- 1 Introduction
- 2 Clinical Features
- 3 Differential Diagnosis
- 4 Investigations
- 5 Management
- 6 Prognosis
- 7 Key Points
More than 8,500 new cases of oesophageal cancer are diagnosed each year in the UK, with their incidence rising faster than any other solid organ tumour. They are three times more common in men.
There are two main types* of oesophageal cancer, squamous cell carcinoma and adenocarcinoma
*Other rare subtypes of oesophageal malignancy include leiomyosarcoma, rhabdomyosarcoma, or lymphoma.
Squamous cell carcinoma, which is more common in the developing world, typically occurring in the middle and upper thirds of the oesophagus. This subtype is more associated with smoking and excessive alcohol consumption (other risk factors include chronic achalasia, low vitamin A levels and, rarely, iron deficiency),
Adenocarcinoma, which are more common in the developed world, typically occur in the lower third of the oesophagus. This subtype 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 fat intake
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.
Dysphagia is a common presenting symptom of oesophageal cancer*, typically progressive in nature (classically this starts with solids only, before affecting liquids).
Patients may also report significant weight loss, due to both dysphagia and cancer-related anorexia. Other less common symptoms include odynophagia or hoarseness
On clinical examination, patients may have evidence of recent weight loss or cachexia, signs of dehydration, supraclavicular lymphadenopathy, or any signs of metastatic disease (such as jaundice, hepatomegaly, or ascites)
*Any patient with dysphagia should be assumed to have oesophageal cancer until proven otherwise
Criteria for Upper GI Endoscopy
Current NICE guidance states the red-flag symptoms for a suspected oesophageal malignancy requiring urgent endoscopy are:
- Any patient with dysphagia
- Any patient >55yrs with weight loss and upper abdominal pain, dyspepsia, or reflux
There are many causes for dysphagia. 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, therefore most patients will have an upper GI endoscopy as first line investigation.
Any patient with a suspected oesophageal malignancy should be offered urgent upper GI endoscopy* (also termed 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, if required, the palliative care team.
The choice of curative treatment strategy will depend on tumour type, tumour site, and patient factors (such as general fitness and co-morbidities).
For the majority of patients, this comprises surgery with or without neoadjuvant chemotherapy or chemo-radiotherapy:
- Squamous cell carcinoma – SCCs of the upper oesophagus are technically difficult to operate on and definitive chemo-radiotherapy is therefore usually the treatment of choice
- Adenocarcinomas – the treatment of choice is typically neoadjuvant chemotherapy or chemo-radiotherapy followed by oesophageal resection
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 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 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.
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 to meet their nutritional requirements.
*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
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.
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 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 large undertaking with significant morbidity and complication rates