Eur J Cardiothorac Surg 2004;26:453-455
© 2004 Elsevier Science NL
Fibrosing mediastinitis as a cause of superior vena cava syndrome
Simon Bays,
Chanaka Rajakaruna,
Ed Sheffield,
Anthony Morgan*
Department of Thoