Oesophageal cancer is the 14th most common malignancy in adults in the UK.
The oesophagus is a muscular tube that is situated within the thorax and runs from the pharynx to stomach. It is pivotal in the transfer of food material to the stomach and broadly divided into upper, middle and lower.
There are two major types of cancer that arise from the oesophagus, depending on the cell of origin.
Rarer forms of oesophageal cancers include small cell carcinoma, sarcoma, lymphoma, melanoma and choriocarcinoma.
The hallmark clinical feature of oesophageal cancer is dysphagia, which refers to difficulty swallowing. This is due to obstruction of the oesophageal lumen.
The incidence of oesophageal adenocarcinoma, particularly at the gastro-oesophageal junction, has increased dramatically.
Oesophageal cancer is rare in young people and more common as we age. The peak incidence is seen in the 7-8th decades.
Globally, SCC is the most common cause of oesophageal cancer, but the incidence of AC is increasing, particularly in Western countries. This is due to an increase in AC risk factors such as obesity.
AC is more commonly seen in men, whereas the male-to-female ratio is more equal in SCC.
Smoking and alcohol consumption are the two major risk factors for the development of SCC.
Oesophageal neoplasia develops due to sequential mutations that occur in the oesophageal epithelium, which allows cells to proliferate uncontrollably. Several risk factors for both SCC and AC increase the likelihood of developing these mutations.
The biggest risk factors for development of SCC include alcohol consumption and smoking.
The majority of AC cases arise from Barrett’s oesophagus, which refers to columnar metaplasia of the lower oesophagus due to chronic reflux. This a pre-malignant lesion.
The majority of oesophageal SCCs arise from the mid-oesophagus and ACs from near the gastro-oesophageal junction (GOJ).
The majority of SCCs occur due to chronic alcohol consumption and smoking, which damage cellular DNA. This leads to development of genetic mutations that promote abnormal cell growth. Overtime, the cells proliferate uncontrollably leading to invasive cancer.
SCC may be seen as an infiltrating and ulcerated mass in the middle oesophagus, especially if advanced. There is early invasion into surrounding lymph nodes and the tumour may metastasize to liver, bone and lung.
Typically, chronic reflux leads to inflammation and damage of the lower oesophageal mucosa. This results in columnar metaplasia, which refers to the transformation of the mature squamous cell type to columnar cell type.
This metaplastic epithelium may become dysplastic, which refers to cells with abnormal growth and development. The dysplastic epithelium acquires further genetic mutation that promotes development of invasive carcinoma. AC is most commonly located near the GOJ and there is usually early lymph node involvement.
The hallmark feature of oesophageal cancer is dysphagia, which refers to difficulty swallowing.
Early recognition and diagnosis is essential to improve mortality.
The NICE (NG12) guideline outlines recommendations for the referral of suspected cancer cases including upper gastrointestinal cancers. Below details the recommended referral for suspected oesophageal cancer.
This means referring a patient for appropriate investigations (e.g. gastroscopy) for suspected oesophageal cancer within two weeks. It is usually combined with a clinic appointment and CT imaging.
This means referral for a non-urgent gastroscopy to assess for oesophageal pathology. Usually performed within 6 weeks.
NOTE: Upper and lower gastrointestinal (GI) investigations should also be considered to investigate for GI malignancy (inc. oesophageal cancer) in all postmenopausal female and male patients where IDA has been confirmed unless there is a history of significant overt non-GI blood loss. British Society of Gastroenterology guidelines 2011.
Oesophageal cancer is diagnosed using upper GI endoscopy and biopsies of suspected lesions.
The principle test for the diagnosis of oesophageal cancer is an upper GI endoscopy known as a gastroscopy. This a camera test that allows direct visualisation of the upper gastrointestinal tract including oesophagus and gastro-oesophageal junction.
Biopsies are taken at the time of endoscopy of any suspicion lesions. These are subsequently sent for histological analysis to see if any features of malignancy are present.
Further investigations allow assessment of distant spread and key-organ function to help guide management.
Human epidermal growth factor receptor 2 (HER2) testing should be completed on tumour or biopsy specimens. Targeted therapy against the HER2 receptor may be offered to patients with HER2 positive metastatic oesophageal cancer.
The stage of a cancer describes the extent of cancer spread.
The stage of a cancer is critical to determine treatments, but can be very complex and important mainly for clinicians involved in treating patients with cancer. Stage also provides prognostic information.
Cancer stage is based on tumour size, presence of lymph node involvement and distant spread. We call these three factors ‘TNM’ (tumour, nodes, metastasis).
The management of oesophageal cancer depends on the extent of cancer and patient fitness.
There are numerous treatment options for the management of oesophageal cancer:
The choice of treatment depends on whether the cancer is limited, locally advanced or advanced/metastatic.
The treatment of choice for limited disease is surgical or endoscopic resection.
Surgical resection, radiotherapy, chemotherapy, or a combination, may be used for patients with limited or locally advanced disease.
Surgical resection of oesophageal cancer is the treatment of choice if the patient is fit to undergo an operation and the disease is limited. Surgery may be an option for patients with locally advanced oesophageal cancer. This can be considered following systemic anti-cancer therapy (e.g. chemoradiotherapy). The idea is to shrink the tumour, which then becomes resectable.
The surgical technique of choice is an oesophagectomy, which refers to removal of part of the oesophagus and subsequent joining of the remaining oesophagus to the stomach. If the tumour involves the GOJ or proximal stomach then an oesophago-gastrectomy or extended total gastrectomy may be undertaken. In addition to surgical removal of the oesophagus, lymph node dissection may be considered at the time of surgery.
Surgical resection is an option for:
Patients with limited disease may be suitable for endoscopic therapy to remove the oesophageal cancer.
Two options include:
These are typically performed in specialist upper gastrointestinal centres any may require preoperative assessment with endoscopic ultrasound (EUS) to ensure no localised lymph node spread.
The two main systemic anti-cancer therapies used in both SCC and AC are chemotherapy and radiotherapy.
Palliative treatment should be considered in patients with locally advanced disease who are not operative candidates or not fit enough to undergo radical treatment (e.g. poor performance status, extensive co-morbidities).
NOTE: oesophageal stenting involves endoscopically placing a stent within the oesophagus to keep the lumen open and prevent dysphagia.
Patients being considered for radical treatment (e.g. surgical resection or chemoradiotherapy) need to have their nutrition optimised. This may mean temporary enteral or parenteral nutrition.
In patients undergoing chemoradiotherapy, they should be considered for enteral nutrition with placement of a radiologically inserted gastrostomy (RIG) tube. This is because chemoradiotherapy will damage the oesophagus leading to localised inflammation during treatment that will impair nutritional intake. A percutaneous endoscopic gastrostomy (PEG) is not suitable in these cases due to the risk of tumour migration from endoscopic pull through of the tube.
Patients with metastatic disease may be offered a number of palliative treatment options.
Treatment options in patients with advanced oesophageal cancer depends on their symptomatology, fitness (measured by performance status) and personal choice.
This describes a patients' level of functioning in terms of their ability to care for themselves, what they can do as part of their daily activities and their physical ability.
Performance status can be measures using the World Health Organisation (WHO) / Eastern Cooperative Oncology Group (ECOG) scale.
The five year survival of oesophageal cancer is poor at ~16%
Survival from oesophageal cancer depends on stage. The five year survival for stage 1 disease is 52.8% but only 16% for stage 3 disease. Early diagnosis and treatment is potentially curative, especially if the disease is resectable. However, oesophagectomy is a major operation with significant morbidity and mortality.
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