Pleural cancers

Pleural cancers can be divided into four subcategories: Malignant pleural mesothelioma; Solitary fibrous tumour of the pleura; Secondary neoplasms (from other cancers); Lymphomas.

Cancers of the mediastinum 

The mediastinum is the central space of the chest between the two pleural cavities. It is bound by the sternum to the front, the spine to the rear, and by the mediastinal pleura at the sides; above it is linked to the neck through the upper opening of the chest and below with the diaphragm.

Mediastinal cancers are divided according to the topography (anterior, medial or posterior mediastinum) and whether benign or malignant.

• Anterior mediastinal cancers are thymoma, germ cell tumours (teratoma, teratocarcinoma, embryonal carcinoma, seminoma, choriocarcinoma), vascular and lymphatic cancers, lipoma, carcinoid tumours, fibroma and fibrosarcoma.
• Medial mediastinal cancers are cysts, paraganglioma, pheochromocytoma, carcinoid tumours, heart and trachea cancers.
• Posterior mediastinal cancers are neurogenic tumours (schwannoma, neurofibroma, ganglioneuroma, ganglioneuroblastoma), enterogenic cysts and oesophageal cancers.

The most frequent cancers are of the lymph nodes (lymphoma or lymph node metastases). The mediastinum is the site of onset of various types of cancers both of benign and malignant nature, among which thymus tumours and tumours originating from neural elements (neuromas) are the most frequent and treated by the thoracic surgeon.

Cancers of the oesophagus 

Oesophageal cancer is the sixth most common cancer in non-industrialised countries, and in eighteenth place in industrialised countries. It predominantly affects men (it is three times more common in men than in women). In most cases it develops after the sixth decade of life. Geographical incidence is variable: Eastern countries, including China and Singapore, are those where mortality is higher and there are about 20-30 cases per year per 100,000 inhabitants. In Italy, the annual incidence rate is about 4 cases per 100,000.

Main causes of oesophagus cancers

Causes are various. Some are genetic, others linked to diet, others to lifestyle, and others still are of inflammatory origin. 

• Genetic factors. Oesophageal 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 oesophagus, or the formation of small growths called papillae.
• Alcohol and tobacco are among the most significant risk factors in Europe and the United States. Eighty to ninety percent of oesophageal 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 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 oesophageal 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. 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 cancer (carcinogenesis). Overweight and obese people are often associated with gastro-oesophageal reflux with a consequent risk of developing the disease called Barrett's oesophagus (which occurs in 8 to 20 percent of people with gastro-oesophageal reflux disease).
• Inflammatory factors. Chronic inflammation of the mucosa lining the oesophagus increases the risk. The most frequent form is peptic oesophagitis, or the chronic inflammation of the lower part of the oesophagus caused by reflux of acidic gastric juices due to a faulty sealing of the junction that separates the oesophagus from the stomach. In the long run, chronic irritation causes the epithelium (the tissue lining the inner of the organ) of the oesophagus to be replaced by one similar to that of the stomach, from which cancer may then develop. This situation is called Barrett's oesophagus and it is considered a precancerous disease which sometimes requires surgery to avoid the complete malignant transformation of the epithelium.

Depending on the tissue from which it originated, there are two types of oesophageal cancer:

Tumors of the pleura

Malignant pleural mesothelioma

Mesothelioma is quite a rare disease and for this reason there are no screening programs for the early diagnosis in persons not at risk. In the case of persons exposed to asbestos for longer or shorter periods, some doctors recommend periodic examinations (X-ray or Computed Tomography) to monitor any changes over time in the structure of the lungs that might indicate the presence of mesothelioma or lung cancer. However it is not yet clear whether this strategy could lead to early diagnosis.

The best way to prevent mesothelioma is to avoid or at least minimise the exposure to asbestos. New laws require the verification of the presence of asbestos in public buildings such as schools, but there may also be traces of this material in older homes. It is important to contact professional technicians who will check construction materials and remove the parts that are not up to standard. DIY removal should be avoided, since bad work may cause the risk of contaminating other areas of the house and the inhalation dangerous fibres.

