Malignant pleural disease usually heralds a poor prognosis, whether it represents a primary pleural malignancy or metastatic involvement.
Clinical presentation is variable. Patients may be asymptomatic or have pleuritic pain. If associated with a sizable pleural effusion, then respiratory compromise may be evident. If a tumor is from the chest wall, then musculoskeletal pain or even a palpable mass may be present.
The pleura can be involved by malignancy by three main mechanisms:
- direct extension from an adjacent tumor, e.g. bronchogenic carcinoma
- a primary tumor, e.g. mesothelioma, primary pleural lymphoma
- pleural metastases
In each of these, the manifestation can be either solid disease or a malignant pleural effusion, or a combination of both. The relative etiology is 1,6:
- metastatic carcinoma (e.g. primary lung): 58%
- mesothelioma: 28%
- non-Hodgkin lymphoma: 8%
- metastatic melanoma
- invasive thymoma
- rare primary pleural malignancies 7
- epithelioid angiosarcoma
- epithelioid hemangioendothelioma
- synovial sarcoma
- pleuropulmonary blastoma
- desmoplastic small round cell tumor of the pleura
Conventional imaging is in general highly specific for the diagnosis of pleural malignancy but unfortunately lacks sensitivity 3.
Plain films in the setting of malignant pleural effusion are insensitive at distinguishing it from a benign effusion. See malignant vs benign pleural effusion
In cases where multiple nodular regions or pleural thickening are present the diagnosis may be evident, especially if a primary tumor or other metastatic deposits are visible.
CT is the work-horse of pleural imaging, able to achieve specificities of close to 100% 3. The portal-venous phase (also known as the pleural phase in the chest) is optimal for demonstrating pleural enhancement, rather than arterial phase imaging used routine thoracic CT. A number of features are recognized, including 1,3:
- circumferential pleural thickening
- nodular pleural thickening
- parietal pleural thickening greater than 1 cm
- mediastinal pleural involvement
- pleural calcification generally suggests a benign process
FDG-PET is far more sensitive than conventional imaging in diagnosing malignant pleural disease and distinguishing them from benign processes 3. Hypermetabolism can be seen in either the pleura or pleural effusion. Although both benign and malignant processes, benign processes rarely increase the SUV over 2.0 3.
MRI findings of high signal intensity on T2 in relation to intercostal muscles and/or contrast-enhanced on T1 images together with CT morphology has a 100% and a specificity of 93% in the detection of pleural malignancy 5.
Treatment and prognosis
Both treatment and prognosis are dependent on the underlying type of malignancy. In the case of primary malignancies of the pleura (e.g. mesothelioma) resection may be a possibility in highly selected cases.
Thoracentesis of malignant pleural effusions has limited effect with reaccumulation occurring, on average, at four days.
In general, and certainly in the case of diffuse pleural metastatic disease, systemic therapies or radiotherapy are the only options. These are discussed separately as part of articles pertaining to individual malignancies:
Differential diagnosis depends on the predominant form the pleural disease takes:
- 1. Leung AN, Müller NL, Miller RR. CT in differential diagnosis of diffuse pleural disease. AJR Am J Roentgenol. 1990;154 (3): 487-92. AJR Am J Roentgenol (abstract) - Pubmed citation
- 2. Kuhlman JE, Singha NK. Complex disease of the pleural space: radiographic and CT evaluation. Radiographics. 17 (1): 63-79. Radiographics (abstract) - Pubmed citation
- 3. Naidich DP, Srichai MB, Krinsky GA. Computed tomography and magnetic resonance of the thorax. Lippincott Williams & Wilkins. (2007) ISBN:0781757657. Read it at Google Books - Find it at Amazon
- 4. Reeder MM, Felson B. Reeder and Felson's gamuts in radiology, comprehensive lists of roentgen differential diagnosis. Springer Verlag. (2003) ISBN:0387955887. Read it at Google Books - Find it at Amazon
- 5. Hierholzer J, Luo L, Bittner RC et-al. MRI and CT in the differential diagnosis of pleural disease. Chest. 2000;118 (3): 604-9. Chest (citation) - Pubmed citation
- 6. Muduly D, Deo S, Subi Ts et-al. An update in the management of malignant pleural effusion. Indian J Palliat Care. 2011;17 (2): 98-103. doi:10.4103/0973-1075.84529 - Free text at pubmed - Pubmed citation
- 7. Richard Luther Attanoos, Matthew Richard Pugh. The Diagnosis of Pleural Tumors Other Than Mesothelioma. (2018) Archives of Pathology & Laboratory Medicine. 142 (8): 902-913. doi:10.5858/arpa.2017-0537-RA - Pubmed