The oesophagus is an organ responsible for transporting food from the pharynx to the stomach. It has a tubular shape with 2 sphincter structures at the ends: the upper oesophageal sphincter, which is a high pressure area that prevents reverse passage from the oesophagus to the respiratory tract, and the lower oesophageal sphincter, which prevents reverse passage of material from the stomach to the oesophagus. It is made up of different layers. These are, from the inside outwards, the mucosa, the submucosa, and the two muscular tunicas consisting of the inner circular muscle and the outer longitudinal muscle. These muscular structures are composed of smooth muscle, whose activity is independent of our control. Muscular activity enables the food bolus to pass through the sphincters and along the body of the oesophagus.

Changes in this motor activity can cause the emergence of functional obstruction that affects the transit of the bolus along the oesophagus, giving rise to a clinical picture that includes dysphagia (food transit difficulties), regurgitation (reflux of material from the oesophagus to the oral cavity), chest pain and weight loss. The most alarming clinical picture resulting from changes in the motor function of the oesophagus is achalasia, a rare disease of the oesophagus that can lead to serious malnutrition, and for which surgical treatment is commonly indicated. It is a benign condition but is often seriously debilitating for the patient. In addition to its disabling symptomatology, this condition is significant because it increases the risk of development of a malignant tumour of the oesophagus.

STAFF

At the IEO esopaghus cancer is treated by a multidisciplinary team consisting of specialists in:

• Abdomino - Pelvic Surgery 
• Gastrointestinal and Neuroendocrine Cancer Medical Treatment
• New Drugs and Early Drug Development for Innovative Therapies
• Department of Pathology and Laboratory Medicine
• Department of Medical Imaging and Radiation Sciences

• Genetic factors. Esophageal cancer, in the squamous cell form, appears in almost all patients with hand and plantar tylosis, a rare inherited disorder characterised by thickening of the skin of the palms of hands and soles of feet (hyperkeratosis), and papillomatosis of the esophagus, or the formation of small growths called papillae.
• Alcohol and tobacco. are among the most significant risk factors in Europe and the United States for esophagus cancer. Eighty to ninety percent of esophageal cancers are caused by the consumption of alcohol and tobacco, whether smoked or chewed. Smokers are 5-10 times more likely to succumb than non-smokers, according to the number of cigarettes smoked and years of smoking (the effects of which are enhanced by alcohol). Alcohol not only acts as a direct esophageal cancer cause, but also enhances the carcinogenic action of smoking and people who consume cigarettes and alcohol together have a 100-fold increased risk of getting esophageal cancer.
• Diet. A diet low in fruit and vegetables and a low intake of vitamin A and certain metals such as zinc and molybdenum can increase the risk of esophagus cancer. A fat rich diet and the consequent increase in body fat affect the level of many hormones that create a favourable environment for the onset of esophagus cancer (carcinogenesis). Overweight and obese people are often associated with gastro-oesophageal reflux with a consequent risk of developing the disease called Barrett's esophagus (which occurs in 8 to 20 percent of people with gastro-esophageal reflux disease).
• Inflammatory factors. Chronic inflammation of the mucosa lining the esophagus increases the risk of esophageal cancer. The most frequent form is peptic oesophagitis, or the chronic inflammation of the lower part of the esophagus caused by reflux of acidic gastric juices due to a faulty sealing of the junction that separates the esophagus from the stomach. In the long run, chronic irritation causes the epithelium (the tissue lining the inner of the organ) of the esophagus to be replaced by one similar to that of the stomach, from which cancer may then develop. This situation is called Barrett's esophagus and it is considered a precancerous esophagus cancer which sometimes requires surgery to avoid the complete malignant transformation of the epithelium.

Benign cancer of the oesophagus

Benign tumours of the oesophagus can originate from all cellular components of the bowel. These lesions are quite rare. The most common forms are leiomiomas, namely neoplasms originating in the muscle cells. Less common are neoplasms originating in oesophageal adipose tissue, i.e. lipomas Other neoplastic forms are even rarer. Symptoms of food transit obstruction (dysphagia) can be an indication of these neoplasms. A surgical approach may be taken for symptomatic or growing forms or in the event of diagnostic doubt. The surgical approach often involves the use of minimally invasive, thoracoscopic, laparoscopic or endoscopic procedures.

Malignant cancer of the oesophagus

Malign tumours of the oesophagus can originate from all cellular components of the bowel. The most common forms are squamous cell carcinoma, which originates in the oesophageal mucous lining, and adenocarcinoma, which arises from the transformation of the inner lining from pavement to cylindrical (Barrett’s oesophagus). Rarer forms arise from other types of cells: leiomyosarcoma, (from muscle tissue), melanoma, lymphoma and other, rarer forms.

