
  • Genetic susceptibility
  • Age (>45)
  • Male
Most commonly seen in the lower 1/3 of the oesophagus
 

Clinical Features of Oesophageal carcinoma

Disease if often very advanced by the time of symptomatic presentation
  • Feeling of lump in the throat – not associated with eating
  • Dysphagia / discomfort on swallowing
  • Progressive dysphagiathis is a very characteristic symptom
  • Regurgitation – may be misinterpreted as vomiting
  • Weight loss and anorexia
  • Possible palpable lymph nodes, such as Virchow’s node.
 

Investigations

  • Endoscopy. Investigation of choice. 90% of oesophageal tumours can be imaged
  • Barium swallow (rare) – may be used if endoscopy cannot be tolerated
 

Staging

Tumours are staged using the TNM staging system (see full article for further details)
 

Treatment

essentially the options are surgery, or palliative treatments.
The 5-year survival rate from presentation is 5-9%
Low grade tumours are resectable, higher grade (i.e. T4 and / or M1)) are non-resectable. At presentation, 40% are resectable. Of these patients, 30% are ‘potentially curable’.
The chance of survival can be increased with adjuvant chemotherapy, and to a lesser extent, adjuvant radiotherapy.
 
Palliative treatment may involve:
  • Stenting to allow swallowing
  • Intubation (in the later stages) to allow adequate nutrition
Most patients die from bronchopneumonia as a result of aspiration (due to dysphagia) or oesophageal-broncho fistulas.
 

Other types of tumour

  • Leiomyoma – a benign tumour of the smooth muscle. May cause obstructive symptoms is large (>5cm), in which case they can be removed surgically
  • Squamous Papilloma – caused by the HPV, they are benign, and very rare

 

More Information

 

The main two types of oesophageal carcinoma are squamous cell carcinoma and adenocarcinoma. Benign tumours account for less than 5% of all oesophageal neoplasms.
Oesophageal cancer is the 9th most common cancer worldwide, and the 6th most likely cancer to cause death.

 

Squamous cell carcinoma

Epidemiology

  • Incidence is about 2-3 per 100 000 in Europe, USA and Australia, however there is the Asian oesophageal cancer belt  across the middle east, and including China and India, where the incidence can be as much as 160 per 100 000. South Africa and Kenya also have high incidence rates.
  • It can arise anywhere from the post cricoid region to just above the cardia, although most are above the bottom 1/3 of the oesophagus.
  • It is much more common in males than in females (2:1)
  • Mainly occurs in those aged 60-70
 

Aetiology

  • The main three contributory factors are smoking, alcohol and diet. The following dietary factors have been specifically linked with squamous cell carcinoma:
    • High intake of nitrosamides – these are often used as food preservatives.
    • Low intake of vitamin A and nictonic acid
    • Iron deficiency anaemia
    • Diet high in cereals
    • Diets high in fruit and vegitables (carotenoids and vit C) reduce the risk.
  • Long standing achalasia - probably because it increases the time possible carcinogens are in contact with the oesophageal mucosa for, as well as causing mucosal irritation.
  • Some evidence suggests that human papilloma virus may be involved, as biopsies from oesophageal cancers often contain this virus. It is therefore possible that some variants of HPV can incorporate themselves into the host genome and bring about oncogenic changes leading to carcinoma. There is a well established link between bovine oesophageal cancer and papilloma virus infection.
  • Coeliac disease will increase the risk
  • Chewing tobacco will also increase the risk.
 
Papilloma – this literally means ‘wart’. The papilloma virus was discovered when it was found that warts could be transferred from person to person. There are more than 100 types of HPV (human papilloma virus); some can cause cancer, others cause warts, whilst others appear to do no harm. They are non-enveloped DNA viruses. It is particularly related to cervical cancer.
 
The virus infects the skin and mucous membranes of the body. Most HPV’s are specially adapted just to affect one part of the body, for example. HPV types I and II affect the soles of the feet and palms of the hands and cause warts. About 30-40 types are sexually transmitted; some of these causing warts whilst others have no noticeable effect.
 
Several types of “high-risk” HPV have been identified - these are the types most likely to cause cancer. Prolonged infection with these types leads to increased risk of carcinoma.
 
