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Chemotherapy is the only treatment for mesothelioma that has been proven to improve survival in randomised and controlled trials. The landmark study published in 2003 by Vogelzang and colleagues compared cisplatin chemotherapy alone with a combination of cisplatin and pemetrexed (brand name Alimta) chemotherapy in patients who had not received chemotherapy for malignant pleural mesothelioma previously and were not candidates for more aggressive "curative" surgery. This trial was the first to report a survival advantage from chemotherapy in malignant pleural mesothelioma, showing a statistically significant improvement in median survival from 10 months in the patients treated with cisplatin alone to 13.3 months in the group of patients treated with cisplatin in the combination with pemetrexed and who also received supplementation with folate and vitamin B. Vitamin supplementation was given to most patients in the trial and pemetrexed related side effects were significantly less in patients receiving pemetrexed when they also received daily oral folate 500mcg and intramuscular vitamin B 1000mcg every 9 weeks compared with patients receiving pemetrexed without vitamin supplementation. The objective response rate increased from 20% in the cisplatin group to 46% in the combination pemetrexed group. Some side effects such as nausea and vomiting, stomatitis, and diarrhoea were more common in the combination pemetrexed group but only affected a minority of patients and overall the combination of pemetrexed and cisplatin was well tolerated when patients received vitamin supplementation; both quality of life and lung function tests improved in the combination pemetrexed group. In February 2004, the United States Food and Drug Administration approved pemetrexed for treatment of malignant pleural mesothelioma. However, there are still unanswered questions about the optimal use of chemotherapy, including when to start treatment, and the optimal number of cycles to give. Cisplatin and pemetrexed together give patients a median survival of 12.1 months.
Cisplatin in combination with raltitrexed has shown an improvement in survival similar to that reported for pemetrexed in combination with cisplatin, but raltitrexed is no longer commercially available for this indication. For patients unable to tolerate pemetrexed, cisplatin in combination with gemcitabine or vinorelbine is an alternative, or vinorelbine on its own, although a survival benefit has not been shown for these drugs. For patients in whom cisplatin cannot be used, carboplatin can be substituted but non-randomised data have shown lower response rates and high rates of haematological toxicity for carboplatin-based combinations, albeit with similar survival figures to patients receiving cisplatin.
In January 2009, the United States FDA approved using conventional therapies such as surgery in combination with radiation and or chemotherapy on stage I or II Mesothelioma after research conducted by a nationwide study by Duke University concluded an almost 50 point increase in remission rates.
In pericardial mesothelioma, chemotherapy - typically adriamycin and/or cisplatin - is primarily used to shrink the tumor and is not curative.
For patients with localized disease, and who can tolerate a radical surgery, radiation can be given post-operatively as a consolidative treatment. The entire hemithorax is treated with radiation therapy, often given simultaneously with chemotherapy. Delivering radiation and chemotherapy after a radical surgery has led to extended life expectancy in selected patient populations. It can also induce severe side-effects, including fatal pneumonitis. As part of a curative approach to mesothelioma, radiotherapy is commonly applied to the sites of chest drain insertion, in order to prevent growth of the tumor along the track in the chest wall.
Although mesothelioma is generally resistant to curative treatment with radiotherapy alone, palliative treatment regimens are sometimes used to relieve symptoms arising from tumor growth, such as obstruction of a major blood vessel. Radiation therapy, when given alone with curative intent, has never been shown to improve survival from mesothelioma. The necessary radiation dose to treat mesothelioma that has not been surgically removed would be beyond human tolerance. Radiotherapy is of some use in pericardial mesothelioma.
The goal of treatment of malignant pleural effusions is relief of breathlessness. Occasionally, treatment of the underlying cancer can cause resolution of the effusion. This may be the case with types of cancer that respond well to chemotherapy, such as small cell carcinoma or lymphoma. Simple aspiration of pleural fluid can relieve breathlessness rapidly but fluid and symptoms will usually recur within a couple of weeks. For this reason, more permanent treatments are usually used to prevent fluid recurrence. Standard treatment involves chest tube insertion and pleurodesis. However, this treatment requires an inpatient stay of approximately 2–7 days, can be painful and has a significant failure rate. This has led to the development of tunneled pleural catheters (e.g., Pleurx Catheters), which allow outpatient treatment of effusions.
