Outcome of surgery in patients with solitary fibrous tumors of the pleura

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1 Original Article Outcome of surgery in patients with solitary fibrous tumors of the pleura Asian Cardiovascular & Thoracic Annals 2016, Vol. 24(1) ß The Author(s) 2015 Reprints and permissions: sagepub.co.uk/journalspermissions.nav DOI: / aan.sagepub.com Seyed Hossein Fattahi Masuom 1, Reza Bagheri 2, Ali Sadrizadeh 2, Marziyeh Nouri Dalouee 2, Ali Taherian 3 and Zahra Rajaie 4 Abstract Background: Solitary fibrous tumor of the pleura is rare, and the origin is submesothelial tissue. These tumors are seen in lung infrequently, and most are benign. We report our experience in the diagnosis and treatment of patients with solitary fibrous tumors of the pleura. Methods: We studied 13 patients (6 men and 7 women, aged 26 to 76 years) with a diagnosis of benign solitary fibrous tumor of the pleura who were operated on from 2001 to One had a recurrent tumor after 10 years, and the others had primary tumors. Our approach was complete resection in all cases. Results: After surgical excision, the most essential characteristic on histopathology was a neoplastic lesion composed of spindle-shaped tumor cells with hyperchromatic nuclei and eosinophilic bands of collagen. On immunohistochemical analysis, the cells were positive for desmin and negative for actin, synaptophysin, chromogranin, and CD117. Conclusions: The essential step in the treatment of a patient with a diagnosis of benign solitary fibrous tumor of the pleura is complete resection. These patients should be followed up for a long time because of the possibility of late recurrence. Due to the rarity of these tumors, there has been no systematic assessment of the role of adjuvant therapy for benign solitary fibrous tumor of the pleura. Keywords Pleural neoplasms, Recurrence, Solitary fibrous tumors Introduction Localized solitary fibrous tumors of the pleura (SFTP) can be malignant or benign. The cellular origin of these tumors is subserosal tissue as well as the mesothelial lining of the pleura. Most SFTP are pedunculated and originate from the visceral pleura that projects into the pleural space. Sessile and inward growth to parenchyma tissue of the lung is less common. These tumors can originate from a mediastinal, diaphragmatic, or intralobar fissure of the pleura. A localized fibrous tumor is almost always solitary and can be in a round or oval configuration; a membranous linear capsule is present in 50%. The tumor size can vary from very small (nodular) to a huge mass. The most common histological pattern is a patternless type; a mixed histological pattern and a hemangiopericytoma-like pattern are less common. Immunohistologically, this tumor is positive for vimentin and weakly positive for musclespecific actin. The tumor is also positive for CD34 antibody and bcl-2 oncoprotein. Lack of reaction to creatinine and a positive reaction to anti-cd34 and bcl-2 oncoprotein differentiate a localized SFTP from a desmoplastic mesothelioma. The most common symptoms are chronic cough, chest pain, and dyspnea; 1 Endoscopy Minimally Invasive Surgery Research Center, Ghaem Hospital, Faculty of Medicine, Mashhad University of Medical Sciences, 2 Cardiothoracic Surgery & Transplant Research Center, Emam Reza Hospital, Faculty of Medicine, Mashhad University of Medical Sciences, 3 Department of Pathology, Mashhad University of Medical Sciences, 4 Medical School, Mashhad University of Medical Sciences, Corresponding author: Marziyeh Nouri Dalouee, Cardiothoracic Surgery & Transplant Research Center, Emam Reza Hospital, Faculty of Medicine, Mashhad University of Medical Sciences,. lotfiane1@mums.ac.ir

2 Masuom et al % of patients become symptomatic. 1 In rare cases, galactorrhea, clubbing, hypoglycemia, and hypertrophic pulmonary osteoarthropathy had been reported. 1 3 Chest radiography and computed tomography (CT) are used for diagnosis. CT findings include heterogeneity in tumor density with Hounsfield units. Fine-needle aspiration is a valuable diagnostic method for peripheral lung tumors, and with immunohistological methods, a definite diagnosis can be achieved. Generally, a definite diagnosis can be obtained before surgery. Treatment of peripheral tumors includes segmentectomy, but if the tumor is in the parenchymal tissue of the lung, lobectomy is advised. Wide excision is performed for localized mediastinal, diaphragmatic, and parietal tumors of the pleura. For very large tumors, multiple lesions, inverted fibroma, and malignant lesions, excision is performed through a thoracotomy and the lesion should be removed completely. Other tumors may be excised by video-assisted thoracoscopic surgery if possible. 