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Imaging of pleural plaques, thickening, and tumors

Imaging of pleural plaques, thickening, and tumors
Literature review current through: Jan 2024.
This topic last updated: Aug 26, 2022.

INTRODUCTION — Imaging procedures are commonly used in the diagnostic evaluation of pleural abnormalities. The imaging of pleural plaques, diffuse pleural thickening, and pleural tumors will be reviewed here. The imaging of pleural effusions and pneumothorax are discussed separately. (See "Imaging of pleural effusions in adults".)

PLEURAL PLAQUES — Pleural plaques are deposits of hyalinized collagen fibers in the parietal pleura. They are indicative of asbestos exposure and typically become visible twenty or more years after the inhalation of asbestos fibers, although latency periods of less than 10 years have been observed [1-3].

Pleural plaques are presumed to be the result of pleural inflammation caused by asbestos fibers that are transported to the pleural surface along lymphatic channels and/or direct penetration [4,5]. The former may explain why pleural plaques tend to be located near the stomata where asbestos fibers are resorbed by lymphatic flow (ie, Kampmeier foci) [6]. (See "Asbestos-related pleuropulmonary disease".)

Pleural plaques preferentially involve the parietal pleura adjacent to ribs, particularly the sixth through ninth ribs. They are also common along the diaphragmatic pleura. Pleural plaques are less extensive in the intercostal spaces, only rarely occur in the visceral pleura, and are conspicuously absent in the region of the costophrenic sulci and the lung apices (image 1 and image 2).

Calcifications within pleural plaques are identified by chest radiography in 20 percent of patients (image 3 and image 2), by computed tomography (CT) in 50 percent (image 4A-B and image 5), and by morphological examination of autopsy and biopsy specimens in 80 percent [2,6-8].

On chest radiographs, pleural plaques appear as focal areas of pleural thickening when they are viewed tangentially (image 3 and image 6 and image 7). They can have a nodular, stippled, sunburst, irregular, leaf-like, or geographic appearance. When calcified, pleural plaques may also look like a dripping candle or display rolled margins (image 8 and image 9 and image 10 and figure 1). Several site-specific manifestations have to be highlighted:

Diaphragmatic pleural plaques can appear as curvilinear calcifications or as serrations, flat-topped focal areas of pleural thickening, or scalloping altering the smooth contour of the normal diaphragm.

Within fissures, pleural plaques rarely can mimic solitary pulmonary nodules.

On CT, pleural plaques appear as focal pleural thickening (image 4A-B and image 5). They can have a linear, band-like, or nodular appearance and may impinge slightly on the adjacent lung parenchyma. This impingement may occasionally cause a pulmonary subpleural curvilinear line adjacent to the plaque, which is indicative of focal lung fibrosis.

CT clearly distinguishes pleural plaques from extrapleural fat and endothoracic fascia [7]. This is valuable because extrapleural fat may mimic a pleural plaque on chest radiographs, but a subsequent CT scan will reveal the true nature of the abnormality [9-11]. A large body-mass index can be an important factor that influences the over-reading of pleural plaques and leads to false-positive diagnosis of pleural plaques on chest radiographs. This finding can be clarified with CT scanning that demonstrates the fatty nature of presumed pleural plaques [12].

Pleural plaques do not have further disease potential and, therefore, do not need to be followed up radiologically in asymptomatic patients unless an atypical, mass-like appearance warrants further examination or the patient has a diagnosis of lymphoma which can, on rare occasions mimic noncalcified asbestos-related pleural plaques. However, clinicians should be aware that pleural plaques are markers of asbestos exposure and asbestos exposure is a risk factor for malignant mesothelioma, lung cancer, and asbestosis [13].

DIFFUSE PLEURAL THICKENING — Diffuse pleural thickening typically begins as fibrosis of the visceral pleura with secondary thickening of the parietal pleura. The visceral and parietal pleurae may become inseparable, leading to obliteration of the costophrenic sulci. In addition, fatty tissue deposition external to the parietal pleura is probably due to adipose tissue being drawn inward during pleural retraction and cicatrization (image 11).

Radiologically, diffuse pleural thickening forms a smooth, uninterrupted pleural opacity. It characteristically extends over at least 25 percent of the chest wall and obliterates the costophrenic sulci. CT criteria that define diffuse pleural thickening include the following [10,14,15]:

The pleural thickening extends more than 8 cm in the craniocaudal direction

The pleural thickening extends along more than 5 cm of the chest wall when visualized in cross-section

The pleural thickness exceeds 3 mm

Calcification of the inner aspect of the thickened pleura is common and facilitates accurate measurement of the pleural thickness (image 12).

Diffuse pleural thickening is usually a consequence of intense pleural inflammation, such as that following infection (image 13 and image 14 and image 15 and picture 1). It can also be a response to other types of injuries, such as prior empyema, pleural hemorrhage, prior pneumothorax therapy for tuberculosis [16], occupational exposure (eg, benign asbestos-related pleural effusion), chronic silicosis [17], trauma, radiation, pulmonary embolism, medication, or a neoplasm (image 16) [2,9,18].

Pleural thickening can occur in the lung apices, but this is usually not caused by tuberculosis, unless accompanying adjacent scarring of the lung parenchyma is present. It has been hypothesized that pleural thickening in the lung apices may be related to apical ischemia and gravitational stress due to the upright posture (especially since its frequency increases with age) or to extrapleural fat deposits [8].

Patients with diffuse pleural thickening should be followed for the potential progression to restrictive disease [19]. This generally includes serial assessment by physical examination, chest imaging, and pulmonary function testing. The frequency of these assessments should be individualized according to the suspected rate of progression to restrictive pathophysiology. Patients who report progressive symptoms warrant more frequent follow-up.

A rare cause of diffuse non-neoplastic pleural thickening is Erdheim-Chester disease, a rare multisystem disorder classified as a non-Langerhans cell histiocytosis, characterized by xanthomatous infiltration of organs by foamy lipid-laden macrophages. Most patients with this disease have long bone sclerosis and symmetric circumferential pleural thickening, smooth interlobular septal thickening, centrilobular nodules, pericardial thickening, periaortic thickening, and perinephric thickening [20,21]. Another unusual cause of diffuse pleural thickening is diffuse pulmonary lymphangiomatosis, which consists of a congenital proliferation and dilation of lymphatic channels in the pleura, interlobular septa, and mediastinum. The imaging manifestations include, in addition to pleural thickening, smooth interlobular septal and peribronchovascular interstitial thickening, diffuse mediastinal fat infiltration, pleural effusions, and pericardial effusion. The disease can progress to respiratory failure [20]. Immunoglobulin G4 (IgG4)-related disease [22] can lead to inflammatory and fibrotic changes of multiple organs, including fibrosing mediastinitis, inflammatory pseudotumor of the lung, retroperitoneal fibrosis, sclerosing pancreatitis, and sclerosing cholangitis. Pleural disease has also been described, leading to the formation of pleural nodules either related to the visceral or parietal pleura. On rare occasions such pleural tumors can mimic pleural mesothelioma [23,24].

PLEURAL TUMORS — The majority of pleural tumors are malignant. Among malignant pleural tumors, most are due to metastatic disease (image 17A-B and image 18) rather than to a primary pleural malignant neoplasm (image 19A-B and image 20).

The mainstay for imaging of pleural tumors remains multislice, multidetector row, multichannel, volumetric CT scanning with intravenous, iodinated contrast-material enhancement; it provides exquisite spatial resolution and allows for near-isotropic multiplanar reformatting. Several radiological features identified by CT are helpful in distinguishing malignant from benign pleural disease. Features associated with malignant disease include circumferential thickening, nodular thickening, thickness greater than 1 cm, and involvement of the mediastinal pleura [14,16]. Dual-energy CT derives iodine maps and allows for differentiation of malignant pleural disease with higher concentration of iodine than benign disease [25].

Magnetic resonance imaging (MRI) with gadolinium-based contrast material has a high sensitivity and specificity for pleural malignant tumors, but is usually not performed due to greater expense and longer duration of the procedure [16]. Diffusion-weighted MR imaging can demonstrate restricted diffusion with high signal in malignant lesions, ie, in 96.5 percent of malignant pleural mesothelioma and 70 percent of metastatic pleural disease but in only 15 to 20 percent of benign conditions [26]. Integrated positron emission tomography computed tomography (PET-CT) with 18-fluorodeoxyglucose (FDG) may also be abnormal in the setting of pleural malignancy, but is not accurate enough for routine use [27].

Metastatic disease — Disease can spread from a distant primary malignant tumor to the pleura in several ways: hematogenous dissemination, direct invasion from an adjacent carcinoma or subpleural tumor (eg, lymphoma, (image 21 and image 22)), and pleural seeding (eg, thymoma or bronchogenic carcinoma, (image 23)) [28,29]. Pleural seeding is sometimes referred to as drop metastases and can lead to multiple nodules carpeting the pleura. Lung cancers, breast and ovarian cancers have the highest prevalence for parietal pleural metastases [25].

These mechanisms can result in a spectrum of imaging findings. Pleural involvement by metastatic disease can be wet, resulting in a pleural effusion that can be detected radiologically. Alternatively, pleural involvement can be dry, resulting in a wide variety of imaging abnormalities ranging from smooth or irregular to nodular pleural thickening. Imaging appearances that are suggestive of pleural metastases include pleural puckering, pleural beading, or large pleural nodules. Pleural puckering indicates disruption of the internal elastic lamina by invading tumor. Pleural beading may represent multiple small nodular metastases attached to the visceral pleura, which can be visible during thoracoscopy.

