Extrapleural Pneumonectomy (EPP)

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Understanding the Extrapleural Pneumonectomy

Extrapleural pneumonectomy (EPP) is a thoracic surgery performed to treat pleural mesothelioma, a rare cancer that affects the lining of the lungs (pleura). EPP is considered a radical surgery but is one of the most effective ways to extend the life expectancy of patients with pleural mesothelioma.

The EPP was developed by the late Dr. David Sugarbaker, who modified existing lung-removal techniques so they would be more effective for mesothelioma patients. Dr. Sugarbaker was one of the first surgeons to make a significant breakthrough in mesothelioma treatment, and his EPP surgery remains a standard in the treatment of malignant pleural mesothelioma.

Unlike the pleural mesothelioma surgery pleurectomy with decortication, the EPP procedure involves removing the diseased lung. In some cases, the pleural lining of the other lung, pericardium (lining of the heart), and part of the diaphragm may be removed as well. Numerous studies have proven the treatment option extends overall survival time.

Most patients who receive an EPP have early-stage pleural mesothelioma before cancer has spread to distant lymph nodes and other parts of the body. The ideal patients are those who are healthy enough to recover from the surgery. Losing a lung is an intensive experience that requires a lengthy recovery period, and preoperative heart and lung tests will help confirm whether a person can handle the physical toll.

In some cases, late-stage mesothelioma patients may be able to receive an EPP as well. It’s ultimately up to the doctor to determine whether any given individual has the overall health needed to undergo this surgical treatment.

Patients can receive EPP surgery from specialized mesothelioma doctors at cancer centers across the country.

Veterans may receive EPP surgery and other mesothelioma treatment at VA hospitals if they are enrolled in the VA Health Care System. The VA has several doctors, including Dr. Avi Lebenthal, who specialize in treating mesothelioma and have extensive experience working with veterans.

Veterans with mesothelioma can receive other benefits through the VA as well.

EPP Eligibility

An EPP is an aggressive procedure, and there is a strict set of criteria for patients who can receive the surgery. Doctors prefer to operate on patients with stage 1 or 2 pleural mesothelioma and will consider a patient’s overall health outside of their cancer.

Doctors will consider whether a patient can perform basic, everyday tasks and take care of their needs. Patients who are mobile and functional are significantly more resilient to surgery. Tests are also performed on the heart and lungs to determine whether their function can support surgery.

Some tests your doctor may run before EPP include:

• PET or CT scan: These kinds of imaging scans help doctors see where your disease is. It’s important to know the exact location of the tumors before surgery. These scans can also reveal if your mesothelioma is too advanced for surgery.
• Mediastinoscopy: This procedure uses a camera to look in the center of the chest. Doctors want to see if mesothelioma has spread to the other side of the chest, which is inoperable.
• Lung function tests: Your lungs have to be in working order to recover. These tests can be as simple as breathing through a tube. Another test uses radioactive gas, where the patient inhales a small amount and helps doctors see the active airways in the lung.
• Echocardiogram: This 30-minute procedure uses sound waves to measure your heart. A machine measures how strong your heart is. A strong heart is necessary for any type of surgery.
• Blood tests: Doctors need to know your blood chemistry before an EPP. They measure your red and white blood cell counts.
• Performance tests: These are more tests to determine your lung and heart health. Performance tests measure how long you can exercise and how your organs hold up in the process. You may walk on a treadmill or pedal a stationary bike while you wear a mask that measures your breathing. Other devices measure your heart rate and blood pressure.

Each surgeon or cancer center may have its own set of criteria for patient selection. For example, one cancer center may have age restrictions, another center may care more about your overall health. If you’ve been deemed inoperable due to age, you may want a second opinion.

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The EPP Surgery

Before receiving an EPP, patients need to prepare. Mesothelioma specialists will create a clear plan with prerequisite activities that patients will need to follow before surgery. Patients should take the time to understand and follow every step in preparation. Shortly before surgery, a pre-admission check-up will be performed to confirm a patient’s complete health profile.

In some cases, doctors may administer chemotherapy to shrink tumors before surgery. Immediately before surgery, a patient will receive an intravenous catheter that will deliver general anesthesia, fluids, and medications. Once you’re asleep, a doctor will use a bronchoscope to look into your lungs and confirm surgery is possible.

An EPP can be performed in as little as three hours, although some surgeons take longer and the time increases if the mesothelioma is difficult to resect (remove).

The EPP surgery itself has several steps:

1. Step 1: During the surgery, a large incision is made with immediate removal of the sixth rib. This incision opens up the surgical space and allows doctors to see where the mesothelioma is.
2. Step 2: Doctors separate the pleural and pericardial linings from the chest wall.
3. Step 3: Doctors collapse the lung. They clamp off major blood vessels, nerves, and the bronchial tube leading to the affected lung.
4. Step 4: Doctors remove cancerous tissue. They take out the pleural and pericardial linings. They also remove the lung and part of the diaphragm. They patch up these areas with artificial materials.
5. Step 5: Doctors inspect the lung cavity for signs of cancer. If they see no more, they close the incision with sutures. A tube is placed in the chest cavity to allow fluid and blood buildup to drain out. The drain is removed when it no longer produces fluid.

After surgery, patients need to take care of themselves, physically and mentally. Poor appetite, short-term depression, constipation, and pain are all common symptoms after EPP surgery.

The hospital will provide tips for coping with each of these symptoms, but patients should speak up if they have recovery concerns at any point.

EPP and Multimodal Treatment

An EPP is most effective when it's combined with other mesothelioma treatments. When doctors use two or more treatments together it’s called multimodality therapy. This approach has become a common practice in mesothelioma care.

Traditionally, chemotherapy and radiation therapy are the two standard treatments used with EPP. This trimodality therapy has proven to extend patient survival for months or even years.

