Mesothelioma Extrapleural Pneumonectomy (EPP)

Extrapleural pneumonectomy (EPP) is a surgery for pleural mesothelioma. It removes the affected lung, the entire pleura and any surrounding areas affected by malignant tumors.

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Written by Karen Ritter, RN BSN

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Important Facts About Mesothelioma Extrapleural Pneumonectomy

  • Extrapleural pneumonectomy is an invasive surgery for mesothelioma. Surgeons remove the patient’s lung during the operation. They also remove part or all of the pleura, pericardium and diaphragm.
  • The surgery has two parts. The first is removing the lung and visceral pleura (pneumonectomy). The second is removing the parietal pleura, pericardium and hemidiaphragm.
  • Doctors usually pair EPP with at least one other treatment for mesothelioma, such as chemotherapy or radiation. These therapies can be used before, during or after the operation.
  • EPP usually leads to survival (post operation) of 1‑2 years. Patients with epithelioid mesothelioma have longer survival rates. Recently reported mortality rates for EPP are between 4% and 7%.

How to Get an EPP for Mesothelioma

People with mesothelioma may be a candidate for extrapleural pneumonectomy. Follow these steps if you want to know how to get an EPP for mesothelioma:

1

Get an official diagnosis from a thoracic oncologist. These doctors specialize in thoracic cancers like pleural mesothelioma.

2

Contact a patient advocate from a mesothelioma support organization. These experts can tell you more about EPP, plus other surgeries and therapies for mesothelioma.

3

Meet with a mesothelioma specialist. They’ll provide a second opinion regarding your diagnosis and treatment options. Many are surgeons who can perform EPP, and they’ll tell you whether your cancer is operable.

Overview of Mesothelioma Extrapleural Pneumonectomy

Mesothelioma extrapleural pneumonectomy is an aggressive surgery intended to remove mesothelioma tumors. It is often shortened to the acronym EPP.

Surgeons resect four body parts during EPP:

1

Affected lung

2

Pleura (lining between the lungs and chest)

3

Part or all of the diaphragm (muscle below the lungs)

4

Part or all of the pericardium (lining of the heart)

They also may remove lymph nodes if tumors have spread far enough.

Where Was EPP for Mesothelioma Developed?

EPP stems from pneumonectomy surgery, which is used for various types of diseases. Dr. Evarts Graham performed the first pneumonectomy on April 5, 1933, at Barnes Hospital in St. Louis, Missouri. The patient had carcinoma of the lung.

The first EPP was performed in 1949 to treat tuberculosis. In the latter half of the 20th century, the surgery was used almost exclusively for malignant mesothelioma.

In the 1970s, Dr. Eric Butchart and others published the oldest‑known study on survival and mortality rates for mesothelioma extrapleural pneumonectomy. Around the same time, he created the first staging system for mesothelioma.

Mesothelioma Pneumonectomy

A pneumonectomy is removing part or all of your lung. For pleural mesothelioma, this procedure removes the affected lung, which is the lung on the same side as the tumors. These tumors form in the pleural space and spread beyond the pleura, usually invading the lungs.

By removing the lung, surgeons also remove the visceral pleura. This cell lining is attached to the lung.

What Does Extrapleural Mean?

Extrapleural is a medical term for the tissue outside of the parietal pleura. The parietal pleura is the outer lining of the pleura attached to the chest wall.

The “extrapleural” part of EPP is removing the:

  • Parietal pleura
  • Pericardium (mesothelial lining around the heart)
  • Diaphragm 

What Is the Pleura?

Pleural mesothelioma forms in the pleura, which is a thin lining composed of mesothelial tissue. The lining offers a buffer as the lungs expand and contract against the chest wall.

The pleura is made up of two mesothelial cell linings: the parietal pleura and visceral pleura. The parietal pleura is the outer lining, meaning it’s attached to the chest wall. It is also near the diaphragm and pericardium. The visceral pleura is the inner lining, next to the lung.

Tumors on the parietal pleura can easily spread to the diaphragm and pericardium. Tumors on the visceral pleura can spread to the nearby lung. This is why they are often removed during surgery.

Difference Between EPP and P/D for Mesothelioma

EPP was the first surgery designed for mesothelioma. Pleurectomy with decortication (P/D) is a newer technique, and both surgeries are what doctors rely on to treat pleural mesothelioma.

