Neoplasms
of the oesophagus
Benign
tumours
Benign tumours of the oesophagus are rare. The commonest is a leiomyoma
(Fig. 50.45). Even a large leiomyoma may produce surprisingly mild
symptoms. It is usually possible to enucleate the tumour at thoracotomy without
breaching the mucosa. Small polyps of the oesophagus, such as granular cell
tumours and fibrovascular polyps, may be found in the course of endoscopy for
other conditions.
Sarcoma
Sarcoma of the oesophagus is exceedingly rare, but leiomyosarcoma (Fig.
50.46)
and rhabdomyosarcomas have been reported. Other types of sarcoma have been
reported, but are confined to individual case reports.
Malignant
melanoma
Malignant melanoma of the oesophagus is rare. It may represent secondary
spread, but primary melanoma of the oesophagus does occur. It has a very poor
prognosis.
Carcinoma
of the oesophagus
Cancer of the oesophagus is the ninth most common cancer in the world.
It is in general a disease of mid to late adulthood with a poor survival rate.
Only 5—10 per cent of those diagnosed will survive for 5 years. Despite
this gloomy prognosis there are some encouraging signs of change.
Pathology
and aetiology. Squamous cell cancer (Figs 50.47 and
50.48) and
adenocarcinoma (Figs 50.49 and 50.50) are the commonest types. Squamous
cell carcinoma usually affects the upper two-thirds of the oesophagus and adenocarcinoma
affects the lower one-third, but there are frequent exceptions to this rule. Oat
cell cancer occurs occasionally. World-wide, it is squamous cell cancer that is
commonest, but adenocarcinoma is the commonest type in most westernised
countries and is increasing in incidence.
Geographical variation of
oesophageal cancer
Away
from the endemic areas tobacco and alcohol are the major factors in the
occurrence of squamous cancer. Incidence rates vary from less that five per
100 000 in whites in the USA to 26.5 per 100 000 in some regions of
France. Contrary to popular opinion Japan does not have a particularly
high incidence of oesophageal cancer.
The incidence of adenocarcinoma
of the oesophagus is increasing
In
many westernised countries the incidence of squamous cell cancer has fallen or
remained relatively static, but the incidence of adenocarcinoma of the
oesophagus has increased since the mid-1970s by 5—10 per cent per annum. The
change is greater than that of any other neoplasm in this time. Adenocarcinoma
now accounts for 60—75 per cent of
Involvement of coeliac axis
nodes is a bad prognostic sign
The
poor prognosis of oesophageal cancer is proof of its ability to spread. This may
be locoregional or systemic. Locoregional spread occurs through
the wall of the oesophagus into adjacent structures, along the length of the
oesophagus in the submucosal lymphatics, and to regional
Early endoscopic diagnosis is
the key to good results
Clinical
features. Dysphagia is the usual presenting feature and is generally a sign of
advanced disease. Weight loss is likewise a sign of advanced disease. An
increasing number of cancers is diagnosed at a relatively early stage when the
chances of cure are greater. Patients with early disease may present with rather
nonspecific dyspeptic symptoms or a vague feeling of ‘something that is not
quite right’ during swallowing. Some are diagnosed during screening of
Barrett’s
Hoarseness
due to recurrent laryngeal nerve palsy is a sign of advanced and incurable
disease. Palpable lymphadenopathy in the neck is likewise a sign of advanced
disease.
Staging
and general assessment. Methodical assessment of the patient and the cancer is
essential if the best results are to be achieved. Some patients are quite
obviously not candidates for radical treatment even on the most superficial
inspection and common humanity must always take precedence. Staging must not
delay effective palliation.
The
most important aspect of staging is a careful search for metastatic disease.
Ultrasonography of the liver and computerised tomography (CT) scanning of the
chest and abdomen is mandatory before resection. The purpose of CT scanning is
to exclude metastatic disease from the lungs and liver. Despite considerable
improvements in CT and magnetic resonance imaging these methods are still
inaccurate for staging the primary lesion and for staging lymph nodes.