1 Patients and methods In this retrospective cross-sectional case series, patients with a diagnosis of SFTP, who were referred to Mashhad University of Medical Sciences between 2001 and 2014 were studied. We obtained personal and medical information from questionnaires of all patients who underwent thoracic surgery for SFTP. Information collection was performed with respect to the legal and cultural rights of the patients. The study involved 6 men and 7 women, with a mean age of years (range years). One patient had undergone orchiectomy due to germinoma 6 month earlier, one had an 8-year history of a lung mass that was enlarging gradually, and one had surgery for SFTP 10 years earlier. None had a history of smoking. The most common clinical symptom was chest discomfort (6 patients, 46/ 2%), 2 patients had dyspnea, 2 complained of left upper and lower quadrant pain, and one had hemoptysis. Four patients had chest pain and one had no symptoms (Table 1). All patients underwent a physical examination, routine blood tests, chest radiography, and thoracic CT. The chest radiograph of one patient is showed in Figure 1 and thoracic CT in the same patient is shown in Figure 2. Three cases were pathologically diagnosed preoperatively and confirmed by transthoracic biopsy. One patient had a history of SFTP, and in 3 cases, the diagnosis was confirmed by frozen section intraoperatively. Complete resection was carried out through an open thoracotomy in 12 patients, and in one patient, the tumor was excised by video-assisted thoracoscopic surgery. The tumor originated from the left hemithorax in 8 cases and from the right hemithorax in 5. Two tumors were sessile and the others were pedunculated with Table 1. Clinical symptoms, tumor location, diagnostic method, and surgical technique in 13 patients with solitary fibrous tumors of the pleura. Technique of surgery Diagnostic method One year follow-up Special disease history Location Clinical symptoms Sex Age (years) Frozen excision with margin Normal bronchoscopy Good Orchidectomy 6 months earlier Excision with margin Good Doubt due to complicated hydatid cyst Excision with margin Good Mediastinal tumor operated on 10 years earlier Left Lower and upper abdominal pain M 65 Left Pressure M 50 Left Dyspnea, pressure F 76 Excision with margin TTNB Good Right Pressure M 67 Excision with margin Good Left Pressure F 70 pericardium Excision with margin Not yet Right None M 68 completed Excision with margin Good Left Chest pain M 27 Excision with margin Good Right Cough, chest pain F 31 Lesion excision and lower lobe wedge resection Good Right Lower and upper abdominal pain M 35 Excision with margin Good Left Chest pain F 41 Excision with margin Not yet Right parietal Chest pain F 26 completed pleural VATS excision with margin TTNB-IHC Good Left Pressure F 41 Excision with margin TTNB-IHC Good Left Dyspnea, pressure F 59 IHC: immunohistochemistry; TTNB: transthoracic needle biopsy; VATS: video-assisted thoracoscopic surgery.

3 20 Asian Cardiovascular & Thoracic Annals 24(1) a plural base (11 visceral and one mediastinal pleura, and one from the parietal pleura). Studied variables included age, sex, symptoms, pulmonary location, surgery and sampling techniques, pathology findings, recurrence, and surgical complications. Data were analyzed by SPSS version 19 software Figure 1. Chest radiography showing a huge mass arising from the left hemithorax. (SPSS, Inc., Chicago, IL, USA). Frequency and percentage descriptive statistics were used for analysis. Results The tumors were resected completely, with part of the pleura in 11 patients with pedunculated tumors. One patient with a sessile tumor underwent pulmonary wedge resection and excision of the tumor. One patient had a tumor in the parietal pleura that was excised with endothoracic fascia. A pedunculated tumor is showed in Figure 3. The tumor sizes ranged from 2 to 20 cm, and the weights ranged from 100 to 2300 g. The most essential characteristic on histopathology was a neoplastic lesion composed of spindle-shaped tumor cells with hyperchromatic nuclei and eosinophilic bands of collagen. On immunohistochemical analysis, the cells were positive for desmin and negative for actin, synaptophysin, chromogranin, and CD117 (Figure 4). There was no morbidity or mortality in postoperative followup. There was one recurrence after 10 years; this patient had a pedunculated tumor that was reoperated on with complete resection. The tumor measured cm and weighed 2 kg. Figure 2. Computed tomography showing a mass arising from the left mediastinal pleura.

