Solitary Fibrous Tumors of the Pleura: Surgical Outcome and Clinical Course Sook Hwan Sung, Jee-Won Chang,

Jhingook Kim, Kyung Soo Lee, Jungho Han and Seung Il Park Ann Thorac Surg 2005;79:303-307

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The Annals of Thoracic Surgery is the official journal of The Society of Thoracic Surgeons and the Southern Thoracic Surgical Association. Copyright 2005 by The Society of Thoracic Surgeons. Print ISSN: 0003-4975; eISSN: 1552-6259.

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Sook Hwan Sung, MD, Jee-Won Chang, MD, Jhingook Kim, MD, Kyung Soo Lee, MD, Jungho Han, MD, and Seung Il Park, MD
Department of Thoracic Surgery, Seoul National University, Departments of Thoracic Surgery, Radiology, and Pathology, Samsung Medical Center, Sungkyunkwan University, and Department of Thoracic and Cardiovascular Surgery, Asan Medical Center, University of Ulsan, Seoul, South Korea

Background. The aim of this study was to dene more precisely the surgical outcome and clinical course of solitary brous tumors of the pleura. Methods. We conducted a retrospective review of the clinical records of patients who had undergone surgical resection for benign and malignant solitary brous tumors of the pleura during a 10-year period (1993 to 2003). Results. Sixty-three patients were enrolled in the study (men, 29; women, 34; mean age, 49.6 years). Thirty-six patients (57.1%) were symptomatic at the time of diagnosis. Resection was performed through a thoracotomy (n 37), by means of video-assisted thoracoscopy (n 22), or through a sternotomy (n 4). Mass excision only was performed in 34 cases, and en bloc excision including adjacent structures was performed in 29 cases. Forty-four

cases (69.8%) were benign and 19 (30.2%) were malignant. Local recurrences occurred in three cases and distant metastases in eight. Recurrences occurred only in malignancy. Symptomatic presentation and the impression of a nonpleural tumor by imaging study were found to be related to a malignant pathologic diagnosis. The radiologic impression of solitary brous tumors of the pleura was also related to mass excision only. Conclusions. For malignant cases, complete surgical resection may be insufcient for the cure. Therefore, further study should be performed to dene the role of preoperative and postoperative systemic treatment. (Ann Thorac Surg 2005;79:3037) 2005 by The Society of Thoracic Surgeons

olitary brous tumors of the pleura (SFTP) are rare neoplasms that usually originate from the visceral pleura. Since its pathologic characteristics was rst described by Klemperer and Rabin in 1931 [1], the nomenclature has become confused, and the disease has also been referred to as a localized mesothelioma, localized brous tumor, brous mesothelioma, or a pleural broma [2]. The further development of electron microscopy and immunohistochemistry has claried that the tumor does not originate from the mesothelial layer but from the submesothelial, noncommitted mesenchymal layer [3, 4]. Thus, the various names used for this disease have become unied, and the disease is now referred to as solitary or localized brous tumors of the pleura. Mesenchyme is pluripotent tissue and possesses diverse differentiation potential to bone, cartilage, or blood vessels. Because of this diversity of the mesenchyme, the pathologic morphology of SFTP appears variable [5]. Recent studies on SFTP have mainly involved immunologic markers for pathologic diagnosis [6, 7] and some clinical reports that include postsurgical resection results and description of its clinical behavior [8 10]. Although complete surgical resection of benign SFTP is the usual method of cure, occasional reports advise
Accepted for publication July 6, 2004. Address reprint requests to Dr Park, Department of Thoracic and Cardiovascular Surgery, Asan Medical Center, University of Ulsan, 388-1 Poongnap-dong, Songpa-gu, Seoul 138-736, South Korea; e-mail: sipark@amc.seoul.kr.

caution concerning its unpredictable clinical behavior such as its invasion of adjacent organs [11] or cardiac compression by the huge mass of benign SFTP [12]. Furthermore, the number of reported cases of malignant SFTP is too small to offer an overview, recurrence or survival are inconsistently reported, and no established treatment modality or follow-up plan has been agreed to. The aim of this study was to assess more precisely clinical behavior, surgical outcome, and the propriety of surgical techniques. This study also includes the determination of whether clinical or radiologic information can predict postoperative results.

