Case Study

Two cases of giant solitary fibrous tumor of the pleura: a not-so-rare tumor?

Asian Cardiovascular & Thoracic Annals 2014, Vol. 22(2) 226–228 ß The Author(s) 2013 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav DOI: 10.1177/0218492313478626 aan.sagepub.com

Marcello Migliore, Valerya Okateya, Damiano Calvo, Alessandra Criscione and Borrata Francesco

Abstract Solitary fibrous tumors of the pleura are rare intrathoracic neoplasms, especially in the giant form. The treatment of choice remains complete surgical resection which provides a benign clinical outcome. All patients need long-term followup due to the possibility of late recurrences. We describe 2 new cases of giant pleural fibrous tumor, which underwent resection via thoracotomy, and discuss the diagnostic and therapeutic modalities.

Keywords Diagnosis, differential, Pleural neoplasms, Solitary fibrous tumor, pleural, Thoracotomy

Introduction Solitary fibrous tumor of the pleura (SFTP) represents less than 5% of all pleural neoplasms. It arises from mesenchymal tissue underlying the mesothelial layer of the pleura.1 Most patients are asymptomatic, and the lesion is often discovered incidentally by routine chest radiography.2 The giant form of SFTP is extremely rare and the incidence is still unknown. The mainstay of therapy for SFTP is complete surgical resection with long-term follow-up.1,3

Case reports CASE 1

A 49-year-old man was admitted to our hospital with a 12-month history of chronic cough and dyspnea on exertion; no other signs or symptoms were present. Chest radiography demonstrated a homogenous density in the left lower chest field (Figure 1). Computed tomography (CT) revealed a huge tumor mass filling more than half of the left hemithorax, without radiological signs of local infiltration. Laboratory analysis including arterial oxygen tension, arterial carbon dioxide tension, and respiratory functional tests were normal. A CT-guided biopsy was performed, and a mesenchymal fibronectin tumor was diagnosed. The patient underwent surgery via a left posterolateral thoracotomy. The mass was identified as completely

encapsulated and attached to the atelectatic left lower lobe, aorta, and chest wall by means of adhesions. It arose from the visceral pleura of the same lobe through a single broad-based pedicle. The mass was removed, obtaining complete reexpansion of the lung. Macroscopically, the lesion was smooth surfaced and well circumscribed, measuring 21  15 cm and weighing 1400 g (Figure 2), with a significant blood supply. The cut section had a red-gray fibrous fascicular appearance with necrotic areas. Histologically, the tumor was composed of fascicles of spindle cells within collagenous stroma. Immunohistochemistry was negative for cytokeratin and BCL-2 and positive for vimentin and CD34. The final diagnosis was benign fibroma of the pleura with no signs of malignancy. The postoperative course was uneventful, and the patient was discharged home on the 7th postoperative day. Follow-up at 141 months, performed with CT and positron-emission tomography, showed no signs of recurrence.

Section of Thoracic Surgery, Department of Surgery, University of Catania, Catania, Italy Corresponding author: Marcello Migliore, MD, PhD, Section of Thoracic Surgery, Department of Surgery, University of Catania, Via S. Sofia, 95124, Catania, Italy. Email: [email protected]

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Figure 1. Chest radiograph showing a mass occupying more than half of the left hemithorax.

Figure 3. The mass in case 2, measuring 12  10  7 cm.

that of case 1. Follow-up at 10 months showed no signs of recurrence.

Discussion Figure 2. The mass in case 1, measuring 21  15 cm.

