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Eur J Cardiothorac Surg 2001;19:185-189
© 2001 Elsevier Science NL

Solitary fibrous tumour of the pleura: surgical treatment

Department of Thoracic Surgery, S. Giovanni Battista Hospital, University of Torino, v. Genova 3, 10126 Torino, Italy

Received 5 July 2000; received in revised form 20 October 2000; accepted 15 November 2000.

Corresponding author. Tel.: +39-11-633-6635; fax: +39-11-696-0170
e-mail: pierluigifilosso{at}tiscalinet.it

	Abstract

Top Abstract 1. Introduction 2. Materials and methods 3. Results 4. Discussion References

 
Objective: Solitary fibrous tumours (SFT) of the pleura are rare tumours originated from the mesenchimal tissue underlying the mesothelial layer of the pleura. This tumours present unpredictable clinical course probably related to their histological and morphological characteristics. Methods: Twenty-one patients affected by SFT of the pleura were referred to us for surgical resection from September 1984 to April 2000. They were 15 males and six females with median age of 51 (range 15–73) years. Nine patients (43%) were symptomatic and predominant clinical symptoms or signs were dyspnoea (19%), coughing (14.3%), chest pain (28.5%), finger clubbing (14.3%) and hypoglycaemia (14.3%). Hypoglycaemia was related to a pathological incretion of insulin-like growth factor 2 by the tumour. Chest radiograph and computed tomography of the chest revealed intra-thoracic homogeneous sharply delineated round or lobulated mass sometimes associated with ipsilateral pleural effusion (19%) or causing pulmonary atelectasis with opacification of the complete hemithorax (19%). Surgical excision required 14 posterolateral thoracotomies, six anterior thoracotomies and one video-assisted thoracoscopy. Thirteen tumours arose from visceral pleura and wedge resection was performed, seven tumours arose from parietal pleura and extrapleural resection was carried out without any chest-wall resection, one tumour growth within the upper left lobe and required lobectomy. Tumours weighted from 22 to 1942 g and measured from 22x12x8 to 330x280x190 mm. At cut section seven cases (34%) revealed focal necrosis and hemorrhagic zones and on light microscopy six cases (28.5%) were characterized by high mitotic count: characteristics related with uncertain clinical behaviour. Immuno-histochemical reactions were in all cases positive for CD34. Results: In all our patients resections were complete. Paraneoplastic syndromes like hypoglycaemia and clubbing receded after surgery. No intraoperative or perioperative medical or surgical complications occurred. Median chest-drain duration timed 3 (range 2–5) days and median hospital stay was 5 (range 4–7) days. Perioperative mortality rate was 0%. Median follow-up was 68 (range 2–189) months: during this period patients were submitted to chest X-ray with 6-months interval to evaluate possible local recurrence. Only one patient experienced tumour recurrence after 124 months follow-up: the tumour was suspected after observation of finger clubbing. The tumour was detected and excised by redo-thoracotomy. Conclusions: Surgical resection of benign solitary fibrous tumours is usually curative, but local recurrences can occur years after seemingly adequate surgical treatment. Malignant solitary fibrous tumours generally have a poor prognosis. Clinical follow-up and radiological follow-up are indicated for both benign and malignant solitary fibrous tumours.

Key Words: Solitary fibrous tumours • Insulin-like growth factor 2 • Tumour • Pleura

	1. Introduction

Top Abstract 1. Introduction 2. Materials and methods 3. Results 4. Discussion References

 
Primary tumours of the pleura can be divided into two major categories: diffuse and solitary [1]. Diffuse pleural tumours are mesotheliomas. They are more common than solitary pleural tumours, arise from mesothelial tissue, are associated with asbestos exposure and almost have a fatal course [2].

Solitary tumours have been known by a variety of names that are indicative of their clinical course and controversies surrounding histogenesis.

The first solitary tumour of the pleura was described by Wagner in 1870 [3] and in 1931 Klemperer and Rabin [4] discovered a diffuse type of tumour that arose from mesothelial layer from a localized form that arose from the mesothelial fibrous connective tissue.

In 1952, Clagett et al. [5] used the term localized fibrous mesothelioma to distinguish these usually benign tumours from the more common asbestos related malignant mesothelioma. In the literature there is a great diversity in nomenclature. Some of these terms are localized mesothelioma, benign fibrous mesothelioma, benign localized fibroma, submesothelial fibroma [6]. To evade the discussion about the histogenesis the name solitary fibrous tumour (SFT) is advised [1]. These tumours are not related to asbestos exposure and they follow benign course in >80% of cases [6,7]. There is no relationship between solitary fibrous tumours and mesotheliomas [1,2]. In 1989 England et al. [8] reviewed a large series and found that surgical resection was the single most important indicator of good clinical outcome.