Endoscopic ultrasonography, if available, is the best method for preoperative
staging of oesophageal cancer. Bronchoscopy should be done in lesions of the
upper or middle thirds where there is potential for tracheobronchial invasion (Fig. 50.54).
Laparoscopy is useful for assessing adenocarcinoma of the
distal oesophagus (Fig. 50.55), particularly if it is likely to extend
below the phreno-oesophageal ligament. Laparoscopy is at its best for detecting transperitoneal spread and liver
metastasis. It is an inexpensive technology that deserves to be widely used. At
the cost of some time and effort it is also possible to sample lymph nodes.
Short-term nutritional support
has doubtful benefits
Treatment
of malignant tumours
Principles
A gastrostomy should never be carried out as the ‘palliation’
for oesophageal cancer. Palliation in this disease demands relief of dysphagia.
It is important that staging and general assessment are carried out speedily and
humanely. Those who have incurable disease should not be submitted to needlessly
aggressive treatment that simply prolongs the process of dying. Palliation of
dysphagia can be achieved in a number of ways that do not unduly stress the
patient. Pain is also a surprisingly common feature of advanced oesophageal
Curative treatment involves
radical surgery or radiotherapy. This should be carried out in a specialist
centre with the necessary expertise. The role of palliative resection is
debatable. It is wise not to embark on resection if it is clear beforehand that
palliation is all that can be achieved. However, palliative resection may be
appropriate if incurable disease is found when an operation is already well
under way.
Surgical
resection probably gives the best results for all forms of oesophageal cancer.
Radical radiotherapy can cure both types of cancer, but poses technical problems
at the lower end of the oesophagus. Tumours that involve the stomach are not
generally accepted for radiotherapy. The results of radiotherapy have been
improved by concurrent chemotherapy, so-called chemoradiotherapy. Some
have suggested that this development may challenge the dominant place of surgery
for cure.
Surgery
Beware of satellite nodules
proximal to the primary lesion
Curative surgery involves resection of an appropriate length of the
oesophagus together with any involved stomach and the locoregional lymphatics.
There is controversy about the length of oesophagus that should be resected.
Some surgeons feel that operative trauma should be reduced to the minimum by
resecting only enough oesophagus to clear the tumour. Others advocate subtotal
oesophagectomy pioneered in the UK by McKeown on the grounds that generous
proximal clearance gives the best chance of clearing satellite nodules in the
submucosal lymphatics and gives the best postoperative function with the least
tendency to gastro-oesophageal reflux. Restoration of continuity is almost
always achieved by transposition of the stomach and oesophagogastric
anastomosis.
Colon or, less commonly, small intestine may be interposed between the
oesophageal remnant and the stomach, but is a more major undertaking with a
higher postoperative mortality. There is also controversy about the extent of
lymph node dissection that should be done. Akiyama has pioneered the concept of three
field lymph node dissection, an ultraradical operation involving extensive
removal of the regional lymph nodes in the abdomen, chest and neck.
Lesions
of the cardia that do not involve the oesophagus to any significant extent may
be dealt with by extended total gastrectomy to include the distal oesophagus or
by proximal gastrectomy and distal oesophagectomy.
Subtotal
oesophagectomy. A variety of approaches is possible. The most commonly used is
the Ivor Lewis (or.
Carefully preserve the blood supply of the stomach — venous and arterial
Mobilisation
of the stomach must be done with care as it is essential to have a tension-free,
well-vascularised stomach for transposition. The left gastric artery, the short
gastric vessels and the left gastroepiploic are all divided. The viability of
the transposed stomach mainly depends on the right gastroepiploic artery and
vein with a small contribution from the right gastric. It should be noted that
venous
Right
thoracic approach gives easy access to the oesophagus
The
approach to the oesophagus through the right chest is relatively
straightforward. The azygos vein is divided and this allows easy access to the
whole of the intrathoracic oesophagus. A thoracotomy with entry above the
fifth rib gives best access to the mid-mediastinum and the thoracic inlet. The
oesophagus is divided just below the thoracic inlet. Since most lesions are in
the lower third or middle third this usually gives adequate proximal clearance
of at least 5 cm. If there is any doubt about clearance frozen sections
should be taken from the resection margins and it may be helpful to open the
oesophagus and assess whether the lesion is well circumscribed or whether it is
diffusely infiltrating. Carcinomas of the upper thoracic oesophagus are almost
always incurable at the time of diagnosis and invasion of the trachea is common.