Malignant cancer of the oesophagus mainly affects the male sex, with increasing incidence from 50 years onwards. There has been a significant increase in the incidence of adenocarcinoma in recent decades, especially in the Western world, while a decrease in incidence has been recorded for the squamous cell form. Both neoplastic forms are associated with smoking and alcohol consumption. Adenocarcinoma is more common in patients with a high body mass index, while squamous cancer is more associated with malnutrition.

There are no screening programs for oesophageal cancer available to the general population of the Western world, as the low incidence of neoplasia does not suggest that there is a need. However, it is worth noting that patients with a diagnosis of Barrett's oesophagus or those diagnosed with achalasia are included in monitoring programs due to the higher incidence of neoplasia in patients with these pathological conditions. Other conditions that predispose patients to the onset of oesophageal cancer, thereby including them in monitoring programs, are previous ingestion of caustic substances and some rare forms of genetic predisposition.

Symptoms, diagnosis and staging

The growth of a malignant tumour in the oesophagus can be responsible for the appearance of obstructive symptoms with dysphagia (difficulties in progression of the bolus) odynophagia (pain on swallowing), weight loss (both due to the tumour itself and difficulties in feeding), sialorrhea (salivary regurgitation). More complex symptoms may be caused by the emergence of complications such as oesophagus-tracheal fistula, which affects inhalation in the airways (cough, pneumonia), or the local progression of the disease or lymph node metastases, which may be responsible for recurrent paralysis and, therefore, dysphonia (alteration of the vocal timbre).

Tomography

Diagnosis is essentially based on endoscopy with histological examination of the biopsy sample. An accurate description of the neoplasm site in relation to anatomical landmarks (essentially the oesophageal sphincters) is extremely important for the treatment approach and the possible choice of surgical approach.

Staging is based mainly on computerised tomography of the neck, thorax and abdomen using intravenous contrast medium. We also often rely on other investigations, such as echo-endoscopy, bronchoscopy or PET FDG; laparoscopy is also used in rare cases. It is then important to assess the general condition of the patient by means of clinical and instrumental investigations. These aim to check the cardiovascular system, respiratory system, renal and hepatic functions. It is then important to make a thorough assessment of the nutritional status of the patient and implement any corrective measures in the event of severe malnutrition.

Treatment

The way in which malignant neoplasms of the oesophagus are treated depends on the stage of the lesion and the general condition and age of the patient. The choice of treatment generally requires a multidisciplinary assessment that includes a surgeon, medical oncologist, radiotherapist, endoscopist, anaesthesiologist, nutritionist and possibly other specialists. Early cases are limited to superficial infiltration of the oesophagus wall and can be treated with surgical endoscopy only.

Intermediate forms may benefit directly from surgery, but most cases of neoplasia are diagnosed at a more advanced stage. In this case, metastatic forms are treated using palliative care, while locally advanced forms are treated with integrated therapies, ie with double or triple therapy (chemotherapy and surgery or chemo-radiotherapy and surgery). These combined treatments aim to reduce the size of the neoplasm in order to increase the chances of success of radical surgery. In advanced cases, chemotherapy may also lead to an improvement in therapeutic results; chemotherapy is much more tolerable for the patient if performed before surgery, while post-operative treatment is extremely difficult for patients to endure. The drugs normally used for the treatment of oesophageal cancer are carboplatin, cisplatin, fluorouracil and taxol. Other forms of treatment (biological and immunotherapy) are used selectively, within the context of experimental protocols.

Minimally invasive surgery

 The aim of the surgery is to radically remove (with undamaged margins) the primary tumour and the draining lymph nodes. Radical removal of tumours in the first section of the oesophagus may necessitate the removal of the larynx, thus resulting in loss of phonatory function. For this reason, surgery in this district is used only in highly selected cases of failure of definitive chemoradiotherapy, or in the event of recurrence of the disease after such treatment.

In all other cases, surgery involves the removal of the oesophagus and lymph nodes: the bowel is generally replaced using the stomach, which is transformed into a "tubule" and transposed into the thorax or neck. In special cases, when the stomach is not available or in other specific conditions, the oesophagus is replaced by another part of the bowel: the colon or small intestine.

In the most common procedures, the so-called oesophago-gastroplasty, we increasingly depend on minimally invasive surgery, with laparoscopic and/or thoracoscopic access. However, in all cases, whether traditional surgery or minimally invasive techniques are used, we are dealing with highly complex procedures whose outcome, in terms of both complications and mortality and oncological results, depends on the number of operations performed by the surgeon or members of the individual hospital centre. It is therefore essential that patients suffering from neoplasia of the oesophagus are able to rely on a high-volume centre that can provide the correct multidisciplinary treatment choice using of the most advanced therapeutic techniques.

The head of the Department of Gastro-intestinal Surgery at the European Institute of Oncology has extensive experience in the surgical treatment of these neoplasms and participates in various clinical trials concerning this type of cancer.