Symptoms of the malignant pleural mesothelioma

The symptoms of mesothelioma are initially non-specific and they are often ignored or interpreted as signs of other more common and less severe diseases. Early signs of pleural mesothelioma may include pain in the lower back or side of the chest, shortness of breath, cough, fever, fatigue, weight loss, difficulty swallowing, muscle weakness. Abdominal pain, weight loss, nausea and vomiting are more common symptoms of peritoneal mesothelioma.

Test for diagnosing malignant pleural mesothelioma

The first step for a proper diagnosis remains a visit to the general practitioner or a specialist, inquiring about medical history to determine whether there has been exposure to asbestos and assessing the presence of fluid in the abdomen or in the cavity around the heart. In cases of suspected mesothelioma, more specific tests will be carried out.

• Chest CT with contrast medium is used to determine the presence of the tumour, its exact location and the possible spread to other organs, and assist the surgeon in deciding the type of intervention. A spiral CT has recently been developed that is faster and allows for more detailed images compared to the traditional one.
• PET is used to identify the cells that are growing faster and correspond to cancer cells. The images obtained are not as detailed as those of the TC but may help doctors understand whether the abnormalities of the mesothelium are actually tumours or lesions of other kinds and whether the cancer has spread to lymph nodes or other parts of the body. Today, there are tools that can perform both CT and PET in a single session.
• Biopsy is the most effective tool to confirm a suspected mesothelioma. In some cases  fluid samples are obtained from the chest (thoracentesis), abdomen (paracentesis) or the cavity around the heart (pericardialcentesis) using a long thin needle to ascertain the presence of cancer cells under a microscope. In other cases, it is necessary to withdraw small portions of mesothelial tissue with a thin needle inserted under the skin or with the insertion of a probe equipped with a video camera through a small cut in the skin. This way, the doctor can see the suspicious areas and take the samples to be then analysed under the microscope. To distinguish mesothelioma from other types of tumour with certainty, samples taken by biopsy may be subjected to immune-histochemical assays (to see the proteins present on the surface of the cell) or genetic assays (to detect the expression of genes typical of mesothelioma). Blood tests are not typically used to arrive at diagnosis, but it may be useful to confirm a diagnosis obtained with other techniques or to follow up the evolution of the disease during and after treatment. In particular, levels of osteopontin and SMRP are measured, as those molecules are present in higher concentration in case of mesothelioma.

Solitary fibrous tumor of the pleura: symptoms and test for diagnosis

This variant of the pleural tumour has a mostly silent course and it is often diagnosed by chance through a simple imaging test. The bigger the mass size the more likely and severe the symptoms are: shortness of breath, chest pain and cough are three recurrent symptoms in patients with pleural fibrous tumour. Less frequently, however, patients suffering from this form of pleural cancer may complain of anorexia, chills, fever, haemoptysis, swelling of the lower limbs, paraneoplastic manifestations (osteoarthropathy and hypoglycaemia by overproduction of the Insulin-like growth factor II), syncope and pleural effusion. In the giant forms hypomobility of the hemithorax affected by the neoplasia may be encountered.

Fibrous tumour of the pleura is often diagnosed by chance via a simple radiological investigation performed for other reasons. Among the diagnostic strategies aimed at ascertaining tumour of the pleura: routine blood tests, upper abdomen ultrasonography, CT scan, chest CT and MRI, are included. A few patients have to undergo a further examination, PET, which is useful in selected subjects with a suspected malignant degeneration of the tumour.

Mediastinal cancers

Thymic cancers 

Because of its painless course, the diagnosis of thymoma can often be random. At least 30% of patients are asymptomatic at the time of diagnosis. When the often very vague and hazy symptoms are present, they are due to local compression or infiltration of surrounding structures. The most common are chest pain, cough, dyspnoea, paralysis of a hemidiaphragm through phrenic nerve involvement, but dysphonia may also be present through infiltration of the recurrent nerve and signs relating to superior vena cava syndrome.