If one of these lesions is resectable it is essential to use an incision
in the neck and to resect more of the oesophagus than is customary in the
operation of subtotal oesophagectomy.
Oesophagogastric
anastomosis may be performed equally well by hand or stapler. Both methods
require attention to detail. In experienced hands serious anastomotic leakage
should be uncommon (significantly less than 5 per cent). Minor leakage
detectable by contrast radiology is more common, but should not disturb the
patient. The significance of these minor leaks is debated. Most surgeons still
prefer to keep the patient nil by mouth for 5—7 days and then perform a
contrast swallow If small leaks are to be detected it is essential to use
barium for the examination. Water-soluble contrast media miss 50 per cent of
anastomotic leaks. If leakage is detected the patient is kept nil by mouth until
it has sealed.
Postoperative
nutritional support remains controversial. There is general agreement that
parenteral feeding is associated with more nosocomial infection,
including pneumonia, than enteral feeding. It is also expensive. If
nutritional support is given a feeding jejunostomy is probably the best method.
Transhiatal
oesophagectomy (without thoracotomy). This approach has been popularised by
Orringer in the USA and Pinotti in Brazil. The stomach is mobilised through a
midline abdominal incision and the cervical oesophagus is mobilised through an
incision in the neck. The diaphragm is then opened from the abdomen and the
posterior mediastinum is entered. The lower oesophagus and the tumour are
mobilised
Left
thoracoabdominal approach. A long skin and muscle incision is made on the left
side with entry into the chest above the seventh rib and removal of a short
segment of costal cartilage (Fig. 50.56). The diaphragm is incised and the
oesophagus and stomach are removed. Some surgeons advocate a left
thoracoabdominal approach together with an incision in the neck for subtotal
oesophagectomy, the ‘Birmingham’ approach popularised by Matthews.
Thoracoscopic
oesophagectomy. Oesophagectomy may be done by thoracoscopy or by the hybrid
technique of video-assisted thoracic surgery (VATS) in which a combination of
endoscopic and conventional instruments is used through small thoracic
incisions. Thoracoscopic oesophagectomy in the prone position to minimise injury
to the collapsed lung has been pioneered by Cuschieri. At present this is still
an evolving technique and its place is not yet established. The procedure takes
longer than open surgery and postoperative morbidity is still a problem.
Gastro-oesophageal
reflux following oesophagogastric resection. Gastro-oesophageal reflux may be a
major problem following any operation that involves resecting the cardia.
Postoperative reflux may present with the typical symptoms of GORD or with a
peptic stricture at the site of the anastomosis. However, the presentation may
be different with a miserable patient who fails to thrive following the
operation and who is then suspected of having recurrent cancer. This atypical
presentation is particularly common following total gastrectomy with an
inadequate reconstruction.
Reflux
may be a problem following resection
Symptoms
may be atypical
Reflux
may be limited or avoided by:
1. subtotal oesophagectomy and gastric transposition high in the chest.
The vertical stomach empties rapidly and functions as a barrier to reflux;
2. resection of a generous portion
of proximal stomach if an anastomosis is made to the lower oesophagus. This
reduces gastric secretion;
3. Roux-en-Y reconstruction with a
long ascending jejunal limb (5 0—60 cm);
4. interposition of jejunum or
colon (Fig. 50.57)..
The
old Braun or omega reconstruction with an enteroenteroanastomosis below
an oesophagojejunostomy is mentioned only to be condemned. The side-to-side
anastomosis does not divert bile and pancreatic secretion satisfactorily from
the oesophagus and several studies have shown that these patients fare badly.
