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Diagnosis and Prognostic Factors
Prognosis in malignant mesothelioma is difficult to assess consistently because there is great variability in the time before diagnosis and the rate of disease progression. In large retrospective series of pleural mesothelioma patients, the following were found to be important prognostic factors:[
Prognostic scoring systems
Two prognostic scoring systems have been developed for advanced unresectable mesothelioma and are used to stratify patients enrolling in clinical trials: the Cancer and Leukemia Group B (CALGB) index and the European Organisation for the Research and Treatment of Cancer (EORTC) index.
CALGB index
The CALGB index was developed retrospectively using the clinical characteristics of 337 patients treated on clinical trials of chemotherapy for advanced mesothelioma during a 10-year period.[
The prognostic value of the CALGB index was evaluated retrospectively in a phase II clinical trial of 105 patients.[
EORTC index
The EORTC index was also developed retrospectively using the characteristics of 181 patients from five phase II clinical trials of chemotherapy during a 9-year period.[
Patients were allocated a numerical prognostic score based on each of these variables (+0.55 if WBC >8.3 × 109 /L, +0.60 if ECOG PS ≥1, +0.52 if unconfirmed histology, and +0.60 if male gender). Subsequently, patients were classified into two prognostic groups that included low-risk patients with a prognostic score of 1.27 or lower (0–2 risk factors) and high-risk patients with a prognostic score higher than 1.27 (3–5 risk factors). High-risk patients had a relative risk of death of 2.9 compared with low-risk patients, P < .001; the 1-year survival rate was 40% for the low-risk group compared with 12% for the high-risk group.
Follow-up and Survivorship
Multimodality therapy incorporating radical surgery (extrapulmonary pneumonectomy or radical pleurectomy with decortication) with or without chemotherapy, administered with or without radiation, may be considered for patients with limited disease and has been associated with a relatively long survival in observational series.[
For patients treated with aggressive surgical approaches, nodal status is an important prognostic factor.[
Carcinogenesis
A history of asbestos exposure is reported in about 70% to 80% of all cases of mesothelioma.[
References:
Histologically, these tumors are composed of spindle cells (sarcomatoid) or epithelial elements, or both (biphasic). Desmoplastic mesothelioma, consisting of bland tumor cells between dense bands of stroma, is a subtype of sarcomatoid mesothelioma. The epithelioid form is occasionally confused with lung adenocarcinoma or metastatic carcinomas. Epithelioid tumors account for approximately 60% of mesothelioma diagnoses.[
Examination of the gross tumor at surgery and use of special stains or electron microscopy can often help to determine diagnosis. Pancytokeratin stains are positive in nearly all mesotheliomas.[
References:
American Joint Committee on Cancer (AJCC) Stage Groupings and Definitions of TNM
The AJCC has designated staging by TNM (tumor, node, metastasis) classification to define malignant mesothelioma.[
Cancers staged using the AJCC cancer staging system include classifications for diffuse malignant pleural mesotheliomas but do not include localized malignant pleural mesotheliomas or other primary tumors of the pleura.[
Patients with stage I disease have a significantly better prognosis than patients do with advanced stages. Because of the relative rarity of this disease, exact survival information based on stage is limited.[
Stage | TNM | Description |
---|---|---|
T = primary tumor; N = regional lymph node; M = metastasis. | ||
a Reprinted with permission from AJCC: Malignant Pleural Mesothelioma. In: Amin MB, Edge SB, Greene FL, et al., eds.:AJCC Cancer Staging Manual. 8th ed. New York, NY: Springer, 2017, pp 457–68. | ||
IA | T1, N0, M0 | T1 = Tumor limited to the ipsilateral parietal pleura with or without involvement of: |
–visceral pleura. | ||
–mediastinal pleura. | ||
–diaphragmatic pleura. | ||
N0 = No regional lymph node metastases. | ||
M0 = No distant metastasis. | ||
IB | T2 or T3, N0, M0 | T2 = Tumor involving each of the ipsilateral pleural surfaces (parietal, mediastinal, diaphragmatic, and visceral pleura) with at least one of the following features: |
–involvement of diaphragmatic muscle. | ||
–extension of tumor from visceral pleura into the underlying pulmonary parenchyma. | ||
T3 = Describes locally advanced butpotentially resectable tumor. | ||
Tumor involving all the ipsilateral pleural surfaces (parietal, mediastinal, diaphragmatic, and visceral pleura) with at least one of the following features: | ||
–involvement of the endothoracic fascia. | ||
