ReviewSolitary fibrous tumors of the pleura
Section snippets
Historical background
The first report of a primary localized pleural tumor is attributed to Wagner [14] in 1870. However, it was only in 1931 that Klemperer and Rabin [15] published the first accurate pathologic description and classified mesothelioma as either “localized” or “diffuse.” Because tissue culture [16] and ultrastructural analysis 17, 18 demonstrated the presence of epithelial-like cells in localized mesotheliomas, Stout and Murray [16] in 1942 claimed that localized mesothelioma had a mesothelial
Clinical features
Solitary fibrous tumors of the pleura have been described in all age groups from 5 to 87 years, but they peak in the sixth and seventh decades of life; they have an even distribution between men and women 7, 9, 25. The majority of patients with malignant SFTP are symptomatic and present with large tumors 7, 10, 26, 27. Symptoms usually include cough, chest pain, and dyspnea. More rarely, hemoptysis and obstructive pneumonitis are observed as a result of airway obstruction 7, 10. Digital
Radiologic features
Benign and malignant SFTP usually appear as a well-defined, homogeneous, and rounded mass on the initial chest radiograph 7, 26, 27, 28. Rarely, a pleural effusion is associated with malignant SFTP 7, 27, 28. While small tumors arising from the parietal pleura classically form obtuse angles with the chest wall, large or pedunculated SFTP may form acute angles and be confused with intrapulmonary masses 37, 38. Pedunculated SFTP have occasionally been reported to be moving on successive chest
Histopathology
Benign and malignant SFTP are widely distributed in the chest. Whereas most of the benign SFTP are small pedunculated tumors, the malignant variants are often larger than 10 cm and grow beneath the parietal pleura of the chest wall, diaphragm, or mediastinum 7, 10, 27, 51.
Macroscopically, benign and malignant tumors appear as firm, smoothly lobulated masses. Most of them are encapsulated by a thin, translucent membrane, containing a reticulated vascular network. Firm adhesion without signs of
Differential diagnosis
The main differential diagnoses of malignant SFTP include pleural mesothelioma, neurogenic sarcoma, synovial sarcoma, hemangiopericytoma, fibrosarcoma, and malignant fibrous histiocytoma 56, 57, 58, 59, 60. Pleural mesotheliomas arise from mesothelial cells and are uniformly malignant. They nearly always present as multiple pleural nodules or as a diffuse tumor that encases a portion of the lung. However, now that SFTP have been clearly recognized as a single entity by means of specific
Cytogenetic analysis
El-Naggar and coworkers [3] analyzed 14 histologically benign fibrous tumors by flow cytometry. Although they observed that all tumors presented a diploid DNA content, two of the recurrent tumors exhibited an elevated S-phase (mean 4% versus 2% in the primary tumors) and a higher number of mitoses (5 to 10 mitoses per 10 high-power fields versus 0 to 3 in primary tumors). Hence, the number of mitoses and corresponding S-phase may reflect rapidly growing lesions that exhibit locally aggressive
Histopathogenesis
Malignant SFTP may occur de novo or degenerate from benign tumor [52]. Indeed, some tumors consist entirely of highly atypical cells, whereas others contain benign areas within the tumor [52]. Possible malignant degeneration of SFTP is further emphasized by the fact that chromosomal anomalies and genetic mutations of the proapoptotic gene p53 are located only in some areas of the tumor, and that benign tumors may recur with histologic signs of malignancy several years after resection of a
Classification
Although solitary fibrous tumors are now well recognized as a single entity in the pleura and in other sites, there are as yet no unifying criteria for classifying these tumors.
Morphologic and histologic indicators seem to be important predictors of outcome [6]. In 1981, Briselli and coworkers [9] presented eight new cases and reviewed 360 cases from the literature. Twelve percent of the tumors followed a malignant course and led to death. The authors observed that the growth pattern of the
Treatment
Complete en bloc surgical resection is the mainstay of therapy for all benign and malignant SFTP. A distance of 1 to 2 cm from the tumor is usually recommended to be in healthy tissue. Whereas pedunculated tumors can be safely resected with a wedge resection of the lung, large sessile tumors can be difficult to resect because of extensive adhesions and may occasionally require a lobectomy or a pneumonectomy in order to achieve complete resection 31, 83. Frozen section can be helpful to
Follow-up care
The risk of recurrence is high after resection of a malignant sessile SFTP. However, most of the recurrences are initially located inside the pleural cavity, and distant metastasis seems to be a late event in the evolution of the disease. Some recurrences can be extremely aggressive locally, leading to patients’ death by local invasion and compression without evidence of distant metastasis 26, 52. Our review of the literature has shown that the majority of the recurrences after resection of
Conclusions
Solitary fibrous tumor remains an enigmatic tumor. Recent evidence suggests that these tumors derive from long-lived “fibroblastic” stem cells and that successive mutations may lead to the malignant form. However, further analyses are required to determine more precisely the types of genetic anomalies associated with an increased risk of recurrence. From a review of the recent literature, we have defined the risk of recurrence of these tumors based on histologic and morphologic indicators, and
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