Types of Mesothelioma include pleural mesothelioma, peritoneal mesothelioma and pericardial mesothelioma. Mesothelioma cancer affects the mesothelial tissue lining of the body's three largest cavities; the pleura (lung cavity), the peritoneum (abdominal cavity) and the pericardium (heart sac). A type of malignant mesothelioma is based on the section of the mesothelium that it affects.
There are three main types of mesothelioma, and each affects a different area of the body. The three types of this deadly Mesothelioma cancer are:
Pleural Mesothelioma ( Malignant Pleural Mesothelioma)
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
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
Pleural Mesothelioma Staging
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 Overview
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 (http://www.mesorfa.org/specialist.htm).
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.
Localized Malignant Mesothelioma (Stage I)
Standard treatment options:
1. Solitary mesotheliomas: Surgical resection en bloc (as a whole, or in one large piece) including contiguous structures to ensure wide disease-free margins. Sessile polypoid lesions (tumors sticking up from the pleura) should be treated with surgical resection to maximize the potential for cure.
2. Intracavitary mesothelioma:
- Palliative surgery (i.e., pleurectomy and decortication) with or without postoperative radiation therapy. Palliation means treatment to lessen side effects rather than treatment to try to cure the patient.
- Extrapleural pneumonectomy (removing the affected lung)
- Palliative radiation therapy. Palliation means treatment to lessen side effects rather than treatment to try to cure the patient.
Treatment options under clinical evaluation:
1. Intracavitary chemotherapy (putting chemotherapy directly into the space around the lungs) following surgery to remove the cancer.
2. Multimodality therapy (using more than one mode of treatment).
Treatment for Advanced Malignant Mesothelioma (Stages II, III, and IV)
Standard treatment options:
- Treatment of symptoms including drainage of effusions, chest tube pleurodesis, or thoracoscopic pleurodesis. Pleurodesis is a medical procedure that uses chemicals or drugs to cause inflammation and adhesion between the layers of the pleura (the tissue that covers the lungs and lines the interior wall of the chest cavity). This prevents the buildup of fluid in the pleural cavity. It is used as a treatment for severe pleural effusion.
- Palliative surgical resection in selected patients. Palliation means treatment to lessen side effects rather than treatment to try to cure the patient.
- Palliative radiation therapy.
- Single-agent chemotherapy. Partial responses have been reported with doxorubicin, epirubicin, mitomycin, cyclophosphamide, cisplatin, carboplatin, and ifosfamide.
- Combination chemotherapy (under clinical evaluation).
- Multimodality clinical trials.
- Intracavitary therapy. Intrapleural administration of chemotherapeutic agents (e.g., cisplatin, mitomycin, and cytarabine) has been reported to produce transient reduction in the size of tumor masses and temporary control of effusions in small clinical studies. Additional studies are needed to define the role of intracavitary therapy.
Treatment of Recurrent Malignant Mesothelioma
Treatment of recurrent mesothelioma uses procedures and/or agents (drugs) not previously employed in the initial treatment attempt. No standard treatment approaches have been proven to improve survival or control symptoms for a prolonged period of time. These patients should be considered candidates for phase I and II clinical trials evaluating new biologicals , chemotherapeutic agents, or physical approaches (new surgeries or radiation therapy approaches.) Biological therapies use the body's immune system to fight cancer or to lessen the side effects that may be caused by some cancer treatments.
Peritoneal Mesothelioma ( Malignant Peritoneal Mesothelioma)
Mesothelioma is a rare form of cancer in which malignant (cancerous) cells are found in the mesothelium, a protective sac that covers most of the body's internal organs. The incidence of malignant peritoneal mesothelioma (MPM) is approximately 2 to 2.6 cases per million annually. Most people who develop mesothelioma have worked on jobs where they inhaled asbestos particles.
The mesothelium is a membrane that covers and protects most of the internal organs of the body. It is composed of two layers of cells: One layer immediately surrounds the organ; the other forms a sac around it. The mesothelium produces a lubricating fluid that is released between these layers, allowing moving organs (such as the beating heart and the expanding and contracting lungs) to glide easily against adjacent structures. The mesothelium has different names, depending on its location in the body. The peritoneum is the mesothelial tissue that covers most of the organs in the abdominal cavity.
