“I got mesothelioma, a very bad tumor”. This is the shocking revelation of Franco Di Mare, Rai journalist, on Che tempo che fa. Di Mare, 68 years old, speaks to Fabio Fazio with the aid of a respirator, essential to cope with the effects of the disease.
Mesothelioma, as the Airc explains on its website, is a cancer tumor that arises from the cells of the mesothelium, the membranes that cover the internal organs like a thin film. Depending on the area it covers, the mesothelium takes on different names: it is called pleura in the thorax, peritoneum in the abdomen, pericardium in the space around the heart and tunica vaginalis in the area around the testicles.
In addition to malignant mesothelioma, benign tumors can also develop from the mesothelium which are generally removed surgically and do not require further treatment.
What is mesothelioma
Malignant mesothelioma is a rare disease that predominantly affects men. In Italy it represents 0.8 percent of all tumors diagnosed in men and 0.3 percent of those diagnosed in women. 90% of mesotheliomas are due to exposure to asbestos. The term asbestos or asbestos indicates a family of minerals with a fibrous structure, very resistant to heat. Asbestos fibres, over a thousand times thinner than a human hair, can be inhaled and damage cells. If they are deposited in the lungs, the fibers can give rise to other diseases such as asbestosis (a sort of fibrosis of the lung tissue that prevents the organ from expanding properly) or lung cancer.
Most mesotheliomas affect people who have come into contact with asbestos in the workplace. However, non-professional exposure, for example environmental, to asbestos and other asbestiform mineral fibers also increases the risk of mesothelioma. Family members of workers exposed to asbestos are also at risk, since asbestos fibers can be deposited on clothing and transported from the workplace to home, in which case we speak of passive exposure. The latency period, i.e. the time between exposure to asbestos and the appearance of mesothelioma, is very long, around 40-50 years. The risk increases as the duration of exposure and the amount of asbestos fibers inhaled increases.
Other less common risk factors for mesothelioma are exposure to ionizing radiation or thorium dioxide (used in the past as a contrast medium for x-ray images). There are rare cases of familial predisposition linked to mutations in the BAP1 gene.
Different types of this tumor are distinguished:
– pleural mesothelioma: develops in the thoracic cavity and is the most common type (about 3 out of 4 cases);
– peritoneal mesothelioma: develops in the abdomen and represents almost all the remaining cases of mesothelioma;
– pericardial mesothelioma: develops from the membrane that lines the heart and is extremely rare;
– mesothelioma of the vaginal tunic: it develops from the membrane that lines the testicles and is very rare.
Depending on the characteristics of the cells, four subtypes of pleural mesothelioma are distinguished:
– epithelioid, the most common (70-85 percent of cases), and the one with a better prognosis;
– sarcomatoid or fibrous (10 percent);
– mixed or biphasic (10-25 percent)
– desmoplastic, the rarest (less than 2 percent) and most difficult to diagnose.
The first symptoms of pleural mesothelioma, often linked to the accumulation of fluid in the pleural cavity (pleural effusion), are respiratory: shortness of breath (dyspnea) and cough. Pain in the lower back or one side of the chest and more nonspecific symptoms, such as muscle weakness and weight loss, may also be present. Abdominal pain, weight loss, nausea and vomiting are, however, the most common symptoms in case of peritoneal mesothelioma. The volume of the abdomen may increase due to the accumulation of fluid in the peritoneum (ascites).
Determining the stage of the tumor, i.e. how extensive the disease is, is essential for deciding the type of therapy. For mesothelioma, four stages (I-IV) are identified on the basis of the TNM criteria which take into account the extension of the tumor (T), any involvement of the lymph nodes (N) and metastases (M).
As with most tumors, the lower the stage of mesothelioma, the better the chances of successful treatment. However, the diagnosis of this tumor often arrives when the disease has already passed the initial stages and is now difficult to treat, therefore it is one of the tumors with a rarely positive prognosis. Five years after diagnosis, only 8 percent of men and 10 percent of women affected by mesothelioma are still alive.
Therapy
Mesothelioma is a rare and difficult to treat cancer. Doctors first evaluate the possibility of surgical intervention. In principle, early-stage mesotheliomas are operable, but the opportunity to remove them depends on the subtype, location, size and general condition of the patient. In most cases surgery is not curative, but palliative, i.e. it serves to prevent or reduce symptoms.
There are also other treatments that can be used for palliative purposes: the removal of fluid using a long, thin needle from the thoracic cavity (thoracentesis), abdominal cavity (paracentesis) or around the heart (pericardiocentesis) can, for example, provide relief , but, unfortunately, it must be repeated periodically, because the liquid tends to reform.
The standard treatment for mesothelioma is chemotherapy. The most effective drugs are platinum derivatives, such as cisplatin, and antifolates, such as pemetrexed, often used in combination.
Chemotherapy drugs can be administered systemically, with an intravenous injection that takes them throughout the body, or directly into the thoracic cavity (intrapleurally) or abdominal cavity (intraperitoneally). This localized administration is used above all in the case of peritoneal mesothelioma and allows the tumor to be targeted with higher doses of chemotherapy, which is sometimes heated to increase its effectiveness (hyperthermic chemotherapy), limiting side effects to the rest of the body.
For some years, various therapeutic trials with biological drugs and immunotherapy have been underway, although so far none of these approaches has yet demonstrated a real significant impact on the survival of treated patients. However, the progressive improvement of scientific knowledge on this disease is opening up new interesting therapeutic perspectives.
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