Eur J Cardiothorac Surg 1998;14:98-100
© 1998 Elsevier Science NL
Extrapericardial solitary fibrous tumour of the pericardium
Stefano Michele Andreania,
Luca Tavecchioa,
Roberto Giardinib,
Amedeo Vittorio Bedinia
a Department of Thoracic Surgery, National Cancer Institute, Milan, Italy
b Department of Pathology, National Cancer Institute, Milan, Italy
Received 13 October 1997;
received in revised form 23 March 1998;
accepted 15 April 1998.
Corresponding author. Divisione OCT, Istituto Nazionale Tumori, Via G. Venezian 1, 20133 Milan, Italy. Tel.: +39 2 2390367; fax: +39 2 2360486/76009499.
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Abstract
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Solitary fibrous tumour (SFT) occurs most commonly in the pleura and is extremely rare in the pericardium. The authors report a case of a 60-year-old man in whom a large mediastinal mass was accidentally discovered. Computed tomography showed involvement of the left anterosuperior mediastinum with displacement of the trachea, large vessels and oesophagus; histopathological findings after complete resection of the neoplasia demonstrated an SFT of the pericardium, the first reported case with extrapericardial pattern of growth. A review of the literature on SFTs of the pericardium is provided.
Key Words: Solitary fibrous tumour • Localized mesothelioma • Pericardial tumour
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Introduction
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Solitary fibrous tumour (SFT), so-called localized fibrous tumour or fibrous mesothelioma, was first described as a distinct entity in 1931 by Klemperer and Rabin
[1]. SFT is a rare disease: from 1942 about 700 cases of SFT were described, in 85–90% of cases it arose from pleura
[2], but it rarely occurred in other sites as mediastinum
[3], peritoneum
[2]
[4], retroperitoneum
[1] and lung
[2]. We present here the fifth reported case of SFT arising from pericardium
[4]
[5]
[6]
[7].
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Case report
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A 60-year-old man with no history of occupational exposure to asbestos was referred to our department for a left mediastinal mass accidentally detected on a routine pre-operative chest X-ray for herniated intervertebral disc.
On admission the patient had no complaints except for a slight exertional dyspnoea. Physical examination showed bilateral distension of the jugular veins with no evidence of a superior vena cava syndrome.
Chest X-ray showed a mass occupying the anterosuperior mediastinum and the left hemithorax, with compression and displacement of the trachea. On CT scan (
Fig. 1
) a large anterosuperior mediastinal mass displaced the oesophagus, the left common carotid and left succlavian artery and the trachea, whose lumen was reduced. Trans-thoracic fine needle aspiration biopsy detected a spindle cells tumour without evidence of malignancy.
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Fig. 1. CT scan shows a large mediastinal mass which dislocates the oesophagus, the vessels and the trachea.
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The patient underwent a median sternotomy: resection of the left innominate vein, which proved to be incorporated and inseparable from the tumour at its origin, was required.
The superior vena cava, the left common carotid and left succlavian artery and the trachea were carefully dissected. The left lung was atelettasic for the compression but not infiltrated; the left mediastinal pleura and residual thymic tissue were resected en bloc with pericardium, left vagus and phrenic nerve. No residual tumour was left at the end of the exeresis. A resternotomy was necessary for bleeding a few hours later; no further complications occurred in the post-operative period.
Four years later the patient is asymptomatic, with no evidence of local or distant recurrence.
Grossly, the neoplasia appeared as well circumscribed, oval, firm mass, of 20x18 cm in greater diameters and 1615 g in weight, with homogeneous, gray-white cut surface, with occasional cystic changes and no evidence of necrosis or haemorrhage. The mass arose from a portion of fibrous tissue referred by the surgeon as pericardium. Histologically the neoplasia was characterized mainly by a spindle cell proliferation, which was arranged haphazardly or as short fascicles, in a richly collagenous background (
Fig. 2 ). The neoplastic cells displayed vescicular nuclei with a sharply demarcated nuclear membrane, finely dispersed chromatin and inconspicuous nucleoli; the cytoplasm was scant, poorly defined, and slightly eosinophilic. Variably sized cyst-like spaces lined by spindle cells and cellular areas composed of closely packed spindled or oval cells with increased nuclear size were also present. Mitoses were rare, with an average of two per ten high-power fields.
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Fig. 2. (a) Tumour originating from pericardium (inked) (H&E x100). (b) Spindle cell proliferation without a peculiar histological pattern (H&E x250).
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Immunocytochemically the neoplastic cells showed diffuse cytoplasmic immunoreactivity to vimentin and CD34. Tumour cells were consistently negative for cytokeratin, epithelial membrane antigen, carcinoembryonic antigen, desmin, actin and S100 protein.
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Discussion
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SFT most often occurs in the pleura
[2], 80% in the visceral and 20% in the parietal pleura
[8]. Initially it was thought to be of mesothelial origin
[9]: in many papers it is also called fibrous or localized mesothelioma. On the other hand cases of SFT were reported at other sites and originating from other serous membranes such as the peritoneum
[4], the pericardium
[4]
[5]
[6]
[7] or from the mediastinum
[3]: this suggested that SFT could arise from submesothelial mesenchymal elements, from a primitive mesenchymal cell closely allied to the multipotential subserosal cell, as demonstrated by the immunoreactivity for vimentin but not keratin.
SFT can cause problems in clinical differential diagnosis and treatment: its clinical presentation is similar to that of other mediastinal tumours (thymoma, haemangiopericytoma, lymphoma). Three out of the four cases of SFT arising from the pericardium, described in the literature, showed location within the pericardial sac
[4]
[5]
[7] and of the last one we failed to find information about its pattern of growth
[6]. A case of SFT arising from the epicardium and growing in the pericardial cavity, was recently reported
[10]. The patient herein presented is the first case of SFT of the pericardium with extrapericardial growth.
Surgical resection of SFT can be hazardous, due to the usually large dimensions of these tumours and their connections with the great vessels, but it is usually the treatment of choice: because the clinical behaviour of the disease is unpredictable, complete excision of the tumour is always advisable, even if it implies sacrificing nerves or vessels. Local recurrence may also occur many years after the resection
[4]: a long follow-up is required to detect recurrent tumours.
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References
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- Klemperer P., Rabin C.B. Primary neoplasms of the pleura. A report of 5 cases. Arch Pathol 1931;1:383-412.
- Goodlad J.R., Fletcher C.D.M. Solitary fibrous tumour arising at unusual sites: analysis of a series. Histopathology 1991;19:515-522.[Medline]
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- El-Nagger A.K., Ro J.Y., Ayala A.G., Ward R., Ordonez N.G. Localized fibrous tumor of the serosal cavities. Immunohistochemical, electron-microscopic, and flow-cytometric DNA study. Am J Clin Pathol 1989;92:561-565.[Medline]
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- Roggli VL, Kolbeck J, Sanfilippo S, Shelburne JD. Pathology of human mesothelioma: etiologic and diagnostic considerations. In: Rosen PP, Fecher RE, editors. Pathology Annual, Part 2, Vol. 22. Norwalk: Appleton and Lange, 1989:91–131.
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Abstract
Introduction
