Introduction
Background
Most pleural neoplasms are metastatic in origin. Primary tumors of the pleura can be categorized as diffuse or localized. Diffuse malignant mesothelioma is more common, related to asbestos exposure, and associated with a poor prognosis. Localized mesothelioma, called localized fibrous tumor of the pleura (LFTP), is a less common neoplasm of controversial histogenesis and is unrelated to asbestos exposure.
LFTPs exist in benign and malignant forms. Only rarely is the localized fibrous tumor invasive or does it cause local recurrence after resection. The ratio of benign to malignant tumors is 7:1. The diagnosis of LFTP is important because the tumor is potentially resectable for cure despite its typically large size. In many cases, resection can repeatedly be used to treat recurrence, although usually with increasing difficulty.1
Pathophysiology
The etiology of LFTPs is unknown. No association exists with smoking or asbestos exposure. Although some studies indicate that LFTPs are mesothelial in origin, other reports suggest that they originate from primitive submesothelial mesenchymal cells that are distinct from diffuse malignant mesothelioma. Histologically, the lesions are usually composed of spindle-shaped cells and variable fibrous stroma. Occasionally, oval or polygonal cells may be present. Areas of myxoid degeneration, hyalinization, necrosis, or hemorrhage can be present, especially with large masses.
The malignant variant of the LFTP has high cellularity and nuclear pleomorphic mitotic activity. Hemorrhage and necrosis are more frequent in the malignant form. Grossly, an LFTP is a firm soft-tissue mass that is usually larger than 5 cm in diameter. The tumor can arise anywhere in the chest along the pleura, although it appears more commonly from the visceral pleura than it does from the parietal pleura. Lesions can arise from the interlobar fissures. The tumor is often attached by a short pedicle. In 1 study, approximately 50% of tumors were found to have pedicles, and 50% were broad based.
Intrapulmonary lesions are reported but are exceedingly rare. LFTPs are solitary in the overwhelming majority of cases; the presence of synchronous lesions is extremely rare.
Frequency
United States
LFTP is a rare disease.
Mortality/Morbidity
Resectability has been shown to be the single most important determinant of the patient's clinical outcome.
Race
No racial predilection has been described.
Sex
Males and females are affected in almost equal numbers.
Age
LFTPs can affect all age groups, but they are most often seen in people who are in their sixth or seventh decade.
Presentation
Most patients are asymptomatic, and the lesion is discovered incidentally on chest radiographs. When present, symptoms are usually related to the local mass effect of large lesions or to the associated paraneoplastic phenomena. Symptomatic patients may report dyspnea, cough, or vague chest or shoulder discomfort.
Paraneoplastic manifestations have been reported in LFTP, including hypertrophic pulmonary osteoarthropathy and hypoglycemia. Hypertrophic pulmonary osteoarthropathy was reported in 4-35% of patients in some series, and it was found to be associated with LFTP more frequently than with lung cancer. Hypoglycemia has been less frequently associated with LFTP; it has been reported in 5% of patients and could be related to insulinlike growth factor type 2 [IGF-2].
The tumor may become large, occupying much of the hemithorax.
Preferred Examination
Usually, LFTP is incidentally discovered on chest radiographs. Findings from computed tomography (CT) scanning and magnetic resonance imaging (MRI) can suggest the diagnosis of LFTP. However, histopathologic examination is needed for a definitive diagnosis.
Limitations of Techniques
Chest radiographic findings are nonspecific, and the lesion can sometimes be obscured by associated pleural effusion. CT scans and magnetic resonance images may show characteristic findings that are suggestive of LFTP but that are not always pathognomonic. The pleural origin of large lesions can be difficult to detect, especially on chest radiographs and even on CT scans and magnetic resonance images.
Differential Diagnoses
Lung Cancer, Non-Small Cell
Mesothelioma, Malignant
Other Problems to Be Considered
Sarcoma
Elevated hemidiaphragm
Loculated pleural effusion
Pleural metastases
Pleural desmoid
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References
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Further Reading
Keywords
LFTP, benign mesothelioma, fibrous mesothelioma, localized mesothelioma, diffuse mesothelioma, malignant mesothelioma, subpleural fibroma, submesothelial fibroma, pleural fibroma, pleural fibromyxoma, solitary fibrous tumor, localized fibrous pleural tumor, lung tumor, pleural tumor, pleural neoplasm, lung cancer, diffuse pleural tumor, localized pleural tumor