The late Dr. Sugarbaker’s research has also led to new ways of incorporating chemotherapy in the EPP, including intraoperative chemotherapy.

With intraoperative chemotherapy, a chemotherapy solution is applied to the surgical site immediately following EPP surgery. The goal is to destroy any microscopic mesothelioma cells that remain after the visible tumors and lining are removed.

Thoracic surgeon Dr. Avi Lebenthal at the Boston VA is a pleural mesothelioma specialist who performs EPP surgery for veterans. If you have VA benefits but don’t live near Boston, it’s not a problem.

According to the VA, "We are proud to welcome and treat all eligible veterans regardless of where they live."

Dr. Lebenthal treats his mesothelioma veterans at the nearby Brigham and Women’s Hospital, which has a mesothelioma program developed by the late Dr. Sugarbaker and is one of the best in the world.

Extrapleural Pneumonectomy Recovery Times

Patients who undergo EPP take several weeks to several months to recover.

Patients are typically in the hospital for the first 7-10 days of their recovery. After this hospital stay, patients may be cleared to continue their recovery from the comfort of home.

Patients must take their at-home care seriously, as it is an integral part of the recovery process. Patients are asked to shower daily, keep their incision clean, and support their recovery with nutritious meals and beverages. Pain should be expected, especially as activity levels increase over time, and patients will be prescribed medication for pain management.

Doctors will advise patients when they can return to each of their previous activities.
Typically, EPP patients are unable to work or lift more than 10 pounds for a minimum of 4-6 weeks. Patients also shouldn’t drive until they stop taking pain medication.

Additional time will be required for a patient to adjust to life with only one lung. Patients should not swim or participate in sports for at least 2 months and will need to ease back into those activities when they are finally ready.

In some cases, EPP surgery leads to immediate or long-term complications and risks, which can lengthen recovery time. It’s important that EPP patients are prepared to take the additional time to recover, if necessary, to prevent any potential EPP risks.

EPP Risks

All surgeries have risks, and an EPP is no exception. Because EPP is an invasive and intensive surgical procedure, there is a risk of serious complications during or after the surgery.

Some of these complications include:

• Respiratory failure
• Empyema (a buildup of infected fluid within the pleural space)
• Internal bleeding
• Blood clot or deep vein thrombosis (DVT) in the lower legs which can travel to the lungs
• Pneumonia
• Death due to complications of surgery

According to the American Cancer Society, 1 in 3 patients who undergo surgery may have major complications, including death. However, 87.5 to 96.8% of patients survive more than 30 days after EPP surgery.

Surgical complications can appear during surgery, while recovering in the hospital, or during at-home recovery. For example, a person may bleed out during their operation, experience a blood clot during post-op recovery, or contract pneumonia at home.

Patients should discuss normal and abnormal symptoms with their surgeon and know what to do if an unexpected symptom appears.

EPP Benefits

Despite the risks, an EPP is one of the best treatments available for patients with pleural mesothelioma. Some have even called it the "gold standard" of treatment. EPPs provide patients with their best chance at increasing their lifespan.

Many pleural mesothelioma patients also experience relief from their existing symptoms. An EPP removes the diseased lung and all visible tumors, which can also minimize or eliminate symptoms that the cancer was causing.

Patients who receive EPP surgery eliminate most or all of the cancer cells in their body, which slows disease progression. As a result, pleural mesothelioma patients who have EPP live longer with an improved quality of life.
EPP Survival Rates

The average survival rate after an EPP is 7-47 months, but many patients exceed these statistics. It isn’t unheard of for patients to live 5 or more years after EPP surgery.

Patients with pleural mesothelioma have their best chance of long-term survival when they use the trimodal approach and combine EPP with chemotherapy and radiation.

In one of Dr. Sugarbaker’s studies, patients who received intraoperative chemotherapy lived over 1 year longer than patients who didn’t receive the treatment. The median survival for the patients who had intraoperative chemotherapy was 35.6 months, with half of those patients living even longer.

Cancer researchers continue to make breakthroughs regularly. It is possible that patients with pleural mesothelioma who receive EPP today will have access to treatments in the future that will extend their lifespan even further.

Getting an EPP

Patients can undergo EPP performed by specialized mesothelioma doctors at cancer centers across the country. These specialized doctors are highly experienced in mesothelioma cases and are leading mesothelioma researchers.

It’s essential for patients to receive specialized treatment for their mesothelioma. Mesothelioma is a rare and unusual form of cancer, and general oncologists may not know how to diagnose or treat it. Misdiagnosis is common with mesothelioma and has devastating consequences.

Mesothelioma cancer teams can help guide patients through every step of the preparation, treatment, and recovery process. These teams are comprised of people who are highly familiar with mesothelioma and can provide the highest quality care possible.

Anyone with pleural mesothelioma can access civilian cancer centers in the United States. However, there are also special programs made specifically for veterans that can only be accessed through VA hospitals.

EPP and the VA Health Care System

Veterans have options and can receive an EPP through private cancer centers or the VA Health Care System. In many cases, the VA system is an ideal choice for patients because it streamlines processes and can connect patients to other helpful resources.

Dr. Avi Lebenthal is a pleural mesothelioma specialist affiliated with the VA who can perform the EPP surgery and provide other treatments. Dr. Lebenthal is a veteran of the Israeli Army, and he currently treats mesothelioma patients at the Boston VA Hospital.

Veterans can get treatment from Dr. Lebenthal’s team by applying for VA Health Care and other benefits.

Learn More About Getting an EPP

The Mesothelioma Veterans Center can help patients learn more about the EPP procedure and other medical treatments to improve their lifespan. We help patients assess their options and develop a plan of action.