–extension into the mediastinal fat. | ||
–solitary, completely resectable focus of tumor extending into the soft tissues of the chest wall. | ||
–nontransmural involvement of the pericardium. | ||
N0 = No regional lymph node metastases. | ||
M0 = No distant metastasis. |
Stage | TNM | Description |
---|---|---|
T = primary tumor; N = regional lymph node; M = metastasis. | ||
a Reprinted with permission from AJCC: Malignant Pleural Mesothelioma. In: Amin MB, Edge SB, Greene FL, et al., eds.:AJCC Cancer Staging Manual. 8th ed. New York, NY: Springer, 2017, pp 457–68. | ||
II | T1, N1, M0 | T1 = Tumor limited to the ipsilateral parietal pleura with or without involvement of: |
–visceral pleura. | ||
–mediastinal pleura. | ||
–diaphragmatic pleura. | ||
N1 = Metastases in the ipsilateral bronchopulmonary, hilar, or mediastinal (including the internal mammary, peridiaphragmatic, pericardial fat pad, or intercostal) lymph nodes. | ||
M0 = No distant metastasis. | ||
T2, N1, M0 | T2 = Tumor involving each of the ipsilateral pleural surfaces (parietal, mediastinal, diaphragmatic, and visceral pleura) with at least one of the following features: | |
–involvement of diaphragmatic muscle. | ||
–extension of tumor from visceral pleura into the underlying pulmonary parenchyma. | ||
N1 = Metastases in the ipsilateral bronchopulmonary, hilar, or mediastinal (including the internal mammary, peridiaphragmatic, pericardial fat pad, or intercostal) lymph nodes. | ||
M0 = No distant metastasis. |
Stage | TNM | Description |
---|---|---|
T = primary tumor; N = regional lymph node; M = metastasis. | ||
a Reprinted with permission from AJCC: Malignant Pleural Mesothelioma. In: Amin MB, Edge SB, Greene FL, et al., eds.:AJCC Cancer Staging Manual. 8th ed. New York, NY: Springer, 2017, pp 457–68. | ||
IIIA | T3, N1, M0 | T3 = Describes locally advanced butpotentially resectable tumor. |
Tumor involving all of the ipsilateral pleural surfaces (parietal, mediastinal, diaphragmatic, and visceral pleura) with at least one of the following features: | ||
–involvement of the endothoracic fascia. | ||
–extension into the mediastinal fat. | ||
–solitary, completely resectable focus of tumor extending into the soft tissues of the chest wall. | ||
–nontransmural involvement of the pericardium. | ||
N1 = Metastases in the ipsilateral bronchopulmonary, hilar, or mediastinal (including the internal mammary, peridiaphragmatic, pericardial fat pad, or intercostal) lymph nodes. | ||
M0 = No distant metastasis. | ||
IIIB | T1–3, N2, M0 | T1 = Tumor limited to the ipsilateral parietal pleura with or without involvement of: |
–visceral pleura. | ||
–mediastinal pleura. | ||
–diaphragmatic pleura. | ||
T2 = Tumor involving each of the ipsilateral pleural surfaces (parietal, mediastinal, diaphragmatic, and visceral pleura) with at least one of the following features: | ||
–involvement of diaphragmatic muscle. | ||
–extension of tumor from visceral pleura into the underlying pulmonary parenchyma. | ||
T3 = Describes locally advanced butpotentially resectable tumor. | ||
Tumor involving all of the ipsilateral pleural surfaces (parietal, mediastinal, diaphragmatic, and visceral pleura) with at least one of the following features: | ||
–involvement of the endothoracic fascia. | ||
–extension into the mediastinal fat. | ||
–solitary, completely resectable focus of tumor extending into the soft tissues of the chest wall. | ||
–nontransmural involvement of the pericardium. | ||
N2 = Metastases in the contralateral mediastinal, ipsilateral, or contralateral supraclavicular lymph nodes. | ||
M0 = No distant metastasis. | ||
T4, Any N, M0 | T4 = Describes locally advancedtechnically unresectable tumor. Tumor involving all of the ipsilateral pleural surfaces (parietal, mediastinal, diaphragmatic, and visceral pleura) with at least one of the following features: | |
–diffuse extension or multifocal masses of the tumor in the chest wall, with or without associated rib destruction. | ||
–direct transdiaphragmatic extension of the tumor to the peritoneum. | ||
–direct extension of the tumor to the contralateral pleura. | ||
–direct extension of the tumor to the mediastinal organs. | ||
–direct extension of the tumor into the spine. | ||
–tumor extending through to the internal surface of the pericardium with or without a pericardial effusion; or tumor involving the myocardium. | ||
NX = Regional lymph nodes cannot be assessed. | ||
N0 = No regional lymph node metastases. | ||
N1 = Metastases in the ipsilateral bronchopulmonary, hilar, or mediastinal (including the internal mammary, peridiaphragmatic, pericardial fat pad, or intercostal) lymph nodes. | ||
N2 = Metastases in the contralateral mediastinal, ipsilateral, or contralateral supraclavicular lymph nodes. | ||
M0 = No distant metastasis. |
Stage | TNM | Description |