Patients and Methods

We retrospectively reviewed the clinical records of patients who had undergone surgical resection for benign or malignant SFTP from 1993 to 2003. We reexamined pathologic slides, and malignant SFTP was diagnosed when one of following criteria was satised [10]; (1) increasing mitotic activity (4 mitoses per 10 high-power elds), (2) high cellularity with crowding and overlapping of nuclei, (3) presence of necrosis, or (4) pleomorphism. History taking, a physical examination, a routine blood test, standard chest radiography, electrocardiography, and a thoracic computed tomographic scan were available for all patients. Lung perfusion scans or echocardiographic results were available in selected cases. Recent patient status was determined by using the
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Table 1. Patient Characteristics

Age (mean, y) Sex Male Female Presenting symptoms Dyspnea Chest pain Cough Weight loss Hypertrophic osteoarthropathy Fever Asymptomatic Extent of resection Mass excision Mass excision with lung resection (wedge resection/lobectomy/pneumonectomy) Mass excision with resection of other intrathoracic structures (pericardium/ diaphragm/parietal pleura/chest wall) 49.6 29 (46%) 34 (54%) 16 11 5 2 1 1 27 34 21 8

GENERAL THORACIC

Fig 2. Chest computed tomographic scan of malignant solitary brous tumor of the pleura. This picture shows a very large mass shifting the mediastinum with loculated pleural effusion.

clinical records of outpatient clinics or by telephone interview. Operative mortality was dened as death within 30 days of an operation or during hospitalization. The continuity-corrected 2 test was used to compare percentages. Survival was analyzed with Kaplan-Meier survivor function estimates. A p value of less than 0.05 was considered statistically signicant.

Results
Sixty-three patients underwent surgical resection for a benign or malignant SFTP during the 10-year period. There were 29 men (46%) and 34 women (54%) of a mean

Fig 1. Survival after complete resection of solitary brous tumor of the pleura. Median survival of malignant (group M) solitary brous tumor of the pleura was 24 months. All of the patients with benign (group B) solitary brous tumor of the pleura were alive (p 0.0001). (fu follow-up.)

age of 49.6 years (range, 5 to 83 years). No patients had a history of asbestos exposure. Thirty-six patients (57.1%) were symptomatic at the time of diagnosis, and the most common symptom was dyspnea. One patient presented with hypertrophic osteoarthropathy, but no patient presented with symptomatic hypoglycemia (Table 1). The remainder of the patients were absolutely asymptomatic; tumors were incidentally found during standard chest radiography. The most favorable impression of the radiologist based on a thoracic computed tomographic scan was SFTP or a pleural mass in 36 patients (57%), and the remainder were impressions of a pulmonary mass (lung cancer, sclerosing hemangioma, angiolipoma) or mediastinal tumor (thymoma, neurogenic tumor, submucosal esophageal tumor). Preoperative pathologic diagnosis by neneedle aspiration (FNA) was tried in 35 patients (55.6%). Benign SFTP or brous tumor with benign nature was found in 15 cases, and malignant SFTP or malignant cell containing tumors that was not denitely diagnosed as malignant SFTP was found in 13. The remaining seven cases showed brosis, blood clots with brous tissue debris, or nonspecic inammation. Evaluation modalities for the follow-up included standard chest radiography, chest computed tomography, and additional abdominal or brain computed tomography. Resection was performed through a thoracotomy in 37 cases (58.7%), a video-assisted thoracoscopy in 22 cases (34.9%), and a sternotomy in 4 cases (6.4%). Mass excision only at its implantation was performed in 34 cases, but en bloc resection including surrounding structures was performed in the remainder owing to invasion or severe peritumoral inammatory adhesion (Table 1). Complete resection was performed in all cases except two. There was no operative morbidity or mortality. The mean follow-up time was 25.9 months. There were 44 (69.8%) pathologically benign SFTP cases, and 19 (30.2%)

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Table 2. Correlation Between Clinical Characteristic and Pathologic Type

Variable Average size (cm) Symptomatic patients Most favorable impression of radiologists (radiologists rst impression) SFTP or other pleural tumor (pleura-based tumor) Except SFTP or other pleural tumor (pleura-based tumor)
NS not signicant; SFTP solitary brous tumor of the pleura.

