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Pleural mesothelioma
Last reviewed: 23.04.2024
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[ What causes pleural mesothelioma?
The lifetime risk of developing the disease in asbestos workers is approximately 10%, with an average latency of 30 years. The risk is independent of smoking. Mesothelioma may spread locally or metastasize to the pericardium, diaphragm, peritoneum, and, rarely, the tunica vaginalis testis.
Symptoms of pleural mesothelioma
Patients most commonly report dyspnea and nonpleuritic chest pain. Symptoms of pleural mesothelioma that indicate generalized disease are rare during clinical manifestations of the disease. Invasion of the chest wall and other adjacent structures may cause severe pain, dysphonia, dysphagia, Horner's syndrome, brachial plexopathy, or ascites. Extrathoracic extension occurs in 80% of patients, typically involving the hilar and mediastinal lymph nodes, liver, adrenal glands, and kidneys.
Where does it hurt?
What's bothering you?
Diagnosis of pleural mesothelioma
Pleural mesothelioma, which accounts for more than 90% of all cases, appears on radiography as diffuse unilateral or bilateral pleural thickening that appears to envelop the lungs, usually resulting in enlarged costophrenic angles. Pleural effusion is present in 95% of cases and is usually unilateral and massive. Diagnosis is based on pleural fluid cytology or pleural biopsy and, if nondiagnostic, biopsy during video-assisted thoracoscopy (VATS) or thoracotomy. Staging is performed using chest CT, mediastinoscopy, and MRI. The sensitivity and specificity of MRI and CT are comparable, although MRI is useful in determining tumor extension into the vertebral column or spinal cord. PET may have better sensitivity and specificity for distinguishing benign from malignant pleural thickening. Bronchoscopy may reveal associated endobronchial malignancies. Elevated pleural fluid hyaluronidase is suggestive but not diagnostic of the disease. Soluble mesothelin-associated proteins secreted into the serum by mesothelial cells are being studied as possible tumor markers for disease detection and monitoring.
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Treatment of pleural mesothelioma
Pleural mesothelioma remains an incurable cancer. Surgery to remove the pleura; unilateral pneumonectomy, removal of the phrenic nerve and half of the diaphragm; and pericardial ablation with chemotherapy or radiotherapy are possible treatments, but they do not significantly alter prognosis or survival; long-term survival is rare. Furthermore, complete surgical resection is not feasible in most patients. The combination of pemetrexed (an antifolate antimetabolite) and cisplatin has shown promising results but requires further study.
The main goal of supportive care is to relieve pain and dyspnea. Given the diffuse nature of the disease, radiotherapy is usually not possible except for local pain and metastases, but should not be used to treat radicular pain. Pleurodesis or pleurectomy may be used to relieve dyspnea due to pleural effusion. Adequate analgesia is difficult but should be achieved, usually with opioids, using both percutaneous and epidural catheters for pain control. Chemotherapy with cisplatin and gemcitabine can relieve symptoms of pleural mesothelioma in most cases and has been shown to shrink the tumor in half of the patients studied. Some authors suggest multimodal treatment of pleural mesothelioma. Intrapleural administration of granulocyte-macrophage colony-stimulating factor or interferon gamma, intravenous administration of ranpirnase (ribonuclease), and gene therapy are under investigation.
Drugs
What is the prognosis for pleural mesothelioma?
Pleural mesothelioma has a poor prognosis. No treatment for pleural mesothelioma has been shown to significantly increase survival. Survival from diagnosis averages 8-15 months, depending on cell type and location. A small number of patients, usually younger and with shorter duration of symptoms, have a more favorable prognosis, sometimes surviving for several years after diagnosis.