solitary fibrous tumour of the pleura: a case...

Can Respir J Vol 17 No 3 May/June 2010 113

Solitary fibrous tumour of the pleura: A case report George Rakovich MD, Maxime Laflamme MD, Denise Ouellette MD, Gilles Beauchamp MD

Hôpital Maisonneuve-Rosemont, Université de Montréal, Montréal, QuébecCorrespondence: Dr George Rakovich, Division of Thoracic Surgery, Hôpital Maisonneuve-Rosemont, 5415 boulevard de l’Assomption, Montréal,

Québec H1T 2M4. Telephone 514-252-3400, fax 514-252-3894, e-mail [email protected]

Case presentationA 62-year-old woman presented with progressive exertional dyspnea. Her medical history included obesity, sleep apnea and type 2 diabetes. She was a nonsmoker with no history of asbes-tos exposure. On physical examination, her breath sounds were decreased on the right side; she did not have digital clubbing.

Routine laboratory investigations were normal. Her chest x-ray showed a large opacity in the lower one-half of the right lung field. Computed tomography (CT) imaging revealed a large solid mass in the right hemithorax measuring 25.5 cm × 17 cm × 13.5 cm. The mass was homogenous and well circumscribed but seemed to be adherent to the parietal pleura (Figure 1).

cASe report

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G rakovich, M Laflamme, D ouellette, G Beauchamp. solitary fibrous tumour of the pleura: a case report. Can respir J 2010;17(3):113-114.

Solitary fibrous tumours of the pleura are rare pleural neoplasms that are distinct from mesothelioma. Most of them are benign, although some behave aggressively; morphological and pathological features are important in distinguishing them from mesothelioma and in predicting clinical behaviour. Solitary fibrous tumours often grow to a large size before causing symptoms, and are characteristically associated with hypertrophic pulmo-nary osteoarthropathy in up to 20% of cases. In cases of benign lesions, complete resection is usually curative. A case involving a 62-year-old woman who underwent surgical resection of a solitary fibrous tumour of the pleura measuring 25 cm in size is described.

Key Words: Fibrous tumour; Pleura; Pulmonary osteoarthropathy

Une tumeur fibreuse solitaire de la plèvre : rapport de cas

Les tumeurs fibreuses solitaires de la plèvre sont de rares néoplasmes pleuraux différents des mésothéliomes. La plupart sont bénignes, même si certaines agissent de manière agressive. Les caractéristiques morphologiques et pathologiques sont importantes pour les distinguer des mésothéliomes et prédire leur comportement clinique. Les tumeurs fibreuses solitaires deviennent souvent très grosses avant de déclencher des symptômes et, d’ordinaire, elles s’associent généralement à une ostéoarthropatie pulmonaire hypertrophique dans jusqu’à 20 % des cas. Dans les cas de lésions bénignes, une résection complète est généralement curative. Est présenté le cas d’une femme de 62 ans qui a subi la résection chirurgicale d’une tumeur fibreuse solitaire de la plèvre mesurant 25 cm.

Figure 1) a Chest x-ray showing a large opacity in the lower one-half of the right lung field. B Computed tomography showing a large solid tumour in the right hemithorax

Rakovich et al

Can Respir J Vol 17 No 3 May/June 2010114

CT-guided core-needle biopsy was consistent with a solitary fibrous tumour of the pleura. The patient was taken to the oper-ating room and underwent a right posterolateral thoracotomy through the sixth intercostal space. Given the large size of the tumour, a counterincision in the eighth intercostal space was necessary for optimal exposure. The tumour was found to origin-ate from the right lower lobe on a pedicle of visceral pleura. It was widely adherent to the adjacent lung as well as to the dia-phragmatic and parietal pleurae. The adhesions were very dense and extremely vascular, and the dissection resulted in substantial oozing, with an estimated operative blood loss of 1200 mL. However, once mobilized, the tumour was easily resected by applying a linear stapler across the base of the pedicle (Figure 2). Postoperatively, the patient experienced some transient dyspnea, which subsequently resolved (a thorough workup did not reveal the underlying cause) and she was discharged in good condition on postoperative day 13. Final histological analysis confirmed the diagnosis of a benign solitary fibrous tumour (SFT) of the pleura; immunohistochemistry was positive for CD34.

DisCUssionSFTs are rare (1,2). Electron microscopy and immunohisto-chemistry have confirmed that they originate from mesenchymal cells in the submesothelial tissue of the pleura and not from mesothelial cells themselves, thus distinguishing them from mesothelioma (2,3). Although most SFTs are benign, approxi-mately 12% are malignant (3). Peak incidence occurs in the sixth and seventh decades of life (3), with both sexes equally affected (3). More than 50% of benign tumours are asymptom-atic (2), whereas most patients with malignant SFTs have symp-toms, with chronic cough, chest pain and dyspnea among the most common complaints (1,2,4). Interestingly, these tumours are associated with pulmonary osteoarthropathy, with or without digital clubbing, in up to 20% of cases (1,4), although the inci-dence reported in the literature varies widely (2). Hypoglycemia, believed to be due to the secretion of an insulin-like growth fac-tor, occurs in up to 5% of patients (2-4).

