British Journal of Radiology (2006) 79, e36-e39
© 2006 British Institute of Radiology
doi: 10.1259/bjr/12749434
Epithelioid angiosarcoma of the lung: a rare late complication of Lucite plombage
D Patsios, BM BCh, MRCP, FRCR (UK)
1
M de Perrot, MD, FRCPC
2
M-S Tsao, MD, FRCPC
3 and
G Weisbrod, MD, FRCPC
1
Departments of 1Medical Imaging and 2Thoracic Surgery, University Health Network, Toronto General Hospital, 200 Elizabeth Street, Toronto, Ontario, M5G 2C4 and 3Department of Pathology, University Health Network, University of Toronto, Toronto, Ontario M5G 2M9, Canada
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Abstract
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Epithelioid angiosarcoma of the lung is a rare late complication of Lucite plombage treatment of pulmonary tuberculosis. We describe the clinical, radiological and pathologic findings of a case of epithelioid angiosarcoma of the lung presenting with persistent haemoptysis who had undergone remote lung collapse therapy with Lucite plombage.
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Introduction
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The discovery of effective antimicrobial therapy for the treatment of tuberculosis has effaced collapse therapy as the mainstream of therapy. Such therapy, until the 1950s, included artificial pneumothorax, phrenic nerve crush, thoracoplasty and extrapleural plombage. The latter therapeutic option has been associated with a number of late complications such as chronic pyothorax, migration of plombage, erosion of blood vessels and adjacent organs, and lung cancer [1, 2]. We describe an unusual case of an epithelioid angiosarcoma that developed in a collapsed lung, treated with Lucite plombage, presenting with persistent haemoptysis.
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Case report
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A 76-year-old woman presented with haemoptysis of half a cup a day. She was well until 3 weeks prior to presentation. Her medical history included right upper hemithorax plombage collapse for right upper lobe tuberculosis in 1952. There was no history of tobacco smoking. A chest radiograph showed deviation of the trachea to the left and narrowing of the lumen of the distal thoracic trachea secondary to mass effect from the right upper lung, which contained 16 air-filled well-defined plombage balls, some of which demonstrated an air fluid level. The collapsed right upper lobe demonstrated soft tissue opacity in between the Lucite plombage and caused bulging of the elevated right horizontal fissure and the right oblique fissure on the lateral view of the chest radiograph (Figure 1
). There was right upper chest wall rib deformity consistent with previous remote thoracic surgery and extrapleural plombage. A CT showed similar findings and confirmed the presence of soft tissue opacity between the Lucite plombage, causing mass effect with deviation of the trachea to the left, an effaced right upper lobe bronchus and bulging of the oblique fissure and horizontal fissure (Figure 2). The differential diagnosis included an empyema, erosion of one of the Lucite balls into the remaining right upper lobe and neoplasia of the right upper lobe.
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Figure 1. (a) The frontal radiograph demonstrated deviation of the trachea to the left, narrowing of the lumen of the distal thoracic trachea secondary to mass effect from the right upper lung, which contained multiple air filled well defined plombage balls, some of which demonstrated an air fluid level. The collapsed right upper lobe demonstrated soft tissue opacity in between the Lucite plombage. (b) The lateral view shows bulging of the elevated right horizontal fissure and the right oblique fissure.
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Figure 2. (a) The CT of the thorax confirmed the presence of a soft tissue opacity (big white block arrow) between the Lucite plombage, causing mass effect at the level of the right main bronchus (small white arrow) and (b) an effaced right upper lobe bronchus (black arrow) anterior to the right bronchus intermedius, which is consequently displaced caudally.
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Bronchoscopy demonstrated that bleeding was originating from the right upper lobe. The upper central airways were tortuous secondary to the pressure caused by the plombage cavity. The right upper lobe bronchus was extremely compressed and the orifice was narrowed to a few millimetres. There were no endobronchial lesions seen. Bronchial artery embolisation was attempted but failed.
Thoracotomy revealed a foul smelling large plombage cavity filled with 16 Lucite plombage balls (Figure 3) and fibrin. The right pleural cavity was therefore decorticated and the atelectatic right upper lobe was resected. No tumoural lesion was seen macroscopically at the time of surgery. The patient had an uneventful post-operative recovery, her haemoptysis stopped, and she left the hospital after 1 week with no symptoms. Unfortunately, the patient re-presented 1 month later with septic shock secondary to a right haemorrhagic empyema. A right thoracotomy and decortication were performed in emergency, but the patient died 48 h later from septic shock. No organism was grown from the right pleural cavity during the first or second operation.