Mesothelioma (cancer of the mesothelium) is a disease in which cells of the mesothelium become abnormal and divide without control or order. They can invade and damage nearby tissues and organs. Cancer cells can also metastasize (spread) from their original site to other parts of the body. Most cases of mesothelioma begin in the pleura or peritoneum.
Although reported incidence rates have increased in the past twenty years, mesothelioma is still a relatively rare cancer. About 2,000 new cases of mesothelioma are diagnosed in the United States each year. Mesothelioma occurs more often in men than in women and risk increases with age, but this disease can appear in either men or women at any age.
Symptoms of Peritoneal Mesothelioma
Symptoms of peritoneal mesothelioma include weight loss, and abdominal pain and swelling due to a buildup of fluid (effusion) in the abdomen. Other symptoms may include bowel obstruction, blood clotting abnormalities, anemia, and fever. If the cancer has spread beyond the mesothelium to other parts of the body, symptoms may include pain, trouble swallowing, or swelling of the neck or face.
These symptoms may be caused by mesothelioma or by other, less serious conditions. It is important to see a doctor about any of these symptoms. Only a doctor can make a diagnosis.
Diagnosis of Peritoneal Mesothelioma
Diagnosing mesothelioma is often difficult because the symptoms are similar to those of a number of other conditions. Diagnosis begins with a review of the patient's medical history, including any history of asbestos exposure. A complete physical examination may be performed, including x-rays of the chest or abdomen. A CT (or CAT) scan or an MRI may also be useful. A CT scan is a series of detailed pictures of areas inside the body created by a computer linked to an x-ray machine. In an MRI, a powerful magnet linked to a computer is used to make detailed pictures of areas inside the body. These pictures are viewed on a monitor and can also be printed.
A biopsy is needed to confirm a diagnosis of mesothelioma. In a biopsy, a surgeon or a medical oncologist (a doctor who specializes in diagnosing and treating cancer) removes a sample of tissue for examination under a microscope by a pathologist. To do this the doctor may perform a peritoneoscopy. To obtain tissue for examination, the doctor makes a small opening in the abdomen and inserts a special instrument called a peritoneoscope into the abdominal cavity. If these procedures do not yield enough tissue, more extensive diagnostic surgery may be necessary.
Staging of Peritoneal Mesothelioma
If the diagnosis is mesothelioma, the doctor will want to learn the stage (or extent) of the disease. Staging involves more tests in a careful attempt to find out whether the cancer has spread and, if so, to which parts of the body. Knowing the stage of the disease helps the doctor plan treatment.
MPM is described as localized if the cancer is found only on the membrane surface where it originated. It is classified as advanced if it has spread beyond the original membrane surface to other parts of the body, such as the lymph nodes, or abdominal organs.
Treatment of Peritoneal Mesothelioma
Treatment for mesothelioma depends on the location of the cancer, the stage of the disease, and the patient's age and general health. Standard treatment options include surgery, radiation therapy, and chemotherapy. Sometimes, these treatments are combined.
- Surgery is a common treatment for mesothelioma. The doctor may remove part of the lining of the abdomen and some of the tissue around it.
- Radiation therapy, also called radiotherapy, involves the use of high-energy rays to kill cancer cells and shrink tumors. Radiation therapy affects the cancer cells only in the treated area. The radiation may come from a machine (external radiation) or from putting materials that produce radiation through thin plastic tubes into the area where the cancer cells are found (internal radiation therapy).
- Chemotherapy is the use of anticancer drugs to kill cancer cells throughout the body. Most drugs used to treat mesothelioma are given by injection into a vein (intravenous, or IV). Doctors are also studying the effectiveness of putting chemotherapy directly into the abdomen (intracavitary chemotherapy).
To relieve symptoms and control pain, the doctor may use a needle or a thin tube to drain fluid (effusions) that has built up in the abdomen. Removal of fluid from the abdomen is called paracentesis. Radiation therapy and surgery may also be helpful in relieving symptoms.