Prevalence of HPV in the general population is roughly 25%, although around 90% of people are probably infected at one stage during their lifetime. Prevalence generally decreases with age, and is at its peak at age 20-24.
 
 

Adenocarcinoma

Epidemiology

  • Found in columnar epithelium.
  • Barrett’s oesophagus increases the risk of oesophageal adenocarcinoma by 30-40x
  • Incidence is rising quickly in Europe and the USA.
  • Often a gastric adenocarcinoma can extend into the oesophagus and cause the same symptoms.
  • This is common in the lower 1/3 of the oesophagus, as opposed to squamous cell carcinoma which is usually found above this region.
  • The incidence is approximately 5 per 100 000 in the UK, and is similar in Europe and the USA. In these regions, its incidence often exceeds that of squamous cell carcinoma.
  • In the USA Incidence has risen about 400% since the 1970’s, and surpassed that of squamous cell carcinoma in about 1990. This rise is thought to be linked to reflux disease and obesity.
  • It most commonly presents in white middle-aged males.
  • About 13% of those with Barrett’s oesophagus will have adenocarcinoma. This equates to about a 30x increase in the risk of oesophageal cancer from the general population. However, most patients present with oesophageal carcinoma without a history of Barrett’s.
  • Most patients are over 50 years old and male.
 

Aetiology

  • Barrett’s Oesophagus
  • Obesity
  • Smoking
  • Alcohol
  • Genetic susceptibility
  • If you have over 8cm of Barrett’s oesophagus then you are at greater risk.
  • Length of time of reflux symptoms – the longer the time, the greater the risk.
  • Age (>45)
  • Male
 

Clinical Features

(General – for both types of carcinoma)
Generally the disease is not diagnosed when it is in its early stages because there aren’t many signs and symptoms. When they are present, symptoms will usually be around for 4-6 months, but can be up to 3 years. Patients will often wait 3-4 months before seeking medical help.
  • Early Ill-defined symptoms – these include a feeling of a lump in the throat – not associated with time of eating. Possible slight dysphagia and discomfort. Often the patient has discounted these symptoms as having little meaning.
  • Progressive dysphagia. This is the most important symptom, but it may not be noticed until the oesophageal diameter has reduced by 2/3. Does not regress. Initially the difficulty is only for solids, but later it is also for liquids. In very progressed disease, then the dysphagia can lead to problems swallowing saliva, and aspiration occurs.
  • Benign strictures will also cause dysphagia, but this is more likely to be intermittent.
  • Regurgitation – this is often misinterpreted as vomiting, when in actual fact it is a complication of dysphagia.
  • The lesion usually starts out as an ulcer, but then gets bigger and bigger until it forms a stricture and causes dysphagia.
  • Spread of the tumour is generally by direct invasion of the surrounding tissue. It will first invade the wall of the oesophagus, and then into the trachea wall, bronchi, pericardium, chest wall and diaphragm. Once a fistula has occurred between the oesophagus and an airspace, the cancer in incurable, and life-expectancy is very short.
  • There are extensive lymph nodes around the oesophagus. The spread to these is usually longitudinal and happens quick. Virchow’s node is again a common site, as are the mediastinal nodes and gastric nodes.
  • Weight loss due to dysphagia and anorexia.
 

Investigation

  • Barium swallow – rarely used these days, it may be used in younger patients where history of dysphagia is suggestive of a motility disorder, as it is able to distinguish the two and is less invasive than endoscopy. It is also a good test because it is able to identify the location and length of any oesophageal narrowing, although it cannot tell you if this is due to malignancy or not. A sign that shows advanced oesophageal cancer would be pouching off the main trunk of the oesophagus, and fistula’s to the respiratory tract. It is also often used if you suspect a pharyngeal pouch (because an endoscopy can easily perforate this) or if you suspect the dysphagia is caused by something high up the oesophagus (it is good for imaging things high up.)
  • Endoscopy – this is generally the investigation of choice. 90% of oesophageal carcinoma can be confirmed by endoscopy. Often a biopsy is taken, although in advanced disease (which is when many cases present) then this is not necessary for diagnosis. Sometimes iodine is used to stain the area. In this test, normal oesophagus is stained brown, but cancerous tissue remains unstained. This can help direct the endoscopist to biopsy the correct bit of tissue. This test is only really of use if the disease is not advanced, because in early disease, mucosal lesions may be missed.
  • Further investigations the same as for gastric cancer - although these will rarely be used.
 