Pathology
The resected right upper lobe showed thickened pleura that surrounded the lung parenchyma, which contained a haemorrhagic and necrotic tumour of 7.6 cm. Histological examination of the pleura and tumour edges demonstrated infiltrating clusters of epithelioid tumour cells, which also showed prominent permeation of the lumens of capillaries and venules (Figure 4a,b). Within or bordering the necrotic areas, tumour cells assumed a spindle cell appearance. Tumour cells demonstrated markedly pleomorphic nuclei with prominent nucleoli. Many tumour cells showed intracellular or intercellular vacuoles containing red blood cells. Their mitotic activity was brisk. Immunohistochemistry performed using routinely used diagnostic antibodies demonstrated negative staining of tumour cells for epithelial cell marker (AE1/AE3 and EMA), lymphohaematopoietic cell markers (CD45, CD3, CD5, CD30 and CD68), melanocytic marker (S100), but positive staining for vimentin (Figure 4c), CD31 (Figure 4d) and focally for Factor VIII. Tumour cells also stained negative for the mesothelial cell marker calretinin. The histological finding and immunophenotype were consistent with an epithelioid angiosarcoma. The peribronchial nodes were negative for tumour.
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Figure 4. (a) Tumour infiltrates the pleura and thickened fibrous tissue that encapsulated the necrotic lung parenchyma (haematoxylin and eosin, x50). (b) Tumour cells appear polygonal and epithelioid, and they infiltrate the blood vessel lumens (haematoxylin and eosin, magnification x100). (c) Immunohistochemistry show strong positive staining of tumour cells for vimentin (magnification x200). (d) Many tumour cells also show membranous staining for CD31, a marker of endothelial cells (magnification x200).
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Discussion
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Early spontaneous complications of plombage procedures include superinfection, migration of material and erosion of adjacent structures and broncho-extrapleural fistulae. In the long term, such complications become rare. Superinfection with Mycobacterium tuberculosis or common pyogenic bacteria has been the most frequent complication. The first sign is an acute onset of swelling of the collapsed space. The increased intracavitary tension leads to migration of the material, mechanical osteolysis of adjacent ribs, erosion of adjacent structures and fistula formation [1]. Patients may rarely experience pain because of the increased intracavitary pressure and haemoptysis if adjacent structures are eroded [2].
Malignant change induced by implanted foreign material has been documented in both experimental and clinical surgery [3]. Malignancy may occur rarely in plombage space or adjacent to plombage. Primary non-small cell bronchogenic carcinoma has been reported in non-smokers who had undergone Lucite plombage [4]. Foreign body reaction has been described as a contributing factor to the development of neoplasia in plombage cavities. Sarcomatous change such as malignant histiocytofibroma is recognized to be a specific complication of plombage [5].
Chronic infection itself has also been proposed as a predisposing factor for malignant fibrous histiocytoma and angiosarcoma [6–9]. The latter is a rare malignant tumour that affects various organs, mainly the skin, soft tissue, breast, liver, spleen and bone. Cases of angiosarcoma secondary to chronic pyothorax associated with tuberculosis have been described [6–9]. The calculated frequency rate of angiosarcoma associated with chronic pyothorax was 3600-fold higher than that of the general population [7]. Primary angiosarcoma of the pleura presents as a diffuse pleuropulmonary tumour macroscopically and microscopically simulating mesothelioma [7]. A history of pyothorax was involved in all cases of pleural angiosarcoma reported in a series of 26 cases in the literature [8]. However, it has been suggested that angiosarcoma associated with pyothorax seems to show the ordinary pathologic features of soft tissue angiosarcoma with no resemblance to primary pleural angiosarcoma [6].
Primary lung epithelioid angiosarcoma is a rare entity. Although it may present as a solitary lung mass [10], a unique case of epithelioid angiosarcoma arising in the lungs as bilateral multinodular infiltrates presented as pulmonary haemorrhage [11]. Pulmonary artery epithelioid angiosarcoma with pulmonary parenchymal invasion is also rare and has been reported to present with massive haemoptysis [12]. However, the majority of pulmonary angiosarcomas reported are usually due to secondary pulmonary involvement of soft tissue angiosarcomas [13].
The number of patients treated with plombage is attenuating rapidly and fewer patients will present in the future with late complications. Clinicians and radiologists will therefore have less experience. Epithelioid angiosarcoma should be included in the differential diagnosis, even though a rare entity, when non-smokers who had undergone remote Lucite plombage lung collapse therapy present with haemoptysis and have radiological demonstration of increased mass effect at the site of plombage, as illustrated in the case we present above.
Received for publication June 23, 2005.
Revision received August 29, 2005.
Accepted for publication September 2, 2005.
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References
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Abstract
Introduction