Thymomas may be associated with a number of paraneoplastic syndromes: the most frequent is without doubt Myasthenia Gravis, which is present in 30-45% of patients, while pure red cell aplasia and hypogammaglobulinaemia occur in 2-5% of cases.

Hypogammaglobulinaemia is present in 2-5% of patients with thymoma A. Thymoma A rarely develops distant metastases, however in some cases it may be locally-invasive with infiltration of surrounding organs and spread to the parietal pleura and/or pericardium. The degree of local invasiveness is decisive in the choice of treatment.

A CT scan of the chest is needed to determine the extent of the lesion, the relationship with adjacent structures and possible pleural and/or pericardial effusions. It can also be instrumental in diagnosing small tumours not appreciable with a standard chest X-ray. Surgical biopsy of the lesion is not recommended in cases of encapsulated thymic tumours because of the risk of dissemination; it is necessary in the case of unresectable tumours or for differential diagnosis with other malignant neoplasms affecting the anterior mediastinum for a correct therapeutic choice.

Neuromas

In the majority of cases, these are detected occasionally with chest radiography. The test used to diagnose this disease with certainty is a chest CT scan, to which a spinal MRI might be added, in cases of suspected extension to the spinal canal.

Oesophageal cancers

Precautions to prevent cancers of the oesophagus

Avoiding alcohol and smoking are the main precautions to prevent the formation of squamous cell oesophageal cancer. Regarding adenocarcinoma, in the majority of cases it develops from a Barrett's Oesophagus, so the most effective way to prevent this is reducing the risk of gastro-oesophageal reflux. This is achieved by reducing the consumption of coffee, alcohol and cigarettes and avoiding being overweight and obesity. Although several antacid drugs are able to control the symptoms of reflux, scientific demonstrations of their efficacy in reducing the appearance of Barrett's Oesophagus are not available so far.

Tests for the early diagnosis of the tumors of esophageal cancers

There are no screening tests in healthy patients, but early diagnosis is extremely important once Barrett's Oesophagus has developed in order to catch the possible malignant transformation quickly. In patients where the oesophageal mucosa has turned into gastric mucosa, endoscopy is recommended every two or three years. In patients whose transformed cells show signs of abnormality (dysplasia), it is recommended to repeat endoscopy at least two times every six months and then once a year. Finally, if the degree of dysplasia is high (that is if the cells are highly transformed), endoscopic removal or even surgery is recommended, because this is a precancerous condition at high risk of malignant transformation. 

Symptoms of oesophageal cancer

Almost always, initial symptoms of oesophageal cancer are progressive loss of weight preceded by dysphagia, that is difficulty in swallowing, which usually appears gradually first with solid foods and after with liquids. These symptoms are reported by 90 percent of patients. In addition, growth of the tumour to the outside of the oesophagus can lead to a decrease or an alteration in the tone of the voice because it involves nerves that govern the emission of sounds, or induce paralysis of the diaphragm, or even a pain in the chest just behind the breastbone, if the area between the heart, lungs, sternum and spine is involved.

In the most advanced stages of the cancer, the ability to take food may be reduced. If the tumour is ulcerated, swallowing may become painful. When the mass of the tumour impedes the descent of the food down the oesophagus, regurgitation episodes may occur. In more advanced forms, the lymph nodes on the sides of the neck and above the collarbone can swell, or liquid can form in the lining of the lung (pleural effusion) with the onset of dyspnea (difficulty breathing), or even bone pain may appear or an increase in size of the liver. The cause of these symptoms is usually related to the presence of metastases. 

Diagnostic tests for oesophageal cancer

In symptomatic patients, diagnostic strategy includes an X-ray of the oesophagus with contrast medium and oesophageal endoscopy (oesophagogastroscopy) that allows us to see whether there is a lesion and obtain material for analysis of the cells. The combination of the two procedures increases the diagnostic sensitivity to 99 per cent. X-rays are used to exclude the presence of associated disease, the oesophagogastroscopy is the examination with the greatest diagnostic value because it allows direct visualisation of the structures and to take samples for biopsy.