Benign SFTP 6.1 22 (50%) 24 (55%) 20 (45%)

Malignant SFTP 9 14 (75%) 5 (26%) 14 (74%)

p Value GENERAL THORACIC NS 0.012 NS 0.01

malignant SFTP cases. The mean follow-up time for benign SFTP was 29.8 months and that for malignant SFTP was 18.7 months (p 0.01). Local recurrence occurred in three cases. Two of 3 patients were treated by surgical reresection. Distant metastasis after curative resection occurred in eight cases; the metastatic sites were bone (n 2), brain (n 2), lung (n 2), and an intraabdominal lymph node (n 2). Eight patients died, and they all had malignant SFTP. All of their causes of death were cancer related. All of the benign SFTP patients were alive. Median survival of malignant SFTP patients was 24 months (Fig 1). To identify clinical or radiologic characteristics capable of predicting the pathologic type or clinical outcome, we examined differences in mass size, the proportion of symptomatic patients, and the most favorable impression of the radiologist based on chest computed tomography in malignant and benign cases (Fig 2, Table 2). No statistically signicant difference in mass size was observed between the two groups, although the mean size of the malignant group was larger. The proportion of symptomatic patients was higher in the malignant group (p 0.012). The radiologists impression of nonpleural tumor (ie, pulmonary mass, mediastinal mass) was more prevalent in the malignant SFTP group, and this was statistically signicant. Because decisions on operative approach were mainly determined by preoperative imaging studies in the majority of thoracotomy cases, we studied the correlation between the approach used and the radiologists impression; however, we found nothing of signicance (Table 3). When we compared cases that received mass excision only to those that received en bloc resection, the proportion of symptomatic patients was found to be higher in the en bloc resection group (Table 4). The number of cases giving the impression of a pleura including SFTP was greater among mass excision only cases.

decades, and no history of asbestos exposure are common points of the present study and in the literature. Solitary brous tumors of the pleura can present with various kinds of symptoms such as intrathoracic symptoms (dyspnea, chest pain, hemoptysis), systemic symptoms (hypoglycemia, hypertrophic osteoarthropathy), or nonspecic symptoms (fever, weight loss, fatigue). The proportion of symptomatic patients has been reported to be 50% to 60%, which is similar to the results of this study. As shown in Tables 3 and 4, the proportion of symptomatic patients is larger in cases of malignant SFTP and en bloc resection. Possible causes may be (1) irritation of adjacent structures by invasion or peritumoral adhesion, or (2) the paracrine action of unknown factors secreted by the SFTP. Moreover, presenting symptoms may help predict the clinical course after a thorough medical history taking and physical examination. Preoperative pathologic diagnosis (FNA) was performed in 55% of the enrolled patients. In the present study, according to the radiologists impressions, the FNA group was indistinguishable from the non-FNA group (patients who had undergone resection without preoperative pathologic diagnosis). Furthermore, the radiologists impressions based on imaging studies were of a benign or malignant pulmonary mass, thymoma, neurogenic tumors, esophageal submucosal tumor, or chronic empyema, all of which ultimately require surgical resection. Because surgical resection

Table 3. Correlation Between Radiologists Impression and Type of Operative Approacha

Impression Most favorable impression of radiologists (radiologists rst impression) SFTP or other pleural tumor (pleura-based tumor) Except SFTP or other pleural tumor (pleurabased tumor)
a

VATS (n 22) 64%

Thoracotomy (n 37) 57%

Comment
Solitary brous tumor of the pleura is a rare neoplasm that accounts for 8% of benign pathologic diseases of the chest and 10% of pleural neoplasms [1]. Its incidence is 2.8 per 100,000 registered hospital patients [13]. Approximately 800 cases of SFTP have been reported in the literature between 1931 and 2002 [2]. A slight female preponderance or an even sex distribution, greatest occurrence in the fourth to sixth

36%

43%

There was no signicant difference between surgical approach for either group. SFTP solitary brous tumor of the pleura; thoracoscopy. VATS video-assisted

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Table 4. Correlation Between Extent of Resection and Clinical Variables

Variable Size (cm) Symptomatic patients Most favorable impression of radiologists (radiologists rst impression) SFTP or other pleural tumor
NS not signicant; SFTP solitary brous tumor of the pleura.