The main contrast between EPP and P/D is the removal of the lung. EPP for mesothelioma removes the lung while P/D does not. The latter focuses on removing the pleura, keeping the lung intact. An extended version of P/D removes parts of the diaphragm and pericardium.

The lungs are vital to the respiratory system working properly. Removing one lung is a consequential decision, so patient selection is crucial for EPP.

Doctors usually evaluate patients’ overall health, medical history and age before performing this surgery. P/D, by comparison, doesn’t have as rigorous a patient‑selection process.

Multimodal and Intraoperative Treatments With Mesothelioma Extrapleural Pneumonectomy

Multimodal treatment is a combination of different therapies to fight cancer in multiple ways. Surgery, chemotherapy and radiation as a trio is the most common type of multimodal therapy for mesothelioma.

EPP works best with intravenous chemotherapy and external beam radiation. For decades, doctors used these methods of treatment after surgery to kill any remaining cancer cells

In recent years, doctors have experimented with radiation before EPP. This is called “SMART” (surgery for mesothelioma after radiation therapy).

Doctors may also use intraoperative therapies with EPP. These therapies occur during the surgery and can decrease chances of recurrence.

The two intraoperative therapies combined with EPP are:

  • Hyperthermic intraoperative chemotherapy (HIOC), also called heated intrathoracic chemotherapy (HITHOC)
  • Intraoperative radiation therapy (IORT)

What Happens During an Extrapleural Pneumonectomy for Mesothelioma

Prior to incision, the patient will be sedated with anesthesia, intubated (breathing tube placed), and then properly positioned for surgery.

8 Steps for Mesothelioma Extrapleural Pneumonectomy

According to the MedStar Washington Hospital Center website, EPP for pleural mesothelioma takes at least three hours to complete:

1

Incision — The surgeon makes an incision, approximately 9‑10 inches long, along the side of the patient’s body. This is called a thoracotomy. The surgeon may remove the sixth rib to better access the lung cavity.

2

Chest cavity inspection — The surgical team inspects the chest cavity for visible mesothelioma tumors.

3

Pneumonectomy — The surgeon removes the diseased lung from the body. Along with removing the lung, the surgeon also removes the visceral pleura lining attached to the lung.

4

Diaphragm and pericardial resection — The surgeon looks for visible tumors within the chest cavity that can be resected. They’ll also remove the parietal pleura and either part or all of the diaphragm and pericardium to prevent spread.

5

Lymph node removal — If mesothelioma has spread to the lymph nodes in the chest, doctors may remove them.

6

Possible additional therapies — Doctors may apply intraoperative chemotherapy or intraoperative radiation therapy.

7

Reconstruction — Doctors may use Gore Tex or another substance to reconstruct the removed parts.

8

Cavity examination — Doctors examine the cavity for any potential complications from the surgery. They insert a chest tube to help with breathing by draining fluid from the area where the lung was removed.

Recovery Time After Mesothelioma EPP

Recovery from mesothelioma EPP takes around two months: two weeks in the hospital and 6‑8 weeks after discharge. However, patients may experience respiratory challenges for long after their surgery.

Inpatient EPP Recovery

Following surgery, most patients go to the intensive care unit (ICU) for inpatient recovery. According to the University of California San Francisco Department of Surgery website, inpatient recovery after EPP is around two weeks.

Outpatient EPP Recovery

Outpatient recovery can occur at the patient’s home, a rehabilitation facility or a long‑term care facility (nursing home or assisted living center). Patients usually need 6‑8 weeks of outpatient recovery after EPP.

Who Can Get EPP for Their Mesothelioma?

Doctors look for specific factors as a guideline for who is eligible for mesothelioma EPP. These include: stage of the cancer; overall health; and cell type. However, there’s no definitive set of rules for who can get this surgery.

Anyone told they can’t undergo surgery should look into a second opinion. General cancer surgeons or thoracic oncologists might not have experience performing EPP for mesothelioma. We recommend finding a specialist to talk with about your diagnosis.

EPP Eligibility: Stage

Stage is important for determining who should get EPP for mesothelioma. Many doctors feel a stage 2 or stage 3 disease is best treated with this operation, while stage 1 or early stage 2 is better treated with P/D.

The reasoning is stage 3 mesothelioma likely has invaded the lung, which isn’t salvageable. In earlier stages, tumors haven’t yet reached the lung and the organ can remain in the body.