Echo-endoscopy is another type of test that allows us to determine more accurately how deep the infiltration of the layers in the oesophageal wall is, and can point out lymph nodes that are suspicious for metastatic involvement. Once the tumour has been localised, to complete the diagnostic testing it is appropriate to perform chest CT abdomen with contrast medium and PET in order to exclude the presence of remote metastases.

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Nutritional prevention for the tumors of the esophagus

Based on careful evaluation of the results from current scientific research, it has been possible to identify specific risk factors and protective nutritional factors for specific types of cancer. Experts have classified the results into four levels: "convincing evidence", "probable evidence", "limited evidence" and one last level that collects the effects for which evidence of association with the cancer is “highly unlikely." The probable and convincing evidence for the basis of the recommendations are:

Risk Factors

• alcoholic beverages (convincing evidence)
• being overweight and obesity (convincing evidence for adenocarcinoma).

 Protective factors

• fruit and vegetables
• foods rich in beta-carotene, such as carrots, pumpkin, apricots, spinach, sweet and spicy peppers (probable evidence)
• foods rich in vitamin C, such as citrus fruit juice peel, kiwi, strawberries, sweet and spicy peppers (evidence likely).

Pleural cancers

Malignant pleural mesothelioma

There are no guidelines for the treatment of mesothelioma because of the limited number of cases. Therapy is strictly dependent on the stage. For stage I and selected cases in stage II and III the main option is surgery, pleurectomy with decortication or extrapleural pleuropneumonectomy, preceded by induction chemotherapy (before surgery) and possibly post-operative radiotherapy if the patient’s clinical conditions permit.

The surgery also uses talc in a technique practised in video-thoracoscopy using a pleuroscope. The liquid is removed and talc is introduced. Talc creates a local inflammatory reaction and joins the two pleural layers preventing the effusion to be formed again. This technique must be followed by radiotherapy on the thoracoscopy gap in order to limit or avoid a potential seeding of tumour cells that cause the formation of subcutaneous nodules.

Chemotherapy is the treatment of choice in cases not operable with radical technique or after talc has been used. Platinum and its derivatives are used as standard associated with a second drug that may be pemetrexed, gemcitabine or paclitaxel, in combination with or without radiotherapy.

Solitary fibrous tumour of the pleura

Treatment is surgery. The whole mass should be removed with excision of one or more portions of the parietal/diaphragmatic/mediastinal pleura.

Mediastinal cancers

Thymic cancers

In most cases, the treatment is surgery, with the exception of lymphomas, seminomas and solid tumours in advanced stages that require an integrated treatment with radiotherapy and chemotherapy.

The treatment of thymoma depends on the clinical presentation, and in particular local invasiveness. Surgery is definitely the main approach. With the exception of those with unresectable tumours or distant metastases, all patients with thymoma, should undergo surgery with complete resection of the lesion even if infiltrating surrounding non-vital organs (pleura, pericardium, lung). The presence of lung metastases does not affect surgery.

The surgical access chosen in most cases is longitudinal median sternotomy although in the case of large lesions extending into the pleural cavity, posterolateral thoracotomy is preferable. More recently, for small and capsulated lesions, some authors have proposed minimally-invasive thymectomies with surgical access through thoracoscopy or using a robot.

Pleural localisations (pleural re-implants) may occur even years after surgery. These re-implants can be surgically removed through thoracotomy access.

In invasive forms, treatment normally involves surgery in combination with radiotherapy and chemotherapy.

Neuromas

Surgical removal is always indicated because, although in the majority of cases of benign nature, the slow but gradual growth inevitably leads to the appearance of symptoms. Surgery can be conducted via video-thoracoscopy through three small incisions in the skin with an average hospitalisation time of 4-5 days.

Even in the rare cases of tumours with intraspinal extension, video-toracoscopy techniques can be used associated with a neurosurgery posterior incision. Only for large tumours and in rare cases of malignant neurogenic tumours is it necessary to intervene with traditional thoracotomy incision.