Pericardial Mesothelioma (Malignant Mesothelioma of The Pericardium)
Pericardial mesothelioma is much less common than malignant mesothelioma of the pleura or peritoneum. In fact there are only about 150 cases ever reported in the medical literature. It affects the section of the mesothelium called the pericardium (the mesothelial lining of the heart). People in the fourth to seventh decades of life are most likely to have this cancer, and there is a 2:1 male to female ratio. Currently, surgical excision (removal) of the pericardium is the treatment for pericardial mesothelioma, primarily to lessen symptoms of constriction around the heart.
Symptoms that are associated with pericardial mesothelioma include:
- Chest pain
- Fluid buildup around the heart
- A mass in the space between the lungs
- Abnormal or difficult breathing (dyspnea)
- Chronic coughing
- Irregular heartbeat (palpitations)
A recent review of the primary pericardial mesothelioma has been published by Vigneswaran and Stefanacci. It is a rare neoplasm with a reported incidence of 0.0022% in an autopsy series of 5,000,000 case studies 212 and a calculated annual incidence of 1 in 40 million in a Canadian epidemiologic survey. An antemortem diagnosis was made in less than one-third of 150 reported cases in the literature. Pericardial mesotheliomas can occur at any age, but people in the fourth to seventh decades of life are most likely to be afflicted, and there is a 2:1 male to female ratio. Patients generally present with a pericardial effusion, congestive heart failure, an anterior mediastinal mass, or tamponade. Diagnosis can be difficult given the nonspecific presentation, and chest radiography may demonstrate only an enlarged cardiac silhouette. Echocardiography can reveal evidence of an effusion, thickening of the pericardium, or mass involvement of the myocardium. CT scanning or MRI can show a thickened pericardium and may help determine invasion into myocardium.
Currently, surgical excision is the treatment for primary pericardial mesothelioma primarily to palliate symptoms of constriction or tamponade.
Mesothelioma of the Tunica Vaginalis Testis
Mesothelioma of the tunica vaginalis testis is the least common type of malignant mesothelioma (amounting to less than 100 of all documented cases of the disease) and affects the section of the mesothelium called the tunica vaginalis testis (the mesothelial lining around the testes). Most patients are in their 50s or older, but about ten percent of the patients are younger than 25 years. Patients generally present with a hydrocele (an accumulation of serous fluid in a sac-like cavity (as the scrotum)) or hernia. Treatment is usually a high inguinal orchiectomy (surgical excision of the entire affected testis through an incision in the lower abdomen - called also orchidectomy). Prognosis is somewhat better than for pleural mesothelioma.
Symptoms that are associated with this cancer include:
- Hydrocele (a fluid filled sac attached to a testicle)
- Suspected hernia
Fewer than 100 cases of gonadal mesothelioma have been reported in the literature, and although most patients are in their 50s or older, approximately 10% of the patients are younger than 25 years. Asbestos exposure is documented in approximately one-half of the more recently reported cases. Patients generally present with a hydrocele or hernia. An accurate preoperative diagnosis has been reported in only two cases.
All patients with a suspected testicular malignancy should undergo a radical or high inguinal orchiectomy.
Local resection of the tumor or hydrocelectomy is associated with a high recurrence rate compared with high inguinal orchiectomy. Because preoperative diagnosis of gonadal mesothelioma is difficult, management should be as for any testicular tumor. The inguinal approach avoids interruption of the scrotal lymphatics, which would alter the metastatic pathway of the tumor, and also allows complete removal of the spermatic cord up to the internal ring. Patients with evidence of disease extending into the retroperitoneal nodes should undergo a retroperitoneal lymphadenectomy.
The overall recurrence rate (local and disseminated) for gonadal mesothelioma can be as high as 52%, with 38% of patients dying of disease progression. Local recurrence occurs in 36% of patients who undergo local resection of the hydrocele wall; 10% after scrotal orchiectomy and 12% after inguinal orchiectomy. More than 60% of recurrences developed within the first 2 years of the follow-up. The median survival of the patients averaged 23 months. There are little data regarding the use of adjuvant therapy after resection of gonadal mesothelioma.
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