Pyloroplasty.
Pyloroplasty or pyloromyotomy is an option following oesophageal resection. It may
avoid early problems with gastric emptying, but the stomach always seems to
recover its function even without pyloroplasty. If delayed gastric emptying is a
problem in the early postoperative period erythromycin, which is a motilin
agonist, seems to be the best therapy.
Radiotherapy
for cure
Radiotherapy
may be a useful alternative to surgery, especially in unfit patients
Radical radiotherapy can produce long-term survival in oesophageal
cancer (Fig. 50.58). Although traditionally used for
Chemotherapy
With the advent of regimens containing cis-platinum, chemotherapy for
oesophageal cancer has improved considerably. Chemotherapy never cures the
disease, but can produce worthwhile shrinkage of disease in up to 60 per cent of
cases. The best responses are seen in squamous cell cancers. Survival is
extended modestly.
Multimodality
treatment
Randomised prospective studies of preoperative and postoperative
radiotherapy have not shown any improvement in survival. Thus far there is no
evidence that perioperative chemotherapy improves survival, but the results of
studies using modern combination chemotherapy are awaited. Significant
improvement in survival will only be achieved by a treatment that has a powerful
effect on systemic disease.
Palliative
treatment
Surgical resection and external beam radiotherapy may be used for
palliation, but are not suitable when the expected survival is short, as most of
the remainder of life will be spent recovering from the ‘treatment’.
Surgical bypass is likewise too major a procedure for use in a patient with a
limited life expectancy. A wide variety of relatively simple methods of
palliation is now available that will produce worthwhile relief of dysphagia
with minimal disturbance to the patient.
Palliation
should be simple and effective
Intubation
has been used for many years following the invention of the Souttar tube made of
coiled silver wire (Fig. 50.59). This was superseded by the Celestin tube
whose design gave a better quality of swallowing and was safer to insert. The
Celestin tube was originally designed to be inserted by oesophagoscopy, to
place a plastic rod in the stomach, followed by laparotomy and gastrostomy, to
retrieve the rod and pull the tube down into place. The development of methods
of intubation that could be used with a flexible endoscope was a major advance
pioneered by Atkinson of Nottingham. The Atkinson tube is still in use. It is
made of silastic with a nylon spiral reinforcement and has a distal retaining
flange to prevent proximal displacement. It is inserted over a guidewire with a
specially designed introducer. There are now many other designs of semi rigid
tubes for palliation including the Procter—Livingstone tube which is popular
in South Africa where there is a very high incidence of oesophageal cancer.
The
technology of intubation has now moved on with the invention of various types of
expanding metal stent (Fig. 50.60). These are inserted under X-ray or endoscopic
control. The stent is restrained in the collapsed state during insertion and
then released when it is in the correct position. Expanding stents produce a
better lumen for swallowing than rigid tubes, but are relatively expensive.
Endoscopic
laser treatment may be used to core a channel through the tumour. It produces
worthwhile improvement
Brachytherapy
is a method of delivering intraluminal radiation with a short penetration
distance (hence the term brachy) to a tumour. An introduction system is
inserted through the tumour and the treatment is then delivered in a single
session lasting for 20 minutes or so. The equipment is expensive to purchase,
but running costs are low
Other
methods of palliation that can be given endoscopically include bipolar
diathermy (the BICAP probe), argon beam plasma coagulation and alcohol
injection.
Malignant
tracheo-oesophageal fistula
Malignant tracheo-oesophageal fistula is a sign of incurable disease and
life expectancy is short. Some have advocated surgical bypass and oesophageal
exclusion, but this is a major procedure. An expanding metal stent is probably
the best treatment, but semi rigid prosthetic tubes may also be used (Fig.
50.61).
Postcricoid
carcinoma
Postcricoid carcinoma is considered in the section on neoplasms of the
pharynx (Chapter 43).