Pleural Mesothelioma is the most common type of
malignant mesothelioma (accounting for an approximate 75% of all documented cases of the
disease) and affects the section of the
mesothelium called the
pleura. Although the most common type of
malignant mesothelioma, the disease is still somewhat of a rarity. As a result,
pleural mesothelioma is often confused with other types of diseases, such as
lung cancer and viral
pneumonia.
Lung cancer can be caused by
asbestos (asbestos lung cancer), though it differs from
pleural mesothelioma in that it is a
malignancy of the lung
tissue itself, as opposed to pleural mesothelioma which is a malignancy of the tissue casing of the
lungs. Viral pneumonia shares certain symptomatic similarities with pleural mesothelioma and is often misdiagnosed as such.
The most common presenting symptom of pleural malignant mesothelioma is chronic chest pain. A buildup of fluid inside the pleural space can cause severe and chronic chest pains; this is called pleural effusion. Steps can be taken to drain the fluid and relieve the pain (with the possibility of recurrence) or surgery can be performed to close the pleural space (with virtually no possibility of recurrence). Some of the other notable symptoms associated with pleural mesothelioma include:
- Shortness of breath
- Chronic coughing
- Weight loss
- Fever
Prognosis in this disease is difficult to assess consistently because there is great variability in the time before diagnosis and the rate of disease progression. In large studies of pleural mesothelioma patients, important prognostic factors were found to be stage, age, performance status, and histology. Histology means the specific type of mesothelioma cancer cells that are found. Various surgical procedures may be possible in selected patients. They typically provide long-term survival without cure. For patients treated with aggressive surgical approaches, factors associated with improved long-term survival include epithelial histology, no cancer found in the lymph nodes, and negative surgical margins. That means that the surgeons were able to remove all of the cancer, as far as they can tell, and there is a margin of healthy tissue around the tumor that they removed. For those patients treated with aggressive surgical approaches, nodal status is an important prognostic factor. In other words, if you had cancer in your lymph nodes your prognosis is worse than if no cancer was found in the nodes.
Median survival has been reported as 16 months from date of diagnosis for patients with malignant pleural disease confined to the pleura, and 5 months from date of diagnosis for patients with extensive disease. In some instances the tumor grows through the diaphragm making the site of origin difficult to assess. The diaphragm is the thin muscle that separates the lungs from the abdomen. The diaphragm helps you breathe. Cautious interpretation of treatment results with this disease is important because of the selection differences among series. Patient selection can influence the outcomes of clinic trials. Effusions, both pleural and peritoneal, represent major symptomatic problems for at least two-thirds of patients (the National Cancer Institute has a statement on Cardiopulmonary Syndromes for more information on this subject).
Patients with stage I disease have a significantly better prognosis than those with more advanced stages. Because of the relative rarity of this disease, exact survival information based upon stage is limited. A proposed staging system based upon thoracic surgery principles and clinical data is shown below. It is a modification of an older system. Other staging systems that have been employed include the current international TNM staging system.
- Stage I: Disease confined within the capsule of the parietal pleura (i.e., ipsilateral (on the same side) pleura, lung, pericardium, and diaphragm).
- Stage II: All of stage I with positive intrathoracic (N1 or N2) lymph nodes.
- Stage III: Local extension of disease into the following areas, e.g., chest wall or mediastinum, heart or through the diaphragm or peritoneum, with or without extrathoracic (outside of the thorax) or contralateral (on the opposite side of) (N3) lymph node involvement.
- Stage IV: Distant metastatic disease, meaning spread of the cancer to distant sites.
These stages are then put into two groups: Localized malignant mesothelioma, which is defined as stage I described above; and advanced malignant mesothelioma which includes stages II, III, and IV above. In practice, mesothelioma is generally categorized as either localized or advanced and the stage numbers like stage II or III are not used.
Standard treatment for all but localized mesothelioma is generally not curative. Although some patients will experience long-term survival with aggressive treatment approaches, it remains unclear if overall survival has been significantly altered by the different treatment modalities or by combinations of modalities. Surgery and chemotherapy are examples of different treatment modalities. Extrapleural pneumonectomy in selected patients with early stage disease may improve recurrence-free survival, but its impact on overall survival is unknown. Extrapleural pneumonectomy is surgery to remove a diseased lung, part of the pericardium (membrane covering the heart), part of the diaphragm (muscle between the lungs and the abdomen), and part of the parietal pleura (membrane lining the chest). Pleurectomy (removing the pleura) and decortication (removal of part or all of the external surface of an organ) can provide relief from symptomatic effusions, discomfort caused by tumor burden, and pain caused by invasive tumor. Operative mortality from pleurectomy/decortication is less than 2%, while mortality from extrapleural pneumonectomy has ranged from 6% to 30%. Given the high mortality rate from a pneumonectomy, it is important to find a surgeon who has significant experience in doing such an operation. See our list of mesothelioma specialists to find experienced surgeons .
The addition of radiation therapy and/or chemotherapy following surgical intervention has not demonstrated improved survival. The use of radiation therapy in pleural mesothelioma has been shown to alleviate pain in the majority of patients treated; however, the duration of symptom control is short-lived. Single-agent and combination chemotherapy have been evaluated in single and combined modality studies. The most studied agent is doxorubicin, which has produced partial responses in approximately 15% to 20% of patients studied. Some combination chemotherapy regimens have been reported to have higher response rates in small phase II trials; however, the toxic effects reported are also higher, and there is no evidence that combination regimens result in longer survival or longer control of symptoms. Recurrent pleural effusions may be treated with pleural sclerosing procedures. However, the efficacy of these procedures depends on the bulk of the tumor. If the tumor is too large they may not be helpful.