The radiographic appearance of metastatic pleural disease is not diagnostic of a malignant process. However, the detection of the type or degree of metastatic pleural involvement by CT has been shown in the following studies to have important implications for prognosis and management of patients with non-small cell lung cancer (NSCLC).

Dry pleural dissemination can manifest with subtle beading or nodularity in the interlobar fissures without an accompanying pleural effusion. Compared to wet pleural dissemination, dry pleural dissemination in a single center retrospective study of 98 patients with NSCLC, was associated with a longer median survival (38 versus 13 months) [30].

The infiltration of extra-parietal pleural fat as opposed to pleural nodules or effusion, distinguishes T3 (stage IIB) from M1a (stage IV) disease (table 1) [31]. Stage IIB NSCLC is potentially surgically resectable with improved prognosis compared to stage IV disease, which is nonresectable and carries a poor prognosis. (See "Tumor, node, metastasis (TNM) staging system for lung cancer".)

The likelihood of pleural invasion by NSCLC has been estimated in one study by using the ratio of the arch distance (the length of the interface between the primary tumor and neighboring structures) and the maximum tumor diameter [32]. A cut-off ratio of 0.9 best distinguished between pl3 tumors (tumor that invades any component of the parietal pleura) from pl1 and pl2 tumors with a sensitivity and specificity of 89.7 and 96 percent, respectively. This ratio requires further validation before it can be routinely used to estimate the likelihood of pleural invasion by NSCLC [30]. An area of contact between the tumor and the visceral pleural exceeding 3 cm in width is also suspicious for pleural invasion.

CT-defined visceral pleural invasion (VPI) in T1 lung adenocarcinoma includes tumor contact length greater than one-fourth of the tumor circumference, pleural retraction, and pleural tags with thickening at the pleural end [33,34]. These findings likely indicate pathologic infiltration of the visceral pleural elastic membrane and visceral pleural surface invasion yet they don’t have a consistent negative relationship to disease-free survival (DFS).

Primary tumors — Primary pleural lesions arise directly from the pleural membranes. Chest radiographic findings of pleural and extrapleural tumors include the incomplete border sign: the border of the lesion is indistinct towards the chest wall or the mediastinum, but sharp towards the adjacent lung, particularly when not imaged in tangent.

Pleural and chest wall tumors have tapered borders and obtuse angles of interface with the chest wall or mediastinum. In contrast, pulmonary masses are circumferentially surrounded by lung parenchyma and have acute angles of interface with the chest wall, the mediastinum, and the lung parenchyma [35].

Malignant mesothelioma — Contrast-enhanced CT of the chest and upper abdomen is recommended as the initial method of investigation. PET-CT can add value when CT findings alone are indeterminate. MRI can provide greater soft-tissue detail and add more detailed information on isolated foci of disease, chest-wall invasion, or invasion of surrounding structures [36]. The most common CT findings associated with malignant mesothelioma are pleural thickening (92 percent), thickening and nodularity of the interlobar fissures (86 percent), pleural effusion (74 percent), contraction of a hemithorax (42 percent), midline mediastinum (44 percent), contralateral shift of the mediastinum (14 percent), chest wall involvement (18 percent), and rib destruction (10 percent) (image 19A-B and image 24 and image 25 and image 26A-B and image 27) [37-40]. Associated calcified pleural plaques are seen in 20 percent of patients with malignant mesothelioma on CT scans; they should not be mistaken for rare osteocartilaginous differentiation in malignant mesothelioma, particularly the sarcomatoid variant, which presents as massive or punctate foci of mineralization within the pleural mass or thickening [41]. Epithelioid malignant mesotheliomas have a propensity for the interlobar fissures.

Imaging features of malignant mesothelioma can be mimicked by metastatic adenocarcinoma and rarely by benign, inflammatory pleural disease. However, once malignant mesothelioma is diagnosed by tissue sampling, CT imaging is helpful in evaluating features that characterize the severity of disease: chest wall invasion, mediastinal invasion, lymph node involvement, and transdiaphragmatic growth of tumor into the abdomen. The addition of MRI may improve the evaluation of chest wall and apical involvement, as well as transdiaphragmatic tumor growth (image 28A-B). Multiplanar, near-isotropic imaging with multislice, multidetector-row volumetric CT scanning can display these features as well. (See "Magnetic resonance imaging of the thorax".)

The radiologic differential diagnosis in cases of malignant mesothelioma includes pleural metastases, pleural dissemination of thymoma and epithelioid hemangioendothelioma [41]. Epithelioid hemangioendothelioma represents an unusual vascular neoplasm of the lung, pleural, and liver and may be related to asbestos exposure. It presents with pleural effusions, pleural thickening, and pleural masses and can be differentiated from malignant mesothelioma with immunohistochemistry [41].