---|---|---|
T = primary tumor; N = regional lymph node; M = metastasis. | ||
a Reprinted with permission from AJCC: Malignant Pleural Mesothelioma. In: Amin MB, Edge SB, Greene FL, et al., eds.:AJCC Cancer Staging Manual. 8th ed. New York, NY: Springer, 2017, pp 457–68. | ||
IV | Any T, Any N, M1 | TX = Primary tumor cannot be assessed. |
T0 = No evidence of primary tumor. | ||
T1 = Tumor limited to the ipsilateral parietal pleura with or without involvement of: | ||
–visceral pleura. | ||
–mediastinal pleura. | ||
–diaphragmatic pleura. | ||
T2 = Tumor involving each of the ipsilateral pleural surfaces (parietal, mediastinal, diaphragmatic, and visceral pleura) with at least one of the following features: | ||
–involvement of diaphragmatic muscle. | ||
–extension of tumor from visceral pleura into the underlying pulmonary parenchyma. | ||
T3 = Describes locally advanced butpotentially resectable tumor. | ||
Tumor involving all of the ipsilateral pleural surfaces (parietal, mediastinal, diaphragmatic, and visceral pleura) with at least one of the following features: | ||
–involvement of the endothoracic fascia. | ||
–extension into the mediastinal fat. | ||
–solitary, completely resectable focus of tumor extending into the soft tissues of the chest wall. | ||
–nontransmural involvement of the pericardium. | ||
T4 = Describes locally advancedtechnically unresectable tumor. Tumor involving all of the ipsilateral pleural surfaces (parietal, mediastinal, diaphragmatic, and visceral pleura) with at least one of the following features: | ||
–diffuse extension or multifocal masses of the tumor in the chest wall, with or without associated rib destruction. | ||
–direct transdiaphragmatic extension of the tumor to the peritoneum. | ||
–direct extension of the tumor to the contralateral pleura. | ||
–direct extension of the tumor to the mediastinal organs. | ||
–direct extension of the tumor into the spine. | ||
–tumor extending through to the internal surface of the pericardium with or without a pericardial effusion; or tumor involving the myocardium. | ||
NX = Regional lymph nodes cannot be assessed. | ||
N0 = No regional lymph node metastases. | ||
N1 = Metastases in the ipsilateral bronchopulmonary, hilar, or mediastinal (including the internal mammary, peridiaphragmatic, pericardial fat pad, or intercostal) lymph nodes. | ||
N2 = Metastases in the contralateral mediastinal, ipsilateral, or contralateral supraclavicular lymph nodes. | ||
M1 = Distant metastasis present. |
References:
Standard treatment for all but localized mesothelioma is generally not curative. Although some patients will experience long-term survival with aggressive treatment approaches, it remains unclear if overall survival (OS) has been significantly altered by the different treatment modalities or by combinations of modalities.
Extrapleural pneumonectomy in selected patients with early-stage disease may improve recurrence-free survival, but its impact on OS is unknown.[
Several single-arm phase II studies have demonstrated prolonged survival times (compared with historic controls) for selected patients who received adjuvant radiation therapy after definitive surgery.[
References:
Standard Treatment Options for Localized Malignant Mesothelioma (Stage I)
Standard treatment options for localized malignant mesothelioma (stage I) include the following:[
Treatment Options Under Clinical Evaluation for Localized Malignant Mesothelioma (Stage I)
Treatment options under clinical evaluation for localized malignant mesothelioma (stage I) include the following:
Current Clinical Trials
Use our advanced clinical trial search to find NCI-supported cancer clinical trials that are now enrolling patients. The search can be narrowed by location of the trial, type of treatment, name of the drug, and other criteria. General information about clinical trials is also available.
References:
Standard Treatment Options for Advanced Malignant Mesothelioma (Stages II, III, and IV)
Standard treatment options for advanced malignant mesothelioma (stages II, III, and IV) include the following:
Information about ongoing clinical trials is available from the NCI website.
Treatment Options Under Clinical Evaluation for Advanced Malignant Mesothelioma (Stages II, III, and IV)
Treatment options under clinical evaluation for advanced malignant mesothelioma (stages II, III, and IV) include the following trials:
After 117 patients had enrolled, folic acid and vitamin B12 were added to reduce toxic effects.
Dexamethasone (4 mg) or an equivalent corticosteroid was administered orally twice daily for skin rash prophylaxis to all patients 1 day before, on the day of, and 1 day after each pemetrexed dose.
Combination immune checkpoint inhibitor therapy may be considered as an alternative to combination chemotherapy.
