Mesothelioma Prognosis

"Prognosis" is the term used by doctors to describe the likely outcome of a disease, how long a diagnosed patient is expected to live, and the quality of life expected during that time. Diffuse malignant mesothelioma is an extremely aggressive disease, and the prognosis is very poor, both for pleural mesothelioma (lung related) and peritoneal mesothelioma (abdominal), as well as pericardial mesothelioma (heart related). Median survival time of 6 to 18 months after diagnosis is reported.

Mesothelioma is classified into three types, based on the type of cells involved: epithelial (50% of cases), sarcomatoid (15% of cases), and mixed type (35% of cases) (1). Epithelial mesothelioma has a better survival rate than the sarcomatoid or mixed types. Mesothelioma is also classified based on site of original involvement. The pleural (lung) type is most common. Approximately 80% of cases are pleural in origin (2). Peritoneal (abdominal) mesothelioma is much more rare.

The prognosis for many cancers depends on how early the disease is diagnosed and treatment is begun. One of the main problems in diagnosing mesothelioma is that the first symptoms can be very vague. Patients may at first visit their doctors with symptoms of shortness of breath or chest pain. These symptoms can be present with a large number of other conditions, and by the time other diseases and conditions are ruled out, the mesothelioma may have advanced quite far, since it develops very rapidly. Sometimes mesothelioma develops without symptoms at first, and the first sign may be a pleural effusion (fluid around the lungs) that is noticed incidentally on a chest x-ray done for other reasons. The mesothelioma gets a jumpstart before it is ever diagnosed, making the prognosis all the more severe.

Following a diagnosis of pleural mesothelioma, chest pain and shortness of breath often grow worse, sometimes followed by weight loss, night sweats, and anorexia. The tumor gradually obliterates the pleural space and replaces any pleural fluid. The lung becomes encased and is not free to move. The lung can be completely covered in a thick rind of tumor of 5 cm or more. Operating to remove this degree of tumor encasement may be of very high risk to the patient, or impossible. Deoxygenated blood is shunted through the lung, which leads to fatigue and low oxygen levels that may not be improved by supplemental oxygen. There may be invasion of the chest wall and nearby structures by tumor mass which can cause increasing pain and other abnormalities such as difficulty of swallowing, compression of the superior vena cava, vocal cord or diaphragm paralysis.

Metastases can occur to the other lung, the brain or other sites, though this is fairly uncommon. A much more common problem is extension into the abdominal cavity. This can be particularly a problem for patients who have had a good response to early aggressive therapy for pleural disease; with the increased survival time comes increased chance of spread to the abdominal cavity.

Death usually occurs from infection or respiratory failure rather than as a direct result of metastatic disease. Extension into the abdomen may cause death by bowel obstruction. Invasion of heart or pericardium may cause death from arrhythmia, heart failure or stroke.

Diffuse malignant peritoneal mesothelioma (DMPM), the more rare type that begins in the abdomen, has overall survival rates averaging one year. Most of these are epithelioid types. There are reports of a more "indolent" type of DMPM which progresses slowly and can have significantly longer survival rates, measured in years rather than months. A major predictor of survival rate in DMPM is the depth of invasion of the bowel wall, with cases of only superficial invasion having a longer survival rate. Surgical resection of affected bowel in these cases may improve life expectancy.

Doctors look at a set of indicators when determining prognosis for any disease condition. In cases of mesothelioma, there are several known factors that are associated with better prognosis or worse prognosis.

Indicators of a better prognosis, and thus longer life expectancy and higher quality of life, include: earlier stage epithelial type of mesothelioma, age under 65 years at diagnosis, absence of chest pain, good performance status (a measurement of cancer patient well-being based on factors such as ability to work, care for self, etc.), and presence of symptoms for more than six months before diagnosis. This last factor may indicate a more "indolent" or less aggressive form of mesothelioma.

Indicators of a poor prognosis include: higher stage presence of high platelet counts in the blood (thrombocytosis), elevated white blood cell count, fever of unknown origin, sarcomatoid or mixed type of mesothelioma, age 65 or older at diagnosis, male, and poor performance status.

Most often, treatment for mesothelioma is "palliative," meaning it is based on easing the symptoms, as there are limited cures with poor survival rates. Radiation therapy can help with reducing the spread of tumor tissue (metastases). Ongoing clinical trials use a combination of radiation, chemotherapy and surgery to increase the poor survival rate.

However, experimental treatments such as gene therapy and immunotherapy offer hope for longer survival. Emerging therapies follow a multimodality approach, attacking the disease with surgery, chemotherapy and radiation therapy, and there are trends suggesting greater survival rates. Aggressive surgery may involve removal of pleura, lung, diaphragm and pericardium. This treatment aims to remove as much tumor tissue as possible while leaving normal lung tissue intact. There are some preliminary statistics that suggest that combining such aggressive surgery with radiation and chemotherapy, for a certain subset of younger, healthier patients, may greatly improve life expectancy and perhaps cure.

Sometimes it can be helpful to look at specific numbers rather than large scale statistical studies. At the Dana-Farber Institute in Boston, doctors followed case histories of 120 patients with pleural mesothelioma from 1980 to 1995. All these patients were treated with surgery and a combination of chemotherapy and radiation therapy. Some received immunotherapy. Of patients with sarcomatoid or mixed type mesothelioma, 20% were alive 2 years later. All had died within 5 years. Patients with epithelioid type had a much better outlook. Those with no involvement of lymph nodes showed a 74% survival rate after 2 years, and 39% survived after 5 years (6). Newer therapies will probably push these survival numbers higher as better combinations of treatments are proved in clinical trials.

Though very rare, there are long-term survivors of mesothelioma with greater than 10 years post diagnosis. Researchers speculate that survival rates in these cases may have more to do with individual differences among patients than the types of therapy they receive.

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