	2. Materials and methods

Top Abstract 1. Introduction 2. Materials and methods 3. Results 4. Discussion References

 
Between September 1984 and April 2000, 21 patients affected by SFT of the pleura were referred for surgical resection: 15 were men and six were women. Median age was 51 years (range 15–73). The tumour was right-sided in 14 patients (66.7%) and left-sided in seven (33.3%). Nine patients (43%) were symptomatic: when symptoms were present the predominant ones were dyspnoea (19%), coughing (14.3%), chest pain (28.5%). Extra-thoracic manifestation included arthritic pain and clubbing of the finger (14.3%) and hypoglicemia (14.3%).

Hypoglycaemia was associated with weight loss, night sweats, fatigue, episodes of unsteady gait and dysarthria. In these patients biochemistry revealed median serum glucose levels of 1.3 (range 1.1–1.4) mol/l, non detectable levels of insulin, median insulin-like growth factor (IGF) 1 levels of 10.6 (range 9.8–11.1) nmol/l and IGF 2 levels of 148.6 (range 136.2–154.7) nmol/l. Patients were referred to us with a diagnosis of pleural IGF 2 secreting tumour.

Chest radiograph revealed a sharply delineated round or lobulated mass in 16 patients (76%) (Fig. 1) associated with ipsilateral pleural effusion in four patients (19%). Pulmonary atelectasis with opacification of the complete hemithorax was observed in four cases (19%). In one case (4.7%) the tumour was ‘inverted’ and centrally located in the upper left lobe. Computed tomography (CT) (Fig. 2) confirmed data obtained from chest X-ray and excluded signs of invasion within the contiguous structures. Contrast enhancement was intense and homogeneous in 17 cases (81%) which was of limited dimension. Four cases of giant lesions (19%), diameters from 260x210x170 to 330x280x190 mm, had low density because of probably degeneration and localized necrosis. Bronchoscopic studies were performed in five patients (23.8%), being useful in the exclusion of other lesions when distortion of an intrinsically normal bronchial tree was present. Transthoracic fine needle aspiration biopsy (FNAB) was obtained in eight patients (38%): in three cases (37.5%) specimens revealed benign appearing spindle cells and this suggested diagnosis of benign pleural fibrous tumour; in five cases (62.5%) specimens were not significant.

View larger version (106K): [in this window] [in a new window]   Fig. 1. Chest X-ray demonstrating a large opacity involving the left inferior hemithorax.

View larger version (107K): [in this window] [in a new window]   Fig. 2. CT-scan of the thorax demonstrating a large well-delineated, disomogeneous mass compressing the left lung. No direct signs of chest wall invasion are seen.

Grossly, the tumours appeared polylobulated with smooth surface, weighted from 22 to 1942 g and measured from 22x12x8 to 330x280x190 mm. On cut section tumours showed a whorled, fleshy appearance, sometimes alternating with some myxoid areas. Focal necrosis and hemorrhagic zones were found in seven cases (34%), the largest in diameters. On light microscopy all the tumours reproduced the classic picture of solitary fibrous tumour of the pleura. Six cases (28.5%) were focally characterized by a mitotic count in excess of four mitosis per ten high-power fields or by cellular pleomorphysm. In eight patients (38%) gross or microscopic characteristics typical of malignant lesions were noted. Three of these tumours were pedunculated, one tumour (‘inverted’ and centrally located in the lung parenchyma) was resected by lobectomy, four cases were characterized by broad base of attachment on the diaphragmatic or parietal pleura and large extrapleural resections were required for complete exeresis. By immuno-histochemical analysis all tumours showed a positivity for vimentin and no expression of low molecular weight cytokeratin and desmin. The immuno-histochemical staining with CD34 (Fig. 3) (which helps to differentiate SFT from mesothelioma [9]) was positive in all patients.

View larger version (139K): [in this window] [in a new window]   Fig. 3. Immuno-histochemical CD34 antibody staining: high positivity of the cellular component is an essential pathologic feature for diagnosis of solitary fibrous tumour of the pleura (x40).

	3. Results

Top Abstract 1. Introduction 2. Materials and methods 3. Results 4. Discussion References

	4. Discussion

Top Abstract 1. Introduction 2. Materials and methods 3. Results 4. Discussion References

 
The incidence of SFT of the pleura is approximately 2.8 cases per 100 000 registered hospital patients [10]. SFTs represent <5% of all neoplasms involving the pleura [1]. Two thirds of these tumours arise from the visceral pleura and one third from the parietal pleura [1]. About 50% of patients are symptomatic and the most frequent symptoms, dyspnoea, coughing and chest pain are frequent in about 40% of patients. Extrathoracic manifestations include arthritic pain and clubbing of fingers, called hyperthrophic pulmonary osteoarthropaty (Pierre Marie-Bamberg syndrome) and hypoglycaemia [1,10,12–14].

Clubbing, hypertrophic osteoarthropaty and hypoglycaemia are not typically seen with mesothelioma, so the combination of a large pleural mass and one of these paraneoplastic syndromes should suggest diagnosis of SFT [1]. Hypoglycaemia is caused by tumour production IGF 2 [14–16].

