An EPP involves the removal of the cancerous lung and parts of the pleura (lining of the chest), the pericardium (lining of the heart), the diaphragm and nearby lymph nodes.
An extrapleural pneumonectomy (EPP) is a complex and invasive procedure. Typically, only patients diagnosed with earlier stages of pleural mesothelioma are considered good candidates for this surgery. Candidates must also be in otherwise good health with a strong heart, as the removal of a lung will increase the workload for the heart and the remaining lung. Overall health is assessed and a performance score is assigned, with lower scores indicating greater health. Candidates for surgery must have a score no higher than two.
Other tests help to determine a patient’s eligibility for EPP, such as blood tests and biopsies. Biopsies reveal the cell type of the tumor, and the epithelial cell type responds best to surgery. Imaging tests like an MRI are done to make sure the cancer hasn’t spread beyond the diaphragm or to other distant organs. Tests to assess lung function are done to ensure the remaining lung will work well enough once the cancerous lung is removed. Heart tests like an echocardiogram are done to make sure the heart can sustain surgery and subsequent chemotherapy.
An EPP can make breathing easier and improve overall quality of life. And because it removes the majority of the cancer, it has the potential to improve a patient’s prognosis, especially if it’s combined with other treatments such as chemotherapy or radiation in what is known as multimodal therapy. In fact, studies indicate that pleural mesothelioma patients who undergo an EPP have a longer survival rate than patients who undergo the other surgical option, a pleurectomy/decortication. EPP has a 33 percent rate of cancer recurrence compared with 65 percent for P/D.
Because an EPP is a complicated procedure, it should only be performed by an experienced and qualified thoracic surgeon. The entire procedure takes three hours or longer.
First, the patient is given general anesthesia. Then the surgeon makes a nine- to 10-inch lateral incision along the patient’s chest cavity, either in the front or on the side. The surgeon may remove the sixth rib to gain better access to the cancerous lung and to create more space to work in. Then the lining of the chest and the lining of the heart are separated from the chest wall and the diaphragm, and any blood vessels supplying the cancerous lung are redirected.
Once the chest cavity is open, the surgeon inspects the area and removes all visible cancerous tissue, including the entire diseased lung, parts of the lining of the lung and heart, parts of the diaphragm, and possibly nearby lymph nodes.
The surgeon then reconstructs the lining of the chest, heart and the diaphragm using a medical grade mesh lining. In some cases, heated intrapleural chemotherapy is used after the removal of the lung and before reconstruction to target any leftover cancerous cells. Then the incision is closed with stitches.
Recovery after an EPP is extensive and may require a two-week hospital stay. Generally the patient is on a respirator for the first couple days or longer and is hooked up to drainage tubes to minimize fluid buildup. Complications with this surgery are not uncommon, so patients are monitored closely. A heart monitor will constantly evaluate the heart and blood tests will check for signs of infection, elevated liver enzymes and more. Nurses will encourage deep breathing and teach coughing techniques to prevent pneumonia. Physical therapists will help patients return to walking and using their arms.
Once the patient is discharged from the hospital, home recovery begins. For another six to eight weeks, the patient will be focused on recovery. This includes a lot of rest, as the body adjusts to using just one lung to breathe. At-home recovery may be difficult physically and emotionally. It is important to eat a healthy diet rich in protein and nutrients. Physical therapy and pulmonary rehabilitation therapy help patients to recover fully.
Many patients undergo radiation therapy, chemotherapy or both, five or more weeks after surgery. These treatments help to kill cancer cells left behind in surgery, which can delay cancer recurrence. Patients who qualify for EPP generally qualify for radiation and chemotherapy. Surgery alone doesn’t extend survival as much as multimodal therapy does. The combination of these treatments offers the greatest hope for longer survival.
For eligible candidates, an EPP can be an effective form of treatment. It can slow the cancer’s progression and increase a patient’s life expectancy, especially when combined with chemotherapy and radiation.
While about 40 percent of pleural mesothelioma patients survive up to one year after their diagnosis, EPP can help some patients reach the one-year mark and beyond. About 10 to 15 percent of mesothelioma cases qualify for aggressive surgery at the time of diagnosis.
In one study led by renowned mesothelioma specialist Dr. David Sugarbaker, 70 percent of patients who underwent EPP and other forms of treatment survived up to one year after diagnosis and 48 percent of patients survived at least two years after diagnosis.
Although EPP can be highly successful, it also comes with the risk of complications and even death. The surgery has a 3 percent to 8 percent mortality rate, with lower mortality rates at specialty cancer centers with experienced surgeons. Dr. Sugarbaker helped to keep Brigham and Womenʼs EPP mortality rate at 3.4 percent during his tenure at the hospital.
Other complications can affect the heart, such as an irregular heartbeat, cardiac hernia and cardiac arrest. Blood issues such as blood clots or internal bleeding may develop. Lung complications like respiratory failure, pulmonary embolism, pneumonia and hemothorax (blood collecting in the pleural space) can happen after surgery.
Anatomical changes may occur with the removal of a lung. The space where the lung once was may shrink, and hyperinflation of the remaining lung may develop. The connective tissue in the center of the chest that wraps the heart, esophagus and trachea may shift towards the empty space. Breathing complications are possible if the bronchus airways become obstructed, so be sure to report any progressive breathing issues to a doctor.
Breathing difficulties after a lung is removed will depend on the overall health and function of the remaining lung. For example, cigarette smokers generally have a more difficult post-surgery recovery than nonsmokers.
If the remaining lung is otherwise healthy, it will slowly expand to compensate for the missing lung. After recovery from surgery, regular and gentle movement helps the lung expand. Pulmonary rehabilitation may help as well.
At first, most people feel short of breath or may generally feel like they aren’t getting enough air. Breathing techniques and oxygen supplementation help people adjust as the remaining lung learns to compensate for the missing lung.
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