Oesophageal cancers

Surgical treatment of oesophageal cancers

To treat oesophageal cancers, surgery is the first resort. However, it is difficult to operate lesions in the upper third of the oesophagus, or cases in which the tumour has already involved neighbouring organs such as the trachea and bronchi. Distant metastases sometimes contraindicate the operation as well as poor general health conditions or the presence of other diseases.

Surgery usually consists of removing the section of oesophagus affected by the tumour and then reconstructing it using the stomach, that is made into a tube, and the regional lymph nodes. The procedure is called a partial or total oesophagectomy associated with gastroplasty and regional lymphadenectomy.

Treatment of the oesophageal cancers in non-operable patients

In non-operable patients, chemotherapy accompanied by radiotherapy is the treatment of choice, the combination of the two treatment options increases survival compared to individual options alone. In operable cases but with locally-advanced or suspected lymph node metastases, chemotherapy may be indicated, possibly associated with radiation therapy, before surgery (neoadjuvant therapy).

Patients with advanced stage disease with difficulty in swallowing and pain, for whom neither surgical treatment nor chemo-radiotherapy are feasible, may benefit from palliative care in order to enable adequate nutritional support. These may consist in the endoscopic positioning of a rigid pipe in plastic, silicone or metal through the oesophagus to allow the passage of food, or in laser-therapy, namely the use of a laser beam on the tumour to restore patency.

Oesophageal cancers

Clinical nutrition for patients with stomach cancer

Malnutrition is often found in patients with cancer of the stomach and oesophagus. The latter may be related to dysphagia (difficulty with or obstruction of the passage of food through the mouth, pharynx or oesophagus), cachexia associated with the disease (syndrome characterised by loss of fat and muscle mass) and chemotherapy. The causes of malnutrition common to all cancer patients overlap with the nutritional abnormalities resulting from surgical treatments.

Nutritional abnormalities resulting from surgical treatment for stomach

During surgery for oesophageal or gastric resection, a small probe (nutritional jejunostomy) can be positioned in order to ensure a physiological nutrition in the postoperative period (through the use of Enteral Nutrition - EN) and the required amount of nourishment when intake via the mouth is poor or inadequate compared to the need. The nutritional jejunostomy is left in place at the time of discharge, and it is used in cases when the patient is unable to take a proper diet to cover the nutritional requirements.

After gastrectomy, among the early symptoms developed are the small stomach syndrome (early satiety and gastric distension) and dumping syndrome (occurs after taking the meal and includes hypotension, tachycardia, dizziness, feeling tired, faintness, chills and profuse sweating). Diarrhoea can also be experienced and, in patients who have undergone partial gastrectomy with gastro-jejunal reconstruction, bilious vomiting may occur.

Among the later problems are anaemia and malabsorption of calcium. Blood tests should be carried out to assess any possible supplementation.

Following oesophagectomy surgery, there is a reduced ability to take large volumes of food and in some patients dumping syndrome may occur.

Information for the diet of patients operated for gastric cancer

Dietary guidelines focus on the consumption of small, frequent meals throughout the day, separating liquids from solids. It is recommended to take small bites and chew well to facilitate swallowing and digestion. For the nutritional management of dumping syndrome, simple sugars have to be limited.

In the presence of diarrhoea, it is advisable to limit simple sugars and take liquids in small sips. With regard to the possible presence of bilious vomiting after intervention for subtotal gastrectomy, treatment is essentially surgical, but also in this condition the patient may benefit from a partition of the diet in small and frequent meals.

Following oesophagectomy, sticky foods should be avoided, foods that are fermented and carbonated soft drinks. It is also important that the patient remains seated for 30-60 minutes after eating and at least 2 hours before going to bed.

For patients who have undergone gastric or oesophageal resection, short and long term follow-up are of crucial importance, in order to immediately adapt the more appropriate dietary pattern, correct any errors present in the diet, prevent weight loss and identify any late symptoms.