EPP Eligibility: Physical Health

Physical health is essential to determining if a patient should undergo EPP for mesothelioma. The patient’s respiratory function and health should determine if they can endure a lung‑resection surgery.

Doctors will have patients go through a preoperative patient evaluation. These tests will help doctors analyze whether this aggressive surgery is the best option.

Preoperative Patient Evaluation for Mesothelioma EPP

  • Age and fitness evaluation
  • Blood tests
  • Pulmonary function evaluation
  • Ventilation/perfusion scan
  • CT scan, MRI of the chest and PET scan
  • Echocardiogram
  • Stress test

EPP Eligibility: Cell Type

Cell type is a significant factor for whether mesothelioma EPP is the best treatment option. Epithelioid mesothelioma is the easiest cell type to treat because the tumors don’t spread as quickly. They’re also easier to identify from scans.

Sarcomatoid mesothelioma is the toughest to treat and has high rates of recurrence. Many doctors won’t recommend EPP for this cell type. Biphasic mesothelioma, a mixture of epithelioid and sarcomatoid cells, is determined on a case‑by‑case basis.

The cell type often affects the survival time following EPP. Epithelioid patients usually live for much longer after the surgery than sarcomatoid patients.

Life Expectancy and Survival Rates for EPP

Survival rates have steadily improved for extrapleural pneumonectomy, although some doctors prefer P/D. EPP is considered more aggressive and less tolerable.

A study in the Journal of Thoracic Oncology reported average survival after mesothelioma EPP of around 18.4 months. Most EPP patients in the study had a stage 3 diagnosis, associated with a median survival of 16 months.

Survival After EPP With Multimodal Treatment

Doctors usually pair EPP for mesothelioma with one or multiple other therapies. Chemotherapy and radiation are the safest options with the most data for reference.

Multimodal treatment refers to using multiple treatments for a disease. Surgery is the primary option, while chemotherapy and radiation are secondary methods.

Doctors may use chemotherapy or radiation before or after mesothelioma EPP. Using them after is called “adjuvant therapy” and using them before is “neoadjuvant therapy.”

Multimodal treatment enhances the survival rates for mesothelioma extrapleural pneumonectomy:

  • EPP with neoadjuvant and adjuvant therapies — 18 months median survival, 41% two‑year survival and 14% five‑year survival
  • EPP with adjuvant chemotherapy and radiation — 18 months median survival, 39% two‑year survival and 14% five‑year survival
  • EPP with adjuvant radiation therapy — 14.7 months median survival and 32% two‑year survival

EPP With SMART for Mesothelioma

SMART for mesothelioma is a new protocol using radiation therapy before surgery. The concept originated at Princess Margaret Cancer Centre in Toronto, Canada.

Doctors began SMART with EPP as the preferred surgery. They used radiation to control and shrink tumors days before the operation.

Initial studies showed 3‑year survival rates of 72%. Epithelioid patients saw the best results: median survival of 65 months.

“I went back to double‑check the numbers because I couldn’t believe it at first,” said Dr. John Cho, a specialist at the Toronto hospital. “I thought we made a mistake.”

Survival After EPP With Intraoperative Therapies

Doctors found success using intraoperative chemotherapy with peritoneal mesothelioma surgery. The results led experts to consider intraoperative therapies for pleural mesothelioma.

The two options are heated intraoperative chemotherapy and intraoperative radiation therapy. Thus far, only heated chemotherapy into the chest cavity has shown to be safe and effective.

EPP With HITHOC for Mesothelioma

The combination of EPP and HITHOC is an attempt to completely cleanse the body of mesothelioma. EPP removes most of the tumors, and heated chemotherapy washes the chest cavity in an attempt to kill any remaining cancer cells.

Studies prove that EPP works with HITHOC. Dr. David Sugarbaker’s study in 2013 had a median survival of 35 months after EPP and HITHOC.

EPP With IORT for Mesothelioma

There is little data for EPP with intraoperative radiation therapy. A study co‑authored by mesothelioma specialist Dr. Valerie Rusch noted severe complications for half of patients from EPP with IORT. However, there were only six patients in the group, a small sample size.

Possible Extrapleural Pneumonectomy Complications

Surgical removal of a vital organ is a serious medical decision. Since mesothelioma EPP removes the affected lung, patients may experience breathing issues for months or years after their surgery. Since EPP can affect quality of life, the patient’s overall health is a key element to selection.

EPP is a complex operation that can cause various complications:

  • Atrial fibrillation (rapid heart rate)
  • Blood clots
  • Infection in the chest cavity (empyema)
  • Pneumonia
  • Hemorrhage or bleeding in the chest cavity
  • Respiratory failure

Mesothelioma Recurrence After EPP

Recurrence is the return of cancer. After a specified amount of time with no sign of disease or cancerous activity, doctors will notice signs of cancerous activity on follow‑up scans. Doctors request patients undergo regular follow‑up scans to check for recurrence. Mesothelioma has high recurrence rates due to the disease’s characteristics.

Mesothelioma consists of small microscopic tumors that spread like an avalanche from the mesothelial lining to other nearby areas. This is a contrast from most cancers, which consist of one large tumor mass growing in radius.

The intent of mesothelioma EPP is to remove all tumors. However, even after resecting the lung, some standalone tumors may be hidden within the chest cavity.

Using EPP with SMART led to a 63% recurrence rate within five years of the surgery. Dr. Raja Flores, a highly respected mesothelioma surgeon, reported a 57% recurrence rate after EPP. This data was published in 2020.

Mortality From Mesothelioma EPP

One of the concerns with mesothelioma EPP is the surgery’s mortality (death) rate. It’s higher than the mortality rate for P/D, which is a reason doctors prefer lung‑sparing surgery over lung resection.

In two studies, one reported in 2013 and the other in 2014, the 30‑day mortality rates were 11% and 12%. A 2016 study reported a lower mortality rate (7%), but it was still triple that of P/D for mesothelioma.

Dr. Flores reported in 2020 a 4.5% 30‑day mortality for EPP. Again, the rate is nearly triple that of P/D.

Doctors Who Perform EPP for Pleural Mesothelioma

  • Dr. Raja Flores, Mount Sinai Medical Center (New York)
  • Dr. Taylor Ripley, Baylor St. Luke’s Medical Center (Houston, Texas)
  • Dr. Jacques Fontaine, Moffitt Cancer Center (Tampa, Florida)
  • Dr. Hassan Khalil, Brigham and Women’s Hospital (Boston, Massachusetts)
  • Dr. Elliot Wakeam, Michigan Medicine (Ann Arbor)
  • Dr. Boris Sepesi, MD Anderson Cancer Center (Houston, Texas)

Common Questions About Extrapleural Pneumonectomy for Mesothelioma

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What happens during an extrapleural pneumonectomy?

An extrapleural pneumonectomy involves removing your lung, pleura (thin lining covering your lungs), diaphragm, pericardium (thin lining encompassing your heart) and diseased lymph nodes. A pneumonectomy is a surgical procedure to remove a lung, but mesothelioma often spreads to other areas needing to be removed as well.

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What is the survival for mesothelioma extrapleural pneumonectomy?

In one study, more than 60% of mesothelioma patients survived for one year after an extrapleural pneumonectomy. Around one‑fourth survived for three years. The extreme nature of removing your lung, diaphragm, lymph nodes and pleura decreases the likelihood of a recurrence.

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What are the risks of having an extrapleural pneumonectomy?

Lung resection is a challenge for patients, especially those in older age or poor health. Not all patients enjoy a good quality of life with just one lung. They may experience breathing issues, pneumonia or other pulmonary complications. Other risks of having an extrapleural pneumonectomy include:

  • Infection
  • Internal bleeding
  • Blood clots
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How long does an extrapleural pneumonectomy take?

Extrapleural pneumonectomy for mesothelioma lasts approximately two hours. There is little variance in the time the surgery takes, as most doctors remove the same organs and body areas.

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What is the recovery time for an extrapleural pneumonectomy?

Patients usually stay in the hospital for two weeks after an extrapleural pneumonectomy. Recovery continues for 6‑8 weeks at home. The overall recovery time from mesothelioma EPP is two months, but some patients may need physical therapy to rehab their respiratory system.

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How much does an extrapleural pneumonectomy for mesothelioma cost?

An extrapleural pneumonectomy for mesothelioma costs a median of $62,400, according to a report in Rare Tumors. The range is $48,000‑$76,000. Health insurance often covers most of the costs after the plan’s deductible.

Sources & Author

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About the Writer, Karen Ritter, RN BSN

Karen Ritter is a registered nurse and patient advocate for Mesothelioma Guide. She leads patients and families through their mesothelioma diagnosis, explaining treatments and support options. Karen joined Mesothelioma Guide in 2021.