IGF 2 may be produced by the tumour, and insulin and IGF 1 can be suppressed by IGF 2 and an abnormal IGF 1:IGF 2 ratio may indicate an IGF 2 secreting tumour. In our patients (14.3%) serum IGF 2 levels were high and serum glucose and insulin were preoperatively suppressed with typical signs of hypoglycaemia. Both these hormones and serum glucose returned to normal levels once the tumour was removed. As hypoglycaemia, clubbing and osteoarthropaty resolve after surgical resection of the SFT [11]. In one patient this paraneoplastic syndrome recurred 124 months after surgery: chest X-ray of the thorax were carried out and ipsilateral intrathoracic recurrence of the tumour was detected. The recurred tumour was excised by redo-thoracotomy and 3 months after the second operation clubbing receded.

The most important diagnostic test for these tumours is the chest roentgenogram. Chest X-ray can reveal solitary, circumscribed homogeneous lesions sometimes associated with pleural effusion and rare calcifications within the tumour. In CT scanning, solitary, well-circumscribed sometimes lobulated mass with the same density as the musculature can be seen. Contrast enhancement is usually intense and homogeneous as a result of the rich vascularization of the tumour. However, CT scan also may show low density (25–40 UH) and heterogeneous zones in large tumours that are due to necrosis, myxoid degeneration or hemorrhage within the tumour. Displacement of adjacent structures is characteristic. Sometimes they can be largely indovated within the lung parenchyma (one case in our series) and is often difficult to tell if the mass is pleural or pulmonary in nature.

Magnetic resonance imaging (MRI) is the most sensitive instrument to exclude invasion of neighboring structures. SFT is rarely diagnosed before surgical resection because cytology obtained by transthoracic needle aspiration is usually considered non-specific [17,18]. In our series eight patients (38%) underwent FNAB and in five patients (62.5%) was inconclusive because this tumour is composed of a-cellular and hyper-cellular portions. This makes it difficult to obtain representative materials for cytologic analysis. Moreover in 37.5% of cases aspiration material consisted of mesenchimal cells compatible with a SFT of the pleura. No malignant cells were observed on biopsy. A promising report of two cases in which diagnosis was obtained by FNAB (cytology) recently has been published [11]. Weygnard et al. [19] in 1997 reported five cases of SFT of the pleura in which diagnosis was performed by a large-bore Tru-Cut needle biopsy. Large specimens obtained allowed histological and immunochemical study with high preoperative diagnostic accuracy. It is important to note that these results are reachable if Tru-Cut needle biopsy is routinely performed to establish diagnosis of parietal, pleural or peripheral lung lesions with pleural contact as Weygnard reported [19].

When preoperative biopsy is not conclusive the definitive diagnosis is obtained by histology after surgical resection [10]. Until recently, immuno-histochemical analysis shows these tumours to be negative for keratin, S-100, and positive for vimentin: these characteristics were helpful but non diagnostic; the diagnosis of solitary fibrous tumour has become more precise since CD34 antibody staining was found to be positive in these tumours: it is now an essential pathologic feature for the diagnosis of SFT [9,11].

An important matter of discussion is the malignant potential of this tumour because it may recur locally and even metastasize to distant sites [19–21].

England et al. [8] have defined criteria of malignancy in a large study. These criteria include abundant cellularity, pleomorphism with cytonuclear atypia, more than four mitoses per ten high-power fields, large necrotic or hemorrhagic areas, associated pleural effusion, atypical location and invasion of adjacent structures. Using these criteria 12 to 33% of solitary fibrous tumours of the pleura were considered as malignant. This variant is rarely pedunculated and its size is usually greater than 8 cm. Although no other signs of malignity are observed, the finding of isolated pleomorphism in tumours greater than 8 cm represent an intermediate variant before frank malignancy. England and Okike [8,11] reported that fortunately not all of SFT considered as malignant behaved aggressively: nearly half patients with localized malignant SFT of the pleura were cured by surgery; nevertheless the other half succumbed to their tumour after experiencing local invasion, recurrence or metastasis. Since fibrous tumours of the pleura may rapidly enlarge and are potentially malignant surgical resection is recommended in all cases. Preoperative transthoracic needle biopsy is not necessary because it does not exclude malignant variants and does not influence the need of surgical resection. Care is taken to remove the whole mass. Although the tumour maybe well circumscribed, large resection of lung parenchyma and surrounding pleura is encouraged in particular for tumours presenting a broad base of attachement to the pleural surface. Sometimes tumours originated from the mesenchimal tissue of the parietal pleura stroma require extensive extrapleural resection for surgical exeresis. In rare cases intralobar development of the lesion requires large parenchyma ablation as lobectomy. Recurrence after complete resection is possible in malignant and in benign variants and so long-term annual follow-up with chest roentgenograms is highly recommended.

	References

Top Abstract 1. Introduction 2. Materials and methods 3. Results 4. Discussion References

 

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This Article Abstract Full Text (PDF) Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Add to Personal Folders Download to citation manager Author home page(s): Pier Luigi Filosso Giuliano Maggi Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Rena, O. Articles by Oliaro, A. Search for Related Content PubMed PubMed Citation Articles by Rena, O. Articles by Oliaro, A. Related Collections Pleura