Staging

  • CT – this will probably be used to stage the tumour and may be followed up by an endoscopic ultrasound. A CT is good for addressing metastatic spread and local invasion, but may underestimate the spread, and EUSS is more accurate, although as it is relatively new it is not as common.
  • Liver USS – this can measure metastatic spread and may be useful in determining treatment options.
  • PET scan – with 18 – fluro-deoxy-D-glucose is becoming more common. This procedure is able to locate distant metastasis either systemic or nodal. Locally, this is not as accurate as EUSS at detecting spread.
  • The TNM staging scale is used, similar to that for gastric cancer.
N0
No nodes involved
N1
1-6 nodes involved
N2
7-15 nodes involved
N3
15+ nodes involved
 
T1
Tumour is mucosa / submucosa
T2
Tumour in muscularis propria
T3
Tumour through serosa
T4
Tumour invading other structures
 
M0
No distant metastasis
M1
Distant metastasis

Treatment

Surgery
  • Basically after staging, you can divide people into resectable and non-resectable. Those which are non resectable are either at T4, and / or M1.
  • By the time of presentation, only about 40% of patients are resectable – the rest have presented too late and spread to other organs is already evident.
  • The overall survival rate for people presenting with oesophageal cancer is 5-9%.
  • Survival for people who present with ‘potentially curable disease’ - i.e. the tumour has not spread beyond the wall of the oesophagus and you are confident resection will remove all the visible tumour – is about 30% at five years
  • Preliminary research has shown that this can be improved by first giving chemotherapeutic agents such as cisplatin or 5-flurouracil.
  • Radiotherapuetic treatment alone is associated with a 5-year survival of 5%, despite the fact that these tumours are radiosensitive.
  • The surgery can be quite taxing. Often there will be both a laparotomy to remove the bottom part of the resection (and also often some or all of the stomach is removed), and a thoracotomy is needed to remove parts of the oesophagus higher up. This is known as two-field surgery. Once you have removed the bit you want, you basically join together the two ends. This often involved pulling up the stomach to make a ‘new’ oesophagus. Thebe a large dissection of there may also be a large dissection of the mediastinum as many lymph nodes are removed from here.
  • In Japan, often three field surgery is performed – where the cervical lymph nodes are also removed – and so this requires an extra incision in the neck. Some people believe this provides better cure rates as it doesn’t allow / removes any spread to the cervical lymph nodes, although the evidence for this is patchy.
  • In some patients – particularly those who may have had previous gastric surgery, then the colon and jejunum can be used as a replacement for the oesophagus.
 

Complications

  • Pulmonary complications are the most common, followed by cardiac problems. It is beneficial if patients stop smoking, and have chest physiotherapy to remove retained mucous. Retaining fluid is also a problem.
 

Palliative care

  • This is all that can be offered to 60-70% of patients at presentation.
  • Placing of a stent can reduce dysphagia, and the control of diet and pain relief can provide reasonably adequate comfort for most patients.
  • Many patients end up being intubated to receive adequate nutrition.
  • Most patients will die of bronchopneumonia exacerbated by malnutrition.
  • Unlike many forms of cancer, metastases are rarely found at autopsy. I suppose this means that the diseases spreads directly and causes direct damage to surrounding structures (e.g. fistulas to the respiratory system) and these are the things that cause most damage in this disease.
 
 

Other Types of tumours

Leiomyoma
  • This is the most common type of benign tumour. It is a tumour of the smooth muscle of it is not premalignant – there is virtually no evidence to suggest they can become malignant. As well as in the oesophagus, they commonly occur in the small bowel and in the uterus. They are usually solitary and well encapsulated. They grow very slowly and are usually asymptomatic. If they grow about 5cm they may cause dysphagia.
  • If they are above 5cm in size they are usually removed by surgery. Some very large ones may require oesophageal resection, particularly if they are annular (ring shaped)
 
Squamous papilloma
  • Another benign tumour, this is extremely rare. However, they are of interest because like many other squamous papillomas, they are caused by the human papilloma virus.