A new addition to the NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines™), the NCCN Guidelines for Malignant Pleural Mesothelioma (MPM), was presented at the NCCN 15th Annual Conference. Lee M. Krug, MD of Memorial Sloan-Kettering Cancer Center and a member of the NCCN Guidelines Panel discussed recommended treatment options for patients with MPM as well as first-line therapy regimens.
Mesothelioma is a rare form of cancer in which malignant cells are found in the mesothelium, a protective sac that covers most of the body's internal organs. Pleural mesothelioma refers to the thoracic-based form of the disease, the most common type of mesothelioma.
Although asbestos exposure is a major risk factor for mesothelioma, it can remain latent for more than 20 years and may require a predisposition to the disease. In addition, it can also occur in individuals that previously experienced radiation exposure (e.g. treatment for Hodgkin's lymphoma).
Dr. Krug noted that, "Diagnosing mesothelioma is often difficult, because the symptoms are similar to those of a number of other conditions. In addition, a physician's initial evaluation of a patient may reveal pleural effusion, but it can often be missed on pleural fluid cytology."
Common symptoms of mesothelioma are shortness or breath or chest pain among others including tumor fevers, sweats, weight loss, and pneumonia.
A surgical biopsy is often required to effectively diagnose a patient with mesothelioma according to Dr. Krug. PET scans can aid in staging as well, detecting unexpected metastases in 10 percent of cases.
Prognostic factors include gender, lymph node status, and histology, but Dr. Krug also pointed to other potential markers that may be indicative of the disease.
"Serum markers may also have prognostic significance as studies show that patients with mesothelioma have higher levels of Soluble Mesothelin-Relation Protein (SMRP) and Osteopontin," said Dr. Krug.
Like any cancer, treatment for mesothelioma depends on many factors including the stage of the cancer, where the cancer is, and how far the cancer has spread. Dr. Krug described treatments such as surgery, chemotherapy, and radiation that may benefit patients as described in the NCCN Guidelines for MPM.
Surgical procedures for MPM can range from a pleurectomy/decortication for those patients with early stage disease to more aggressive procedures, such as extrapleural pneumonectomy. Extrapleural pneumonectomy includes the removal of pleura, the lung, diaphragm, and pericardium, but can result in major complications and should only be performed by experienced surgeons.
"The role of aggressive surgery remains controversial," said Dr. Krug. "As outlined in the Principles for Surgical Resection for Malignant Pleural Mesothelioma in the NCCN Guidelines, physicians need to be highly selective when choosing potential candidates for this procedure."
Surgery alone can be inadequate due to residual disease and a high rate of relapse, so the NCCN Guidelines recommend a combined modality therapy approach for select patients noted Dr. Krug.
The NCCN Guidelines consist of a section detailing the Principles of Radiation Therapy for MPM that stress the need for a multimodality approach including evaluation of the patient by radiation oncologists, surgeons, medical oncologists, diagnostic imaging specialists, and pulmonologists.
"Radiation is recommended as an adjuvant therapy to improve local control after surgery, and it is also an effective palliative treatment for relief of chest pain that is often associated with mesothelioma," said Dr. Krug.
Dr. Krug explained that MPM was historically felt to be chemoresistant, but that recent studies have shown that certain regimens can benefit patients and also can be added for systemic therapy either before or after surgery.
"The NCCN Guidelines recommends pemetrexed (Alimta®, Lilly USA, LLC) with cisplatin (Platinol®, Bristol-Myers Squibb) or carboplatin (Paraplatin®, Bristol-Myers Squibb) as the optimal first-line combination chemotherapy regimen for patients, though others can be considered based on comorbid conditions," said Dr. Krug. "On the other hand, there are extremely limited data on the benefit of second line therapy. A few specific regimens are listed as second line options in the NCCN Guidelines."
The NCCN Guidelines are developed and updated through an evidence-based process with explicit review of the scientific evidence integrated with expert judgment by multidisciplinary panels of physicians from NCCN Member Institutions. The most recent version of this and all the NCCN Guidelines are available free of charge at http://www.NCCN.org.
Source: National Comprehensive Cancer Network
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