Mass Excision Only 6.7 40.9% 63.6%

En Bloc Resection 6.9 73.7% 42.1%

p Value NS 0.01 0.01

GENERAL THORACIC

involves simultaneous diagnosis and treatment, preoperative FNA should not always be considered. Surgical resection for diagnosis and treatment is acceptable only if the patient is operable, because operative morbidity and mortality are very small. At least repeated FNA is not required even when a denitive preoperative pathologic result is not obtained. The choice of surgical approach is affected by location of tumors and by spatial relations in the imaging study rather than by the radiologists impressions (Table 3). In sternotomy cases, impressions on imaging study were thymoma (benign or invasive) or other anterior mediastinal mass. Therefore, the locations of tumors and the surgeons preference are considered more decisive than the radiologists impression in terms of the choice of the surgical approach. The radiologists impressions are considered related to surgical extent. The impressions of nonpleural tumor were more prevalent among en bloc resection cases. One of the possible causes of this nding may be the atypical images produced by a malignant SFTP showing invasion, severe peritumoral adhesion, necrosis, or hemorrhage. These atypical images differ from images of typical, well-circumscribed pleural tumor. If an SFTP originating from the visceral pleural fold at the interlobar ssure shows such atypical ndings, it may more resemble a malignant pulmonary mass than a pleural tumor [14, 15]. Considering the nding that the proportion of patients with the impression of a pleural tumor was higher in the mass excision only group, the radiologists impression strongly favoring SFTP could be a means of predicting surgical extent preoperatively. The reported incidence of malignant SFTP varies from 7% to 60%, a variation attributed to slight institutional pathologic criteria differences. Although some authors advocate that the number of mitoses is the most signicant criterion among the four criteria of malignant SFTP, useful immunohistochemical prognostic markers of malignant SFTP are still being investigated. Perrot and colleagues [2] classied SFTP as benign pedunculated, benign sessile, malignant pedunculated, and malignant sessile, ie, a classication on the basis of a combination of gross morphologic features and pathologic type. They reported signicant differences in the recurrences and survivals of these morphopathologic subtypes. Recurrences were reported to occur in 63% of

those in the malignant sessile group, in 14% in the malignant pedunculated group, in 8% in the benign sessile group, and in 2% of those in the benign pedunculated group. From the patients enrolled in the present study, morphopathologic information could be obtained in 45 cases; 18 were benign sessile cases, 16 benign pedunculated, 10 malignant sessile, and 1 malignant pedunculated. Local recurrence or distant metastasis occurred only in the malignant sessile type. Although the result is not absolutely the same as that of Perrot and associates [2], the pathologically malignant or the sessile forms are considered to be related to a poor prognosis. Prognostic signicance of this morphopathologic classication should be prospectively investigated in larger groups. The treatment of choice for benign SFTP is complete surgical resection. However, as far as malignant SFTP cases are concerned, there is no established systemic therapy, either preoperatively or postoperatively, despite the fact that malignant SFTP has shown distant metastasis [8, 16]. Because a small number of malignant SFTP patients showed survival times of more than 40 months in the present study, preoperative or postoperative systemic therapy should be considered in selected patients who are predicted to achieve a satisfactory result [17]. Thus further study should be performed to identify those factors affecting therapeutic response.

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GENERAL THORACIC

Solitary Fibrous Tumors of the Pleura: Surgical Outcome and Clinical Course Sook Hwan Sung, Jee-Won Chang, Jhingook Kim, Kyung Soo Lee, Jungho Han and Seung Il Park Ann Thorac Surg 2005;79:303-307
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