Pleural Mesothelioma
Pleural Mesothelioma is the most common type of malignant mesothelioma (accounting for an approximate 75% of all documented cases of the disease) and affects the section of the mesothelium called the pleura. Although the most common type of malignant mesothelioma, the disease is still somewhat of a rarity. As a result, pleural mesothelioma is often confused with other types of diseases, such as lung cancer and viral pneumonia. Lung cancer can be caused by asbestos (asbestos lung cancer), though it differs from pleural mesothelioma in that it is a malignancy of the lung tissue itself, as opposed to pleural mesothelioma which is a malignancy of the tissue casing of the lungs. Viral pneumonia shares certain symptomatic similarities with pleural mesothelioma and is often misdiagnosed as such.
The most common presenting symptom of pleural malignant mesothelioma is chronic chest pain. A buildup of fluid inside the pleural space can cause severe and chronic chest pains; this is called pleural effusion. Steps can be taken to drain the fluid and relieve the pain (with the possibility of recurrence) or surgery can be performed to close the pleural space (with virtually no possibility of recurrence). Some of the other notable symptoms associated with pleural mesothelioma include:
Shortness of breath
Chronic coughing
Weight loss
Fever
Prognosis in this disease is difficult to assess consistently because there is great variability in the time before diagnosis and the rate of disease progression. In large studies of pleural mesothelioma patients, important prognostic factors were found to be stage, age, performance status, and histology. Histology means the specific type of mesothelioma cancer cells that are found. Various surgical procedures may be possible in selected patients. They typically provide long-term survival without cure. For patients treated with aggressive surgical approaches, factors associated with improved long-term survival include epithelial histology, no cancer found in the lymph nodes, and negative surgical margins. That means that the surgeons were able to remove all of the cancer, as far as they can tell, and there is a margin of healthy tissue around the tumor that they removed. For those patients treated with aggressive surgical approaches, nodal status is an important prognostic factor. In other words, if you had cancer in your lymph nodes your prognosis is worse than if no cancer was found in the nodes.
Median survival has been reported as 16 months from date of diagnosis for patients with malignant pleural disease confined to the pleura, and 5 months from date of diagnosis for patients with extensive disease. In some instances the tumor grows through the diaphragm making the site of origin difficult to assess. The diaphragm is the thin muscle that separates the lungs from the abdomen. The diaphragm helps you breathe. Cautious interpretation of treatment results with this disease is important because of the selection differences among series. Patient selection can influence the outcomes of clinic trials. Effusions, both pleural and peritoneal, represent major symptomatic problems for at least two-thirds of patients (the National Cancer Institute has a statement on Cardiopulmonary Syndromes for more information on this subject).
Staging of Pleural Mesothelioma
Patients with stage I disease have a significantly better prognosis than those with more advanced stages. Because of the relative rarity of this disease, exact survival information based upon stage is limited. A proposed staging system based upon thoracic surgery principles and clinical data is shown below. It is a modification of an older system. Other staging systems that have been employed include the current international TNM staging system.
Stage I: Disease confined within the capsule of the parietal pleura (i.e., ipsilateral (on the same side) pleura, lung, pericardium, and diaphragm).
Stage II: All of stage I with positive intrathoracic (N1 or N2) lymph nodes.
Stage III: Local extension of disease into the following areas, e.g., chest wall or mediastinum, heart or through the diaphragm or peritoneum, with or without extrathoracic (outside of the thorax) or contralateral (on the opposite side of) (N3) lymph node involvement.
Stage IV: Distant metastatic disease, meaning spread of the cancer to distant sites.
These stages are then put into two groups: Localized malignant mesothelioma, which is defined as stage I described above; and advanced malignant mesothelioma which includes stages II, III, and IV above. In practice, mesothelioma is generally categorized as either localized or advanced and the stage numbers like stage II or III are not used.
Treatment Option for Pleural Mesothelioma
Standard treatment for all but localized mesothelioma is generally not curative. Although some patients will experience long-term survival with aggressive treatment approaches, it remains unclear if overall survival has been significantly altered by the different treatment modalities or by combinations of modalities. Surgery and chemotherapy are examples of different treatment modalities. Extrapleural pneumonectomy in selected patients with early stage disease may improve recurrence-free survival, but its impact on overall survival is unknown. Extrapleural pneumonectomy is surgery to remove a diseased lung, part of the pericardium (membrane covering the heart), part of the diaphragm (muscle between the lungs and the abdomen), and part of the parietal pleura (membrane lining the chest). Pleurectomy (removing the pleura) and decortication (removal of part or all of the external surface of an organ) can provide relief from symptomatic effusions, discomfort caused by tumor burden, and pain caused by invasive tumor. Operative mortality from pleurectomy/decortication is less than 2%, while mortality from extrapleural pneumonectomy has ranged from 6% to 30%. Given the high mortality rate from a pneumonectomy, it is important to find a surgeon who has significant experience in doing such an operation. See our list of mesothelioma specialists to find experienced surgeons .
The addition of radiation therapy and/or chemotherapy following surgical intervention has not demonstrated improved survival. The use of radiation therapy in pleural mesothelioma has been shown to alleviate pain in the majority of patients treated; however, the duration of symptom control is short-lived. Single-agent and combination chemotherapy have been evaluated in single and combined modality studies. The most studied agent is doxorubicin, which has produced partial responses in approximately 15% to 20% of patients studied. Some combination chemotherapy regimens have been reported to have higher response rates in small phase II trials; however, the toxic effects reported are also higher, and there is no evidence that combination regimens result in longer survival or longer control of symptoms. Recurrent pleural effusions may be treated with pleural sclerosing procedures. However, the efficacy of these procedures depends on the bulk of the tumor. If the tumor is too large they may not be helpful.