Malignant Peritoneal Mesothelioma
Malignant peritoneal mesothelioma arises from the mesothelial cells of the peritoneum and spreads within the confines of the abdominal cavity. There have been few prospective clinical trials conducted in this patient population. Most clinical evidence arises from single-center retrospective studies. Male sex and sarcomatoid or biphasic subtype disease are correlated with poor prognosis.[
Cytoreductive surgery and hyperthermic intraperitoneal chemotherapy (HIPEC) are offered to patients with diffuse malignant peritoneal mesothelioma, no extraperitoneal disease spread, a good performance status, and those predicted to achieve complete surgical cytoreduction. Among centers with expertise in this form of therapy, reported median survival for appropriately selected patients approaches 3 to 4 years.[
In patients with malignant peritoneal mesothelioma, efficacy of pemetrexed plus cisplatin appeared comparable to that seen in pleural mesothelioma, and was been well tolerated. Prospective phase II or III clinical trials of this regimen have not been conducted in patients with peritoneal mesothelioma.
Carboplatin may be substituted for cisplatin.
The pemetrexed/cisplatin/bevacizumab and nivolumab/ipilimumab regimens that improved OS were conducted exclusively in patients with malignant pleural mesothelioma, and data to support the efficacy and safety of these regimens in patients with malignant peritoneal mesothelioma are required.
Current Clinical Trials
Use our advanced clinical trial search to find NCI-supported cancer clinical trials that are now enrolling patients. The search can be narrowed by location of the trial, type of treatment, name of the drug, and other criteria. General information about clinical trials is also available.
References:
Treatment of patients with recurrent malignant mesothelioma usually utilizes procedures and agents not previously employed in the initial treatment attempt. No standard treatment approaches have improved survival or controlled symptoms for a prolonged period. Selected patients with localized disease recurrence may be candidates for additional chest wall resection. One trial of 47 carefully selected patients at a single institution indicated that chest wall resection could be safely performed; time-to-recurrence from initial resection appeared to be predictive of expected survival benefit and was factored into decision-making.[
Patients with recurrence are candidates for phase I and II clinical trials evaluating new targeted therapies, biologicals, chemotherapeutic agents, or physical approaches.[
Treatment Options for Recurrent Malignant Mesothelioma
Current Clinical Trials
Use our advanced clinical trial search to find NCI-supported cancer clinical trials that are now enrolling patients. The search can be narrowed by location of the trial, type of treatment, name of the drug, and other criteria. General information about clinical trials is also available.
References:
The PDQ cancer information summaries are reviewed regularly and updated as new information becomes available. This section describes the latest changes made to this summary as of the date above.
Recurrent Malignant Mesothelioma Treatment
Added Treatment Options for Recurrent Malignant Mesothelioma as a new subsection.
This summary is written and maintained by the PDQ Adult Treatment Editorial Board, which is editorially independent of NCI. The summary reflects an independent review of the literature and does not represent a policy statement of NCI or NIH. More information about summary policies and the role of the PDQ Editorial Boards in maintaining the PDQ summaries can be found on the About This PDQ Summary and PDQ® - NCI's Comprehensive Cancer Database pages.
Purpose of This Summary
This PDQ cancer information summary for health professionals provides comprehensive, peer-reviewed, evidence-based information about the treatment of adult malignant mesothelioma. It is intended as a resource to inform and assist clinicians in the care of their patients. It does not provide formal guidelines or recommendations for making health care decisions.
Reviewers and Updates
This summary is reviewed regularly and updated as necessary by the PDQ Adult Treatment Editorial Board, which is editorially independent of the National Cancer Institute (NCI). The summary reflects an independent review of the literature and does not represent a policy statement of NCI or the National Institutes of Health (NIH).
Board members review recently published articles each month to determine whether an article should:
Changes to the summaries are made through a consensus process in which Board members evaluate the strength of the evidence in the published articles and determine how the article should be included in the summary.
The lead reviewers for Malignant Mesothelioma Treatment (Adult) are:
Any comments or questions about the summary content should be submitted to Cancer.gov through the NCI website's Email Us. Do not contact the individual Board Members with questions or comments about the summaries. Board members will not respond to individual inquiries.
Levels of Evidence
Some of the reference citations in this summary are accompanied by a level-of-evidence designation. These designations are intended to help readers assess the strength of the evidence supporting the use of specific interventions or approaches. The PDQ Adult Treatment Editorial Board uses a formal evidence ranking system in developing its level-of-evidence designations.
Permission to Use This Summary
PDQ is a registered trademark. Although the content of PDQ documents can be used freely as text, it cannot be identified as an NCI PDQ cancer information summary unless it is presented in its entirety and is regularly updated. However, an author would be permitted to write a sentence such as "NCI's PDQ cancer information summary about breast cancer prevention states the risks succinctly: [include excerpt from the summary]."
The preferred citation for this PDQ summary is:
PDQ® Adult Treatment Editorial Board. PDQ Malignant Mesothelioma Treatment (Adult). Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/mesothelioma/hp/mesothelioma-treatment-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389420]
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Last Revised: 2022-01-14
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