Given its rarity, there are no established guidelines for the treatment of peritoneal mesothelioma. The modern approach to malignant peritoneal mesothelioma includes cytoreductive surgery, hyperthermic intraperitoneal chemotherapy (HIPEC), intraperitoneal chemotherapy, and intravenous chemotherapy. These are often used in conjunction and in a complementary fashion, and this multifaceted approach has significantly improved outcomes when compared to intravenous chemotherapy alone. For instance, the reported median survival time for patients with stage IV mesothelioma as reported by the American Cancer Society is 12 months; however, with adequate cytoreduction, intraperitoneal, and intravenous chemotherapy combined, some authors report 10-year survival rates projected at nearly 75%.
Multiple factors have been shown to be significant in predicting the outcome and overall survival. Age greater than 60 at surgery, more overall disease burden (defined as a PCI greater than 15), complete cytoreduction (no visible disease), and epitheliod subtype pathology have all been shown to be predictors of both mortality and disease progression. These known predictors notwithstanding, many patients with advanced peritoneal mesothelioma are still surgical candidates, and even patients with the highest possible score on the peritoneal carcinomatosis index (39) can be completely reduced to a PCI of 0 with adequate surgery.
The Stehlin Foundation currently offers DSRCT patients the opportunity to send samples of their tumors free of charge for testing. Research scientists are growing the samples on nude mice and testing various chemical agents to find which are most effective against the individual's tumor.
Patients with advanced DSRCT may qualify to participate in clinical trials that are researching new drugs to treat the disease.
The prognosis for DSRCT remains poor. Prognosis depends upon the stage of the cancer. Because the disease can be misdiagnosed or remain undetected, tumors frequently grow large within the abdomen and metastasize or seed to other parts of the body.
There is no known organ or area of origin. DSRCT can metastasize through lymph nodes or the blood stream. Sites of metastasis include the spleen, diaphragm, liver, large and small intestine, lungs, central nervous system, bones, uterus, bladder, genitals, abdominal cavity, and the brain.
A multi-modality approach of high-dose chemotherapy, aggressive surgical resection, radiation, and stem cell rescue improves survival for some patients. Reports have indicated that patients will initially respond to first line chemotherapy and treatment but that relapse is common.
Some patients in remission or with inoperable tumor seem to benefit from long term low dose chemotherapy, turning DSRCT into a chronic disease.
There is no cure available for asbestosis. Oxygen therapy at home is often necessary to relieve the shortness of breath and correct underlying low blood oxygen levels. Supportive treatment of symptoms includes respiratory physiotherapy to remove secretions from the lungs by postural drainage, chest percussion, and vibration. Nebulized medications may be prescribed in order to loosen secretions or treat underlying chronic obstructive pulmonary disease. Immunization against pneumococcal pneumonia and annual influenza vaccination is administered due to increased sensitivity to the diseases. Those with asbestosis are at increased risk for certain cancers. If the person smokes, quitting the habit reduces further damage. Periodic pulmonary function tests, chest x-rays, and clinical evaluations, including cancer screening/evaluations, are given to detect additional hazards.
Peritoneal mesothelioma is the name given to the cancer that attacks the lining of the abdomen. This type of cancer affects the lining that protects the contents of the abdomen and which also provides a lubricating fluid to enable the organs to move and work properly.
The peritoneum is made of two parts, the visceral and parietal peritoneum. The visceral peritoneum covers the internal organs and makes up most of the outer layer of the intestinal tract. Covering the abdominal cavity is the parietal peritoneum.
Malignant pleural effusion is a condition in which cancer causes an abnormal amount of fluid to collect between the thin layers of tissue (pleura) lining the outside of the lung and the wall of the chest cavity. Lung cancer and breast cancer account for about 50-65% of malignant pleural effusions. Other common causes include pleural mesothelioma and lymphoma.
Asbestos can cause lung cancer that is identical to lung cancer from other causes. Exposure to asbestos is associated with all major histological types of lung carcinoma (adenocarcinoma, squamous cell carcinoma, large-cell carcinoma and small-cell carcinoma). The latency period between exposure and development of lung cancer is 20 to 30 years. It is estimated that 3%-8% of all lung cancers are related to asbestos. The risk of developing lung cancer depends on the level, duration, and frequency of asbestos exposure (cumulative exposure). Smoking and individual susceptibility are other contributing factors towards lung cancer. Smokers who have been exposed to asbestos are at far greater risk of lung cancer. Smoking and asbestos exposure have a multiplicative (synergistic) effect on the risk of lung cancer. Symptoms include chronic cough, chest pain, breathlessness, haemoptysis (coughing up blood), wheezing or hoarseness of the voice, weight loss and fatigue. Treatment involves surgical removal of the cancer, chemotherapy, radiotherapy, or a combination of these (multimodality treatment). Prognosis is generally poor unless the cancer is detected in its early stages. Out of all patients diagnosed with lung cancer, only 15% survive for five years after diagnosis.
