Review
Solitary fibrous tumors of the pleura

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Abstract

Solitary fibrous tumor of the pleura is a mesenchymal tumor that has been increasingly recognized over the past few years. The tumor was initially described in the pleura, but it has been reported in many other sites lately. Although the majority of these tumors have a benign course, the malignant form still remains enigmatic. Indeed, the behavior of these tumors is often unpredictable and does not always correlate with histologic findings. In addition, benign tumors may remain unproblematic for several years before changing into a malignant form. In order to define more precisely the clinical behavior of solitary fibrous tumors of the pleura, we reviewed the literature with particular attention to the clinical presentation, histopathologic characteristics, and cytogenetic differentiation of these tumors. A staging system and an algorithm for the management and follow-up of these patients are proposed.

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

References (89)

  • T Sakamoto et al.

    Localized pleural mesothelioma with elevation of high molecular weight insulin-like growth factor II and hypoglycemia

    Chest

    (1994)
  • E.G Theros et al.

    Pleural tumors and pulmonary tumorsdifferential diagnosis

    Semin Roentgenol

    (1977)
  • D.S Mendelson et al.

    Localized fibrous pleural mesotheliomaCT findings

    Clin Imaging

    (1991)
  • M.I Lewis et al.

    The case of the moving intrathoracic mass

    Chest

    (1985)
  • S Fischer et al.

    Giant bronchial carcinoid tumorsa multidisciplinary approach

    Ann Thorac Surg

    (2001)
  • C.A Hanau et al.

    Solitary fibrous tumorhistological and immunohistochemical spectrum of benign and malignant variants presenting at different sites

    Hum Pathol

    (1995)
  • J.R Utley et al.

    Recurrent benign fibrous mesothelioma of the pleura

    J Thorac Cardiovasc Surg

    (1973)
  • F Imamura et al.

    Primary primitive neuroectodermal tumor of the lungreport of two cases

    Lung Cancer

    (2000)
  • P Dal Cin et al.

    Trisomy 21 in solitary fibrous tumor

    Cancer Genet Cytogenet

    (1996)
  • Y.L Chang et al.

    Thoracic solitary fibrous tumorclinical and pathological diversity

    Lung Cancer

    (1999)
  • T.X Aufiero et al.

    Intrapulmonary benign fibrous tumor of the pleura

    J Thorac Cardiovasc Surg

    (1995)
  • N Martini et al.

    Pleural mesothelioma

    Ann Thorac Surg

    (1987)
  • P.A Dervan et al.

    Solitary (localized) fibrous mesotheliomaevidence against mesothelial cell origin

    Histopathology

    (1986)
  • A.K El-Naggar et al.

    Localized fibrous tumor of the serosal cavitiesimmunohistochemical, electron-microscopic, and flow-cytometric DNA study

    Am J Clin Pathol

    (1989)
  • M van de Rijn et al.

    Expression of CD34 by solitary fibrous tumors of the pleura, mediastinum, and lung

    Am J Surg Pathol

    (1994)
  • J.K.C Chan

    Solitary fibrous tumour—everywhere, and a diagnosis in vogue

    Histopathology

    (1997)
  • D.M England et al.

    Localized benign and malignant fibrous tumors of the pleuraa clinicopathologic review of 223 cases

    Am J Surg Pathol

    (1989)
  • M Briselli et al.

    Solitary fibrous tumors of the pleuraeight new cases and review of 360 cases in the literature

    Cancer

    (1981)
  • T Hasegawa et al.

    Solitary fibrous tumor of the soft tissuean immunohistochemical and ultrastructural study

    Am J Clin Pathol

    (1996)
  • A.V Vallat-Decouvelaere et al.

    Atypical and malignant solitary fibrous tumors in extrathoracic locationsevidence of their comparability to intra-thoracic tumors

    Am J Surg Pathol

    (1998)
  • E Wagner

    Das tuberkelahnliche Lymphadenom (Der cytogene oder reticulirte Tuberkel)

    Arch Heilk (Leipzig)

    (1870)
  • P Klemperer et al.

    Primary neoplasm of the pleuraa report of five cases

    Arch Pathol

    (1931)
  • A.P Stout et al.

    Localized pleural mesothelioma

    Arch Pathol

    (1942)
  • E.A Foster et al.

    Localized mesotheliomas of the pleura

    Am J Clin Pathol

    (1960)
  • S.A Luse et al.

    An electron microscopic study of solitary pleural mesothelioma

    Cancer

    (1964)
  • D Scharifker et al.

    Localized fibrous mesothelioma of pleura (submesothelial fibroma)a clinicopathologic study of 18 cases

    Cancer

    (1979)
  • F.J Hernandez et al.

    Localized fibrous tumors of pleuraa light and electron microscopic study

    Cancer

    (1974)
  • M Al-Azzi et al.

    Pleural mesothelioma of connective tissue type, localized fibrous tumor of the pleura, and reactive submesothelial hyperplasiaan immunohistochemical comparison

    J Pathol

    (1989)
  • S.W Weiss et al.

    CD34 is expressed by a distinctive cell population in peripheral nerve, nerve sheath tumours, and related lesions

    Am J Surg Pathol

    (1993)
  • J Nickoloff

    The human progenitor cell antigen (CD34) is localized on endothelial cells, dermal dendritic cells, and perifollicular cells in formalin-fixed normal skin, and on proliferating endothelial cells and stromal spindle shaped cells in Kaposi’s sarcoma

    Arch Dermatol

    (1991)
  • M van de Rijn et al.

    CD34, a review

    Appl Immunohistochem

    (1994)
  • M Suter et al.

    Localized fibrous tumours of the pleura15 new cases and review of the literature

    Eur J Cardiothorac Surg

    (1998)
  • O Rena et al.

    Solitary fibrous tumour of the pleurasurgical treatment

    Eur J Cardiothorac Surg

    (2001)
  • H Chaugle et al.

    Hypoglycemia associated with a solitary fibrous tumour of the pleura

    Eur J Cardiothorac Surg

    (1999)
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