• The survival rate for malignant pleural mesothelioma without treatment ranges from 4 to 12 months.
• The prognosis is poor even with treatment. Fifty percent of patients are dead within 1 year after diagnosis. The prognosis depends on the stage of the tumor, the histological type, and lymph node status.
• Treatment often includes combination therapy involving surgery with radiation, chemotherapy, or immunotherapy.
• Pleurectomy/decortication is the most common surgery performed and involves removing the lining of the chest. This procedure is usually reserved for early stage disease.
• Alimta (pemetrexed) is a new drug recently approved by the FDA for treatment of malignant pleural mesothelioma. Clinical trials have demonstrated an increase in survival time when used in combination with Cisplatin.
Immountherapy with interferon alpha has lead to a reduction in tumor mass in more than 20% of patients.
Although the onset of the disease is insidious, once established, malignant mesothelioma progresses rapidly by local extension to the pericardium, mediastinum, and contralateral pleura. Pain and dyspnea become progressive. Extension to other thoracic structures creates superior vena cava syndrome, hoarseness, and dysphagia. Abdominal extension is common and involves the abdominal lymph nodes and organs. Mesothelioma is associated with the development of paraneoplastic disorders, which include thrombocytosis, hemolytic anemia, Disseminated Intravascular Coagulation, and migratory thromboplebitis. Tumor recurrence rate after surgery is as high as 50%.
Death results from respiratory failure and local extension to other organs. Untreated mesothelioma is fatal in 4-8 months. Patients who receive multimodality care may survive 16-19 months. The five-year survival rate is less than 5%.
Prognosis of mesothelioma is determined by staging the disease
according to severity (stages I-IV). Most people (60%) will die within 2 years of their diagnosis. Only 10% will make it 5 years beyond their initial diagnosis.
Treatment is based on the location and the stage of the illness, along with the overall health of the patient. The aim of treatment is for a cure but the results are more palliative.
Treatment can be surgery, chemo or radiation alone but most frequently it consists of a combination of the 3 treatment methods. Surgical cure is rare but can be attempted by extra pleural pneumonectomy in patients with epitheloidal mesothelioma of the pleura. Most attention is paid to palliative care that will help with the pain and suffering. Thus treatments may consist of various methods of psychotherapy, pain management, nutritional therapy, blood transfusions and a host of other modalities depending on the effects of the disease and the side effects of treatments.
The prognosis for someone with mesothelioma is quite poor. Typically, at the time of diagnosis the disease is in advanced stages and survival time from that point rarely extends past one year, with life expectancy from the time of diagnosis averaging between 4 and 8 months. Some studies have shown only a 10% survival rate at 3 years and 5% survival rate at 5 years.
The epithelioid type has been shown to have a slightly better prognosis; those with Stage I disease also have a slightly better prognosis. Younger age at the time of diagnosis, diagnosis early in the course of disease leads to a better prognosis, and aggressive treatment all improve prognosis.
Localized:
a. Benign – with appropriate surgical resection (1 cm surgical margins), most patients have no further complications of the disease. Transformation from a benign to a malignant mesothelioma is a possibility without proper surgical resection.
b. Malignant – these rates very greatly across literature. Generally speaking, without treatment the disease will be fatal in 4-8 months. With multi-modality treatment (surgery, radiation therapy, and chemotherapy), most patients survive from 8-14 months with some surviving up to two years.
Diffuse:
Diffuse mesothelioma has the poorest prognosis, with an overall average life span after diagnosis of 1 year – with or without treatment. In general, the extent of tumor invasion (chest wall, mediastinum, and diaphragm being common) correlates with length of survival.
Overall prognosis is poor with a mean survival of 8-14 months. Staging is not routinely performed because of the overwhelming lack of surgical exploration. Rather, disease severity is determined to guide treatment. Therefore, information acquired at the time of diagnosis will provide some insight into the prognosis. Sarcomatous subtype histology (accounting for 10%) or uncertain histology is correlated with a poorer prognosis. Poor performance status, male gender and age over 75 consistently carry poor prognosis. Symptoms of chest pain and dyspnea as well as pleural involvement do not bode well for the patient. Certain lab tests will also indicate shorter survival, such as low hemoglobin, platelet count greater than 400 000, WBC count over 8300, or LDH over 500.
The prognosis is generally poor with a median survival rate of four months for patients with metastatic disease and sixteen months for those with localized disease.
With a latency period of 20-40 years from exposure to presentation as well as symptoms which are often confused with other diseases, malignant mesothelioma frequently spreads beyond local disease before the patient presents with symptoms.
Prognosis after treatment often depends on how completely the tumor was resected in surgery. There are four measures of cytoreduction:
CC-0 in which no tumor is visualized
CC-1 in which nodules are seen but less than 2.5 cm; these are likely to respond to chemotherapy
CC-2 in which nodules between 2.5 and 5 cm persist
CC-3 in which nodules greater than 5cm cannot be resected.
Chemotherapy and radiation may help increase rate of survival.
The time from exposure to the development of the disease and its symptoms is very long. Usually it is 30-40 years after exposure before the disease develops. For this reason connecting the symptoms to the cause of the disease can be difficult. Additionally, once these symptoms develop, many are nonspecific and very similar to other more common diagnoses making it difficult to correctly identify the patients diagnosis. This means that the correct treatment is often delayed. These factors contribute to the poor prognosis often associated with this disease.
The average survival once the diagnosis is made averages a year or less. At experienced centers involved in clinical trials this rate is higher and in certain cases approaches a 40% five year survival rate. Early diagnosis and treatment is essential to long term survival.
50%. Excepting the case of stage I disease (a localized, non-invasive lesion), which can often be completely surgically resected, mesothelioma is usually fatal eventually, even with the best available treatment. Treatment strategy will depend on the location, size, and staging of the individual tumor and can include surgery (wedge resection, lobectomy, or pneumonectomy), chemotherapy, radiation, and photodynamic therapy (a chemically-targeted laser therapy). Numerous clinical trials are currently ongoing, which seek to improve survival rates for established therapeutic modalities (especially chemotherapy).
As the disease progresses patients develop chest pain and shortness of breath.
Fatigue, dyspnea and hypoxia ensue. As the lesion invades thoracic structures, the following complications may develop:
- dysphagia
- hoarseness
- cord compression
- brachial plexopathy
- Horner’s syndrome
- superior vena cava syndrome
- disseminated intravascular coagulation
- migratory thrombophlebitis
- thrombocytosis
- coombs-positive hemolytic anemia
- hypoglycemia
- hypercalcemia