The treatment of choice for both benign and malignant SFT is complete "en bloc" surgical resection.
Prognosis in benign SFTs is excellent. About 8% will recur after first resection, with the recurrence usually cured after additional surgery.
The prognosis in malignant SFTs is much more guarded. Approximately 63% of patients will have a recurrence of their tumor, of which more than half will succumb to disease progression within 2 years. Adjuvant chemotherapy and/or radiotherapy in malignant SFT remains controversial.
Radiation (radiotherapy) is frequently used for the treatment of many cancer types, and can be highly effective. Unfortunately, it also can lead to pulmonary toxicity as a side effect.
Radiotherapists are well aware of possible pulmonary toxicity, and take a number of precautions to minimise the incidence of this side effect. There are research efforts to possibly eliminate this side effect in the future.
The "medical benefit-cost ratio" for the patient should be kept in mind. Medicinal drugs should not be ruled out completely right from the start just because they possibly could cause pulmonary toxicity. A number of medicinal drugs that could cause pulmonary toxicity can be life-saving for certain patients with specific diseases. For example, amiodarone falls into this category. Ideally, the pros and cons should be weighed at the start of therapy and in regular intervals thereafter, based on the available scientific/medical evidence, by an expert physician, together with an informed patient.
Many occupational cancers are preventable. Personal protective gear, workplace controls, and worker education can prevent exposure to carcinogens in the workplace. Tobacco smoking has also been shown to increase the risk of work-related cancers; decreasing or abstaining from smoking can decrease cancer risk.
Agencies like the US Food and Drug Administration, Environmental Protection Agency, and Occupational Safety and Health Administration have developed safety standards and limits for chemical and radiation exposure.
Lewis lung carcinoma is a tumor discovered by Dr. Margaret R. Lewis of the Wistar Institute in 1951. This tumor originated spontaneously as a carcinoma of the lung of a C57BL mouse. The tumor does not appear to be grossly hemorrhagic and the majority of the tumor tissue is a semifirm homogeneous mass. It is also called 3LL and LLC and is used as a transplantable malignancy. It has been used in many studies.
In 1975, Munson discovered that cannabinoids suppress Lewis lung carcinoma cell growth. The mechanism of this action was shown to be inhibition of DNA synthesis Cannabinoids increase the life span of mice carrying Lewis lung tumors and decrease primary tumor size. There are multiple modes of action.
Malignant mesothelioma is an aggressive and incurable tumour caused by asbestos arising from mesothelial cells of the pleura, peritoneum (the lining of the abdominal cavity) and rarely elsewhere. Pleural mesothelioma is the most common type of mesothelioma, representing about 75 percent of cases. Peritoneal mesothelioma is the second most common type, consisting of about 10 to 20 percent of cases. Mesothelioma appears from 20 to 50 years after the initial exposure to asbestos. The symptoms include shortness of breath, chronic chest pain, cough, and weight loss. Diagnosing mesothelioma is often difficult and can include physical examination, chest X-ray and lung function tests, followed by CT scan and MRI. A biopsy is needed to confirm a diagnosis of malignant mesothelioma. Mesothelioma has a poor prognosis, with most patients dying within 1 year of diagnosis. The treatment strategies include surgery, radiotherapy, chemotherapy or multimodality treatment. Several tumour biomarkers (soluble mesothelin-related protein (SMRP), osteopontin and fibulin3) have been evaluated for diagnostic purposes to allow early detection of this disease. Novel biomarkers such as volatile organic compounds measured in exhaled breath are also promising.
Occupational exposure to chemicals, dusts, radiation, and certain industrial processes have been tied to occupational cancer. Exposure to cancer-causing chemicals, also called Carcinogens, may cause mutations that allow cells to grow out of control, causing cancer. Carcinogens in the workplace may include chemicals like anilines, chromates, dinitrotoluenes, arsenic and inorganic arsenic compounds, beryllium and beryllium compounds, cadmium compounds, and nickel compounds. Dusts that can cause cancer leather or wood dusts, asbestos, crystalline forms of silica, coal tar pitch volatiles, coke oven emissions, diesel exhaust and environmental tobacco smoke. sunlight; radon gas; and industrial, medical, or other exposure to ionizing radiation can all cause cancer in the workplace. Industrial processes associated with cancer include aluminum production; iron and steel founding; and underground mining with exposure to uranium or radon.
