More than 8,500 new cases of oesophageal cancer are diagnosed each year. More than 80% of oesophageal cancers are diagnosed in people aged >60yrs and it is 3 times more common in men.
There are two main types of oesophageal cancer:
Squamous cell carcinoma (more common in the developing world) typically occurs in the middle and upper thirds of the oesophagus
- Strongly associated with smoking and excessive alcohol consumption, as well as chronic achalasia, low vitamin A levels and, rarely, iron deficiency
- Adenocarcinoma (more common in the developed world) typically occurs in the lower third of the oesophagus
- Arises as a consequence of metaplastic epithelium (termed Barrett’s oesophagus) which progresses to dysplasia, to eventually become malignant.
- Risk factors for this subtype are long-standing GORD, obesity, and high dietary fat intake
Other subtypes of oesophageal malignancy include leiomyosarcoma, rhabdomyosarcoma, or lymphoma.
Early stage oesophageal cancer often lacks well-defined symptoms, which may account for the majority of patients presenting in the later course of the disease. There may be some retrosternal discomfort and dyspepsia, but they are often discounted or ignored by patients, possibly on a background of GORD.
However, as the condition progresses, the symptoms that can present include:
- Dysphagia – characteristically progressive, initially being to solids (especially meats or breads) then liquids
- Significant weight loss – due to both dysphagia and cancer-related anorexia (this is a marker of late-stage disease)
- Other less common symptoms include odonyphagia, productive cough, or hoarseness
NICE guidance states the red-flag symptoms for a suspected oesophageal malignancy requiring urgent endoscopy are:
- Patients with dysphagia
- Any patient >55yrs with weight loss and upper abdominal pain, dyspepsia, or reflux
On clinical examination, patients may have evidence of recent weight loss or cachexia, signs of dehydration, supraclavicular lymphadenopathy, or any signs of metastatic disease (e.g. jaundice, hepatomegaly, or ascites)
There are many causes for dysphagia, as discussed in our dysphagia article. Importantly, the dysphagia should be classified as either a mechanical or neuromuscular disorder, as this can significantly affect future investigations. However, any patient presenting with dysphagia should be assumed to have oesophageal cancer until proven otherwise.
Any patient with a suspected oesophageal malignancy should be offered urgent upper gastrointestinal endoscopy (also termed an oesophago-gastro-duodenoscopy, OGD), to be performed within 2 weeks. Any malignancy seen on OGD will be biopsied and sent for histology.
Patients who are not fit for an OGD can occasionally have a CT scan (neck and thorax) but this is much less sensitive and specific.
Before undergoing curative treatment, patients may require a variety of the following staging investigations:
- CT Chest-Abdomen-Pelvis (or PET-CT scan, as often more sensitive in detecting metastases) is the most definitive first-line staging scan
- Endoscopic Ultrasound to measure the penetration into the oesophageal wall (T stage) and assess and biopsy suspicious mediastinal lymph nodes
- Often used if CT imaging has shown no metastasis, can be useful to determine local disease staging
- Staging laparoscopy (for junctional tumours with an intra-abdominal component) to look for intra-peritoneal metastases
Any palpable cervical lymph nodes may be investigated via Fine Needle Aspiration (FNA) biopsy and any hoarseness or haemoptysis may warrant investigation via bronchoscopy.
Sadly, the majority of patients present with advanced disease. Approximately 70% of patients are therefore only treated palliatively.
As with all cancers, the management of oesophageal cancer patients should be determined by the multidisciplinary team (MDT), with input from general surgeons, oncologists, specialist nurses, nutritionists, and the palliative care team (as necessary).
The choice of curative treatment strategy will depend on tumour type, site and the patient’s general fitness and co-morbidities. For the majority of patients, this comprises surgery with or without neoadjuvant chemotherapy or chemo-radiotherapy (CRT):
- Squamous cell carcinomas – tumours of the upper oesophagus (which are usually SCCs) are technically difficult to operate on and definitive CRT is therefore usually the treatment of choice. For SCCs of the middle or lower oesophagus, either definitive CRT or neoadjuvant CRT followed by surgery can be performed.
- Adenocarcinomas – neoadjuvant chemotherapy or chemo-radiotherapy followed by an oesophageal resection. Some patients who are less fit (but still fit enough to undergo surgery) will receive surgical treatment alone.
Surgical treatment is a major undertaking as both the abdominal and chest cavities need to be opened. Patients have one lung deflated for about 2 hours during surgery; 30-day mortality rates are ~4% and it takes 6-9 months for patients to recover their pre-operative quality of life. The main specific complications are death (4%), anastomotic leak* (8%), re-operation, and pneumonia (30%).
Post-operative nutrition is a major problem for these patients as they lose the reservoir function of the stomach (see below). Many centres will routinely insert a feeding tube into the small bowel (a “feeding jejunostomy”) to aid nutrition. However, most patients will need to eat 5-6 small meals per day and “graze” to meet their nutritional requirements as they physically cannot fit in 3 normal size but intermittent meals.
*Rates of anastomotic leak are relatively high; any deterioration, even minor, in an oesophagectomy patient should be considered to be an anastomotic leak until proven otherwise, requiring resuscitation, placed NBM, and have an urgent CT Chest Abdomen Pelvis with oral contrast for further assessment.
The main surgical management option for oesophageal cancer is an oesophagectomy, with a variety of approaches possible. They all involve removal of the tumour, top of the stomach, and surrounding lymph nodes. The stomach is then made into a tube (“the conduit”) and brought up into the chest to replace the oesophagus. Specific approaches include:
- Right thoracotomy with laparotomy (termed an Ivor-Lewis procedure)
- Right thoracotomy with abdominal incision and neck incision (termed an McKeown procedure)
- Left thoracotomy with or without neck incision
- Left thoraco-abdominal incision (one large incision starting above the umbilicus and extending round the back to below the left shoulder blade)
For a small number of patients with very early cancers or high grade Barrett’s oesophagus, an option is Endoscopic Mucosal Resection (EMR), which is the removal of just the mucosal layer of the oesophagus. EMR can be combined with radiofrequency ablation (RFA) or photodynamic therapy (PDT) afterwards to destroy any malignant cells that may be left.
Those patients deemed too unfit or unsuitable for curative therapy can be offered a range of palliative options.
Patients with difficulty in swallowing should have an oesophageal stent placed where possible. Radiotherapy and/or chemotherapy can be used for palliation to reduce tumour size and bleeding, temporarily improving the patient’s symptoms.
Photodynamic therapy (PDT) is a treatment that uses a photosensitizing agent, that when exposed to a specific wavelength of light produces a form of oxygen that kills nearby cells.
Nutritional support is essential for this patient group, as progression of the disease can lead to significant dysphagia and cachexia. Thickened fluid and nutritional supplements should be offered (usually via the nutrition team). If dysphagia becomes too severe to tolerate enteral feeds, a Radiologically-Inserted Gastrectomy (RIG) tube may need to be inserted, to bypass the obstruction.
The prognosis for oesophageal cancer is generally poor due to late presentation. The outcome of surgically treated patients have survival depending on stage of the disease, with a 5 year survival for stage 1 cancers at around 60%. Palliative treated patients have a median survival of 4 months. Overall five-year survival is 5-10%.
- Squamous cell carcinoma subtypes typically affect the upper and middle third of the oesophagus whilst adenomacarcinoma subtypes typically affect the lower third of the oesophagus
- Endoscopy with biopsy and staging CT scan (or PET scan) are the definitive investigations in most cases
- Only a small proportion of oesophageal cancers are suitable for surgical intervention
- Speech and Language Therapy and Dietician involvement is essential for all cases of oesophageal cancer