CASE 2

A 71-year-old asymptomatic woman was admitted with chest trauma sustained in a home accident. She presented with dyspnea and chest pain in the right hemithorax. Her past clinical history included only hypertension. A CT scan showed a large mass in the right hemithorax, measuring 10  8  12 cm with solid density and no signs of infiltration, pleural effusion, significant regional lymph nodes, or extrathoracic lesions. A diagnosis of benign solitary fibrous tumor of the pleura was considered. A right thoracotomy was carried out. The tumor appeared between the middle and lower right lobes, and was connected by a pedicle to the visceral pleura of the middle lobe. The mass was completely removed. Macroscopic examination confirmed a rounded well-defined mass of 12  10  7 cm, weighing 1050 g (Figure 3). Histology revealed the typical pattern of a benign solitary fibrous tumor of the pleura, without malignant morphologic aspects (hypercellularity, high mitotic index, tumor necrosis). The immunohistochemistry was similar to

Solitary fibrous tumor of the pleura is a rare form of pleural neoplasm; 80% arise from the visceral pleura and 20% from the parietal pleura.2 The majority of these tumors are benign, but approximately 20% fulfill the criteria for malignancy.4 The tumor may occur in any age group but the highest incidence is observed in the 5th and 7th decades of life.5 As in other tumors, there are no exact dimensional criteria to distinguish the SFTP as small or giant.6 Some reports have considered a tumor to be giant if its diameter is larger than 10 cm, while others considered only those tumors occupying at least 40% of the affected hemithorax to be giant.2,5,7,8 Although more than 50% of patients are asymptomatic and the tumor is discovered incidentally on chest radiography, a giant SFTP often presents with cough, dyspnea, chest pain, or hemoptysis.4 These symptoms are related to the size of the lesion and its compression of adjacent structures.9 Pulmonary atelectasis or complete lung collapse and contralateral mediastinal shift may occur with giant SFTP.4 Paraneoplastic manifestations have been described, such us hypertrophic osteoarthropathy (Pierre-MarieLambert syndrome), hypoglycemia due to the production of insulin-like growth factor II, galactorrhea, and digital clubbing.1,3,7

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Chest radiography and CT are the imaging studies of choice. The tumor appears as a huge lobulated mass, homogeneous with regular margins, or as a heterogeneous mass because of hemorrhage or necrosis.7 De Perrot and colleagues10 introduced a classification of SFTP, which includes 4 stages according to the presence of malignancy and multiple synchronous metastases. Comparing our cases with those reported in the literature, we noted that the dimensions of the masses did not correlate well with the weights; in fact, the reported weight of an SFTP of 20 cm ranges from 1400 (as in our cases) to 3 kg.9 We do not find any explanation for this difference in weight except for the fact that the presence of necrosis, hemorrhage, and calcification can modify the weight. Differential diagnoses include pleural mesothelioma, neurogenic sarcoma, monophasic synovial sarcoma, hemangiopericytoma, fibroblastic sarcoma, and malignant fibrous histiocytoma, as well as bronchogenic carcinoma, metastatic pulmonary carcinoma, and calcified fibrous pseudotumor of the pleura.5 There is a debate about the utility of CT-guided biopsy for definitive diagnosis because in most cases, biopsy is inconclusive.3 On the contrary, in our cases, CT-guided biopsy disclosed the mesenchymal chest tumors. Macroscopically, the SFTP appears as a firm, encapsulated, and lobulated mass with a whorled appearance on the cut surface. Immunohistochemistry is indispensable for differential diagnosis between SFTP and malignant tumors of the chest.5,10 Most SFTP are positive for CD 34, vimentin and Bcl-2.2.3 Nevertheless, the nature of the tumor is generally established on postoperative histological examination.11 The treatment of choice for giant SFTP remains complete surgical resection which provides a benign clinical outcome.5,9 In the era of minimally invasive surgery, it is clear that small SFTP can be operated on by video-assisted thoracoscopic surgery, but the giant type requires a thoracotomy or in some cases, a double thoracotomy.3 The role of adjuvant therapy has not been determined, and it is probably not useful.12 All patients affected by giant SFTP need long-term follow-up due to the possibility of late recurrences.1,3

Funding This research received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors.

Conflict of interest statement None declared.

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Two cases of giant solitary fibrous tumor of the pleura: a not-so-rare tumor?

Solitary fibrous tumors of the pleura are rare intrathoracic neoplasms, especially in the giant form. The treatment of choice remains complete surgica...
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