Other factors that play a role in cancer include:
- Personal characteristics such as age, sex, and race
- Family history of cancer
- Diet and personal habits such as cigarette smoking and alcohol consumption
- The presence of certain medical conditions or past medical treatments, including chemotherapy, radiation treatment, or some immune-system suppressing drugs.
- Exposure to cancer-causing agents in the environment (for example, sunlight, radon gas, air pollution, and infectious agents)
Tumor-like disorders of the lung pleura are a group of conditions that on initial radiological studies might be confused with malignant lesions. Radiologists must be aware of these conditions in order to avoid misdiagnosing patients. Examples of such lesions are: pleural plaques, thoracic splenosis, catamenial pneumothorax, pleural pseudotumor, diffuse pleural thickening, diffuse pulmonary lymphangiomatosis and Erdheim-Chester Disease.
Solitary fibrous tumor (SFT), also known as fibrous tumor of the pleura, is a rare mesenchymal tumor originating in the pleura or at virtually any site in the soft tissue including seminal vesicle. Approximately 78% to 88% of SFT's are benign and 12% to 22% are malignant.
Ectopic endometrial tissue reaches the pleural space of the lung or the right hemi-diaphragmatic region and erodes the visceral pleura, causing the formation of a spontaneous pneumothorax. The condition is often cyclical, due to its associations with the beginning of the menstrual cycle.
Affected persons usually present with recurrent spontaneous pneumothorax associated with the onset of the menstrual cycle. Additionally, chest/scapular pain and/or evidence of endometriosis in the abdominopelvic cavity are other manifestations.
On radiological studies, pneumothorax is visualized using conventional chest x-rays and CT scans. In 90% of the cases, the pneumothorax is located on the right side. In some cases, small nodules can be seen in the pleura using CT scans. Confirmation can be done using video assisted thoracoscopic surgery (VATS).
Treatment for the pneumothorax is with chest tube placement. As for the ectopic endometrial tissue, therapy with gonadotropin-releasing–hormone or resection of the lesions can improve symptoms.
Appearance and location of the tumor is enough to identify it as a mammary tumor. Biopsy will give type and invasiveness of the tumor. In addition, newer studies showed that certain gene expression patterns are associated with malignant behaviour of canine mammary tumors.
Surgical removal is the treatment of choice, but chest x-rays should be taken first to rule out metastasis. Removal should be with wide margins to prevent recurrence, taking the whole mammary gland if necessary. Because 40 to 50 percent of dog mammary tumors have estrogen receptors, spaying is recommended by many veterinarians. A recent study showed a better prognosis in dogs that are spayed at the time of surgery or that had been recently spayed. However, several other studies found no improvement of disease outcome when spaying was performed after the tumor had developed. Chemotherapy is rarely used.
Asbestosis is long term inflammation and scarring of the lungs due to asbestos. Symptoms may include shortness of breath, cough, wheezing, and chest pain. Complications may include lung cancer, mesothelioma, and pulmonary heart disease.
Asbestosis is caused by breathing in asbestos fibers. Generally it required a relatively large exposure over a long period of time. Such levels of exposure typically only occur in those who work with the material. All types of asbestos fibers are associated with concerns. It is generally recommended that currently existing asbestos be left undisturbed. Diagnosis is based upon a history of exposure together with medical imaging. It is a type of interstitial pulmonary fibrosis.
There is no specific treatment. Recommendations may include stopping smoking, influenza vaccination, pneumococcal vaccination, or oxygen therapy. Asbestosis affected about 157,000 people and resulted in 3,600 deaths in 2015. Asbestos use has been banned in a number of countries in an effort to prevent disease.
Pleural tumors may be benign (i.e. solitary fibromas) or malignant in nature. Pleural Mesothelioma is a type of malignant cancer associated with asbestos exposure.
- Mesothelial tumors: pleural malignant mesothelioma.
- Pleural sarcomas
- Pleural angiosarcoma
- Pleural desmoplastic small round cell tumor (pleural DSRCT)
- Pleural synovial sarcoma
- Pleural solitary fibrous tumor (pleural SFT)
- Smooth muscle tumors of the pleura
- Pleural carcinomas
- Pleural mucoepidermoid carcinoma
- Pleural pseudomesotheliomatous adenocarcinoma
The treatment of cystic tumours of the atrioventricular nodal region is surgical excision.
No specific treatment or cure exists. Affected children usually need total parenteral nutrition through a central venous catheter. Further worsening of liver damage should however be avoided if possible. Diarrhea will likely continue even though food stops passing through the gastrointestinal system. They can subsequently be managed with tube feeding, and some may be weaned from nutritional support during adolescence.