Benign tumours usually originate from the visceral pleura, are pedunculated and grow outwardly into the pleural space (1); malignant tumours tend to arise from the parietal pleura, diaphragmatic pleura or within a fissure, and grow into the lung (1). The presence of symptoms, pleural effusion and lack of a

pedicle have been shown to have a statistically significant asso-ciation with the likelihood of malignancy (2).

In the vast majority of patients, the tumours are large and readily identified on standard chest x-ray (2). A mobile mass may be demonstrated, suggestive of its pedunculated nature (1,3). CT scanning shows a homogenous, well-circumscribed lesion, but is nonspecific (2-4). SFTs can be difficult to distin-guish from tumours of the mediastinum and chest wall (1,5). Occasionally, the lesion may appear heterogenous on CT imaging because of hemorrhage or necrosis, making it difficult to distinguish from bronchogenic carcinoma (2,3). Magnetic resonance imaging may be helpful by revealing the characteris-tic fibrous nature of the tumour (2). Fluorodeoxyglucose-positron emission tomography may help identify SFTs with aggressive features, although the data are limited (6). Fine-needle aspiration may not yield the diagnosis in more than 50% of patients (2,4).

Histologically, SFTs exhibit a proliferation of uniform elon-gated spindle cells intimately intertwined with various amounts of connective tissue arranged in a haphazard distribution or ‘patternless pattern’ (1,3). CD34 is a specific marker that dif-ferentiates SFTs from mesothelioma (4). Criteria used to dif-ferentiate malignant from benign tumours include high mitotic activity, presence of necrosis and pleomorphism (2,4). However, these are not 100% reliable because in one review, 2% of patients with tumours categorized as benign on the basis of macroscopic and microscopic features succumbed to the disease (3). Complete resection with negative margin remains the treat-ment of choice in all cases (1-3); this may necessitate en bloc resection of the chest wall, pericardium or diaphragm in cases of invasion (2). There is no established role for conventional chemotherapy or radiotherapy (1,4); however, recent data on the genetic profile of SFTs suggests that they may respond to tyrosine kinase inhibitors (7,8). Video-assisted thoracoscopic resection may be feasible in selected cases, but port site metas-tasis has been reported, which may be due to attempts at tumour extraction through small incisions (3,4). Vascular adhesions to adjacent visceral or parietal pleurae are frequent and may bleed significantly (1,2); however, formal lobectomy is rarely necessary (1). The prognosis of benign lesions is excel-lent, although careful follow-up is necessary (1,4).

reFerenCes1. Shields TW, Yeldandi AV. Localized fibrous tumors of the pleura.

In: Shields TW, Locicero J III, Ponn RB, Rusch VW, eds. General Thoracic Surgery, 6th edn. Philadelphia: Lippincott Williams & Wilkins; 2005:889-900.

2. Magdeleinat P, Alifano M, Petino A, et al. Solitary fibrous tumors of the pleura: Clinical characteristics, surgical treatment and outcome. Eur J Cardiothorac Surg 2002;21:1087-93.

3. De Perrot M, Fischer S, Bründler MA, Sekine Y, Keshavjee S. Solitary fibrous tumors of the pleura. Ann Thorac Surg 2002;74:285-93.

4. Lu C, Ji Y, Shan F, Guo W, Ding J, Ge D. Solitary fibrous tumor of the pleura: An analysis of 13 cases. World J Surg 2008;32:1663-8.

5. Sung SH, Chang JW, Kim J, Lee KS, Han J, Park SI. Solitary fibrous tumor of the pleura: Surgical outcome and clinical course. Ann Thorac Surg 2005;79:303-7.

6. Hara M, Kume M, Oshima H, et al. F-18 FDG uptake in a malignant localized fibrous tumor of the pleura. J Thorac Imaging 2005;20:118-9.

7. Rossi G, Schirosi L, Giovanardi F, Sartori G, Paci M, Cavazza A. Pleural malignant solitary fibrous tumor with sarcomatous overgrowth showing PDGFRb mutation. Chest 2006;130:581-3.

8. George S, Merriam P, Maki RG, et al. Multicenter phase II trial of sunitinib in the treatment of nongastrointestinal stromal tumor sarcomas. J Clin Oncol 2009;27:3154-60.

Figure 2) Solitary fibrous tumour of the pleura: operative specimen. The tumour measured 25.5 cm × 17 cm × 13.5 cm and weighed 2 kg

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