4 Masuom et al. 21 Figure 3. Intraoperative photograph of a pedunculated tumor. Figure 4. Histopathological examination showing a neoplastic lesion composed of spindle-shaped tumor cells, hyperchromatic nuclei, and eosinophilic bands of collagen. Discussion Solitary fibrous tumor of the pleura represents less than 5% of pleural tumors. 4 These tumors are commonly found in the thoracic (pleural) cavity (80%). Solitary fibrous tumor can affect a wide range of anatomic sites outside the pleura (20%), including the retroperitoneum, meningeal and nasal cavities. 5 SFTP is a rare tumor of mesenchymal origin, probably originating from submesothelial soft tissue. Although these tumors are usually benign, some may recur after surgery or metastasize, especially those with morphologic and histologically malignant behavior. 6 Among our 13 patients with SFTP, only one had a recurrence after 10 years. Although SFTP usually originates from the pleura, in rare cases, an intrapulmonary origin has been reported. Rao and colleagues 7 studied 24 cases of primary intrapulmonary solitary fibrous tumor, and Hideo and colleagues 8 found pulmonary involvement due to metastasis from a solitary fibrous tumor of the kidney to the liver and lung after 8 years. Sakellaridis and colleagues 9 reported a case of sigmoidal adenocarcinoma with a lung nodule at the same time; pathologic evaluation showed it to be a solitary fibrous tumor. 9 All of our patients lesions originated from the pleura: one from the mediastinal pleura, one from the parietal pleura, and the others from the visceral pleura. SFTP frequency is reported to be equally distributed in men and women and it presents in all age groups, but most often in the 5th to 8th decades of life. Our patients presented in 3rd to 7th decades of life, and the incidence was greater in females. Rao and colleagues 7 noted that the range of tumor sizes was 2.3 to 22 cm, similar to our findngs (2 to 20 cm). The clinical symptoms were cough, chest pain, and dyspnea. Symptoms such as clubbing, hemoptysis, hypertrophic osteoarthropathy, hypoglycemia, and seizure have also been reported. 1 3,9 There is no evidence of an association between SFTP and smoking or asbestosis. 9 Diagnostic modalities are chest radiography initially with confirmation by CT. A noninvasive lesion with a clear margin, calcified, and with strong enhancement is usually reported in CT scans. Due to the patternless histological pattern of this tumor, immunohistochemical testing is valuable for a definite diagnosis. All SFTP in our patients were positive for vimentin and CD34 but negative for creatinine, 10 and the most essential characteristic on histopathology was a neoplastic lesion with spindle-shaped tumor cells, hyperchromatic nuclei, and eosinophilic bands of collagen. On immunohistochemical analysis, the cells were positive for desmin and negative for actin, synaptophysin, chromogranin, and CD117. The imaging data from a study on ultrasonography as a diagnostic modality for

5 22 Asian Cardiovascular & Thoracic Annals 24(1) evaluating 3 patients by Sasaki and colleagues 11 showed hemi-cycle, extrapulmonary, homogenous, and hypoechoic lesions moving via respiratory movements, which confirmed pedunculation of the tumor. As reported by Pusiol and Scialpi, 12 CT for preoperative diagnosis of a benign solitary fibrous tumor was able to show that the tumor was pedunculate, which could be completely removed. CT evidence in a symptomatic patient with a non-pleural lesion pointed to a pathologically malignant tumor, thus CT may be superior to fine-needle aspiration. 12 The main treatment for SFTP is complete resection which can be performed by video-assisted thoracoscopic surgery for pedunculated tumors. To avoid tumoral cell transfer and metastasis, tumor connection with the port passages should be carefully prevented. Sessile tumors originating from the visceral pleura or tumors originating from the lung parenchyma need more extensive excision of pulmonary tissue to prevent local relapse of the tumor. To assure complete excision of the tumor, a frozen section sample must assessed. For sessile tumors originating from the chest wall, diaphragm, or mediastinal parietal pleura, extensive extrapleural excision should be performed. 