The non-radiological aspects of malignant mesothelioma are discussed elsewhere. (See "Epidemiology of malignant pleural mesothelioma" and "Presentation, initial evaluation, and prognosis of malignant pleural mesothelioma" and "Initial management of malignant pleural mesothelioma".)

Other — Other primary pleural tumors include lipomas, fibrous tumors, and chest wall tumors like neurogenic tumors (image 29 and image 30 and image 31 and image 32 and image 33 and image 34). Benign desmoid tumors can form at the site of a previous thoracotomy or in paraspinal location along the mediastinal pleura. These masses that develop at the incision site can grow relatively fast, be locally aggressive, are FDG-PET negative, and can mimic lung cancer recurrence [42].

Lipomas are pleural or extrapleural masses with homogeneous fatty content. They tend to occur in the upper chest along the second or third ribs. Their diagnosis is facilitated by CT, which can reliably distinguish fat from other tissues, due to fat’s low attenuation coefficient of -50HU to -100HU (image 35 and image 36 and image 37) [8].

Solitary fibrous tumors of the pleura originate from submesothelial cells, typically appear on CT as a mass with a diameter of 5 to 30 cm (image 38). A pedicle is present in 40 percent of cases, resulting in marked tumor mobility (image 39) [43,44]. In rare cases, torsion of the tumor pedicle can lead to necrosis of the tumor with gas accumulation in the mass. Solitary fibrous tumors can have cystic components and may enhance after intravenous contrast material administration. Occasionally, they may simulate mediastinal, paravertebral, diaphragmatic or intrapulmonary masses, depending upon their exact location within the pleural cavity (image 40A-B and image 41 and image 42) (see "Solitary fibrous tumor"). The radiologic diagnosis is supported by the clinical findings of hypoglycemia, the so-called Doege-Potter syndrome and hypertrophic pulmonary osteoarthropathy with periosteal new bone formation [45,46].

Focal tumor-like conditions can mimic pleural tumors and include traumatic diaphragmatic rupture with hernias [47], posttraumatic thoracic splenosis which presents as vascular enhancing pleural nodules: Technetium 99m nuclear medicine scans are positive in splenosis.

Pleural endometriosis, which can present with pleural nodules and supradiaphragmatic nodularity, is best displayed on CT scans and pleural effusion and catamenial pneumothorax visible on chest radiographs and CT scans [48,49]. Intrathoracic migration of silicone breast implants after cardiothoracic surgery has been described as an exceedingly rare complication [50], presenting as a dependent pleural mass. Extrapleural hematoma can displace the endothoracic fascia and extrapleural fat into the pleural space and lead to compression of the adjacent lung [51,52]. (See "Clinical features, diagnostic approach, and treatment of adults with thoracic endometriosis".)

Intercostal lung hernias lead to a chest wall bulge, containing lung that can be mistaken clinically for a chest wall tumor. They occur after prior surgery at the site of incision and they result due to absence of intercostal muscles and weakening of the endothoracic fascia [53,54].

Loculated pleural effusions, particularly pleural pseudotumors in the interlobar fissures can mimic solid tumors on chest radiographs; their nature is clarified with CT scanning (image 43 and image 44 and image 45) [20].

SUMMARY AND RECOMMENDATIONS

Pleural plaques – Pleural plaques are markers of asbestos exposure. They appear as focal areas of pleural thickening on both chest radiography and CT. They preferentially involve the parietal pleura adjacent to ribs, particularly the sixth through ninth ribs. Pleural plaques are common along the diaphragm and are often calcified. Pleural plaques can be simulated by extrapleural fat deposits, particularly in persons with large body habitus. Pleural plaques alone do not need to be followed up radiologically in asymptomatic patients unless an atypical, mass-like appearance warrants further examination or the patient has a diagnosis of lymphoma, which can mimic noncalcified asbestos-related pleural plaques. (See 'Pleural plaques' above.)

Pleural thickening – Diffuse pleural thickening forms a smooth, uninterrupted pleural peel. When visualized by CT, it extends more than 8 cm in the craniocaudal direction, encompasses more than 5 cm of the chest wall when visualized in cross-section, and has a thickness that exceeds 3 mm. In addition, chest radiography reveals ipsilateral blunting of the costophrenic sulcus. (See 'Diffuse pleural thickening' above.)

Pleural tumors – Most pleural tumors are malignant. CT features that may be helpful in distinguishing malignant from benign pleural disease include circumferential thickening, nodular thickening, thickness exceeding 1 cm, and involvement of the mediastinal pleura. (See 'Pleural tumors' above.)

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