More than 8,500 new cases of oesophageal cancer are diagnosed each year, with the incidence of cancers of the lower oesophagus / gastro-oesophageal junction rinsing faster than any other solid organ tumour. They are 3 times more common in men.
There are two main types of oesophageal cancer:
Squamous cell carcinoma (more common in the developing world) typically occurring 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 occurring 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 rare 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.
However, as the condition progresses, the symptoms that can present include:
- Dysphagia – characteristically progressive, initially being to solids (especially meats or breads) then liquids
- Any patient with dysphagia should be assumed to have oesophageal cancer until proven otherwise.
- 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 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) however this is much less sensitive and specific.
Before undergoing curative treatment, patients often require a variety of the staging investigations including:
- CT Chest-Abdomen-Pelvis and PET-CT scan are used together to investigate for distant metastases
- Endoscopic Ultrasound to measure the penetration into the oesophageal wall (T stage) and assess and biopsy suspicious mediastinal lymph nodes
- 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.
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
- SCCs of the upper oesophagus are technically difficult to operate on and definitive CRT is therefore usually the treatment of choice
- SCCs of the middle or lower oesophagus will warrant either definitive CRT or neoadjuvant CRT followed then by surgery
- Adenocarcinomas– neoadjuvant chemotherapy or chemo-radiotherapy followed by an oesophageal resection
- Patients who are less fit (but still fit enough to undergo surgery) may simply 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 around 4% and it takes 6-9 months for patients to recover to their pre-operative quality of life.
The main complications are anastomotic leak* (8%), re-operation, pneumonia (30%), and death (4%)
Post-operative nutrition is a major problem for these patients as they lose the reservoir function of the stomach. 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
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 a 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 (Fig. 3). 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 Gastrostomy (RIG) tube may need to be inserted, to bypass the obstruction.
The prognosis for oesophageal cancer is generally poor due to late presentation. Overall five-year survival is 5-10%.
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.
- Any patient with dysphagia (difficulty swallowing) has oesophageal cancer until proven otherwise.
- The initial investigation for suspected case is endoscopy
- Only a small proportion of oesophageal cancers are suitable for surgical intervention
- Surgery is a massive undertaking with significant morbidity and complication rates