13 Two patients with malignant solitary fibrous tumor were reported by Tapias-Vargas and colleagues; 6 one with a pulmonary lesion inside the chest wall underwent pulmonary wedge resection and chest wall excision, and the other with multiple pulmonary lesions underwent multiple wedge resections. 6 Benign solitary fibrous tumor prognosis is excellent if complete excision is performed, but strict follow-up is mandatory. Recurrence of 63% of malignant sessile, 14% of malignant pedunculated, 8% of benign sessile, and 2% of benign pedunculated tumors has been reported. 13 We concluded that SFTP is a rare disease, and in spite of its benign appearance, it can exhibit malignant behavior. The SFTP should be excised completely and the patient followed up over a long time because of the possibility of late recurrence. Although these tumors may be huge at presentation, in most cases, surgery can be carried out without any complications. Acknowledgments This paper has been extracted from a student s theses which supported and approved by cardiothoracic surgery and transplant research center, Mashhad University of Medical Sciences. We would like to thank Mrs. Elham Lotfian for her kind assistance in preparing the paper. Declaration of conflicting interests The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article. Funding The author(s) received no financial support for the research, authorship, and/or publication of this article. References 1. Tomas WS and AnjanaVY. Localized fibrous tumors of the pleura. In: Shields TW, Locicero J, Reed C, Feins R (eds) General Thoracic Surgery. Vol. 1, 7th ed. Philadelphia: Lippincott Williams and Wilkins, 2009, pp Valenzuela Membrives M, Cassini Go mez de Cádiz L, Cueto Ladro` n de Guevara A and Garcı a Pacheco M. Pleura solitary fibrous tumor associated with Doege- Potter syndrome. Pneumologie 2013; 67: Wang JL, Chang GC, Hsu JY, Hsia JY and Kwan PC. Solitary pulmonary fibrous tumor with initial presentation of hypoglycemia a case report and literature review. Thorac Med 2007; 22: (Available at: vol22410.htm. Accessed October 03, Harrison-Phipps KM, Nichols FC, Schleck CD, et al. Solitary fibrous tumor of the pleura: result of surgical treatment and long-term prognosis. J Thorac Cardiovasc Surg 2009; 138: Takizawa I, Saito T, Kitamura Y, et al. Primary solitary fibrous tumor (SFT) in the retroperitoneum. Urol Oncol 2008; 26: Tapias-Vargas LF, Santamarı a-ariza CM, Tapias- Vargas L and Tapias-Dı az L. Malignant solitary fibrous tumor of the pleura: report of two cases. Cir Cir 2012; 80: Rao N, Colby TV, Falconieri G, Cohen H, Moran CA and Suster S. Intrapulmonary solitary fibrous tumors: clinicopathologic and immunohistochemical study of 24 cases. Am J Surg Pathol 2013; 37: Sasaki H, Kurihara T, Katsuoka Y, et al. Distant metastasis from benign solitary fibrous tumor of the kidney. Case Rep Nephrol Urol 2013; 3: Sakellaridis T, Koukis I, Marouflidou T, Panagiotou I, Piyis A and Tsolakis K. Intrapulmonary solitary fibrous tumor masquerade sigmoid adenocarcinoma metastasis. Korean J Thorac Cardiovasc Surg 2013; 46: Barrettara B, Napoli G, Lacitignola A and Sardelli P. Fibrous lung tumor: a peculiar case. J Thorac Dis 2013; 5: Sekiya M, Yoshimi K, Muraki K, et al. Solitary fibrous tumor of the pleura: ultrasonographic imaging findings of 3 cases. Respir Investig 2013; 51: Pusiol T and Scialpi M. Role of computed tomography in the preoperative diagnosis of giant benign solitary fibrous tumor pleura. Lung India 2013; 30: Zhu Y, Du K, Ye X, Song D and Long D. Solitary fibrous tumors of pleura and lung: report of twelve cases. J Thorac Dis 2013; 5: