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Mesothelioma Extrapleural Pneumonectomy (EPP)

Extrapleural pneumonectomy is a surgical treatment for malignant mesothelioma. It involves the removal of a lung, a portion of the diaphragm, the linings of the lungs and heart (parietal pleura and pericardium).

Extrapleural pneumonectomy (EPP) is a common surgical option for eligible pleural mesothelioma patients. Typically, the surgery is included as part of a multimodal mesothelioma treatment plan with chemotherapy and radiation, and is often performed on patients with early-stage disease to remove as much of the cancer as possible and extend life expectancy. Because the surgery is so invasive, it is only a viable treatment option for select patients, like those with early stage mesothelioma and better overall health.

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How Extrapleural Pneumonectomy Treats Mesothelioma

Extrapleural pneumonectomy is a form of macroscopic complete resection (MCR). The goal of any MCR procedure is to completely remove all signs of cancer that are visible to the naked eye. To that end, the EPP procedure removes the entirety of a pleural mesothelioma patient’s diseased lung, the pleural lining of the heart and chest, parts or all of the diaphragm and any visible cancer.

Traditionally, the surgery is completed at large cancer centers by mesothelioma specialists with considerable experience performing the procedure. The surgery is performed under general anesthesia and includes a large incision into the patient’s chest cavity. A surgeon may opt to perform the surgery from the front, known as a sternotomy, or from the patient's side, known as a thoracotomy.

To achieve optimal view of the area and ease lung removal, the sixth rib may also be removed during the procedure. After collapsing the diseased lung and clamping blood vessels, the surgeon will remove as much cancerous tissue as possible. A two-week hospital stay may be necessary in order for the patient to recover under the care of medical professionals following surgery, since it is such an invasive procedure.

Though the surgery is intensive and takes longer to recover from than other treatment options, studies show an EPP can improve quality of life and ease breathing difficulties for pleural mesothelioma patients. However, treating the disease via surgery alone has only shown moderate success. EPP is typically part of multimodal treatment approaches, meaning it is combined with other therapies including chemotherapy and radiation. When applied multimodally, studies have shown that pleural mesothelioma patients who underwent intensity-modulated radiation therapy (IMRT) prior to EPP achieved median survival of 51 months, with 47 months disease-free. The reverse order, EPP and then IMRT, has also shown to have favorable results. In a 2018 study, pleural mesothelioma patients treated first with EPP and then radiation experienced a median relapse-free survival of more than 24 months, with a median overall survival of over 38 months. The average life expectancy for pleural mesothelioma patients is six months to one year.

Eligibility for Mesothelioma Extrapleural Pneumonectomy

An extrapleural pneumonectomy is a radical surgery and is not suitable for all mesothelioma patients. Typically, only patients diagnosed in the early stages of disease are viable candidates for surgical mesothelioma treatment. Research has shown that the malignant pleural mesothelioma patients that most benefit from adding an EPP to their multimodality therapy include those that are female, have epithelial tumors and early stage disease with no nodal involvement.

Imaging tests, such as a CT scan and PET/CT scan, are often completed first to help determine the best course of treatment for pleural mesothelioma. These scans enable a patient's medical team to see the extent of disease and if metastasis has occurred. Patients whose disease has not metastasized may be eligible for EPP or other surgical options. Doctors will also ensure that patients have adequate heart and lung function prior to an extrapleural pneumonectomy, as complete removal of one lung puts more strain on the remaining lung and the heart muscle.

Success following EPP is also impacted by whether the diseased lung sits on the right or left side of the chest. Studies show that EPP is better tolerated when performed on the left lung compared to the right. A study including more than 60 pleural mesothelioma patients found that 54% of patients who received an EPP on the right side had major complications, including bronchopleural fistula (a passageway that develops between the bronchi and pleural cavity), irregular heartbeat and cardiac herniation. Comparatively, only 21% of those that underwent an EPP on the left side experienced such complications.

For those with pleural mesothelioma impacting the right side of the body, a pleurectomy/decortication (P/D) may be a safer option. Researchers have noted the two surgeries generally have negligible differences in overall patient survival. According to a 2017 report by the National Cancer Database, patients who received EPP had median overall survival of 19 months, compared to a 16 month median overall survival in those who underwent P/D.

Side Effects and Risks of Extrapleural Pneumonectomy

After undergoing an EPP, patients may stay in the hospital for at least two weeks for recovery. However, it may take between six and eight weeks for a patient to fully recover from the surgical procedure because it takes time for a patient’s remaining lung to be able to complete necessary respiratory functions. As with any serious thoracic surgery, there may be side effects that develop at any time during recovery from EPP. Some risks are short-lived, beginning immediately after surgery completion and may diminish over time. Others are more long-lasting and serious complications, such as internal bleeding.

Risks Associated with Extrapleural Pneumonectomy
Common Side Effects
  • Breathing difficulty
  • Fatigue
  • Blood clots
Serious Complications
  • Atrial fibrillation (irregular heartbeat)
  • Empyema (pus in the pleural cavity)
  • Pneumonia
  • Postoperative hemorrhage (internal bleeding)
  • Respiratory failure

For all mesothelioma patients, regardless of treatment, there is also a high risk of recurrence, including for those treated with an extrapleural pneumonectomy. A retrospective study compared the local and distant recurrence rates for EPP and P/D surgeries. Of more than 600 mesothelioma patients, researchers found P/D has a distant recurrence rate of about 35%, compared to 66% recurrence rate after EPP. However, EPP has lower rates of local mesothelioma recurrence. Patients with EPP generally have a 33% chance of local recurrence compared to the 65% chance of recurring mesothelioma cancer in patients who’ve undergone P/D.

A study examining recurrence in 59 mesothelioma patients who underwent EPP found median time to recurrence was 11.6 months. Researchers found the patients that experienced recurrent mesothelioma following EPP could extend survival after treating the disease with additional chemotherapy. These 21 patients achieved median survival of 39.2 months. The 18 patients with recurrent cancer that did not treat the disease with chemotherapy had a median survival time of just 12.2 months.

While EPP was the first surgical mesothelioma treatment option, there have been questions raised by surgeons and researchers regarding the benefits of EPP versus P/D for mesothelioma treatment. The efficacy of EPP has been called into question due to varying mortality rates reported following the surgical procedure. Between 1985 and 2010, studies reported mortality as low as 0 and as high as 11.8%. However, when performed by knowledgeable mesothelioma thoracic surgeons on the appropriate patients after thorough patient selection, the procedure has been able to extend life expectancies and improve overall quality of life for pleural mesothelioma patients. When considering mesothelioma treatment options, patients should understand the risks and benefits of all viable therapies to make the best treatment decision for their individual case.

Author: Linda Molinari

Editor in Chief, Mesothelioma Cancer Alliance

Linda Molinari

Reviewer: Dr. James Stevenson

Medical Reviewer and Thoracic Medical Oncologist

Dr. James Stevenson
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Sources

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Jhavar S, Pruszynski J, et al. Intensity modulated radiation therapy after extra-pleural pneumonectomy for malignant pleural mesothelioma is feasible without fatal pulmonary toxicity and provides good survival. Asia-Pacific Journal of Clinical Oncology. April 2018;14(2):e88-e94. doi: 10.1111/ajco.12680

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Takuwa T, Hashimoto M, et al. Post-recurrence chemotherapy for mesothelioma patients undergoing extrapleural pneumonectomy. International Journal of Clinical Oncology. October 2017;22(5):857-864. doi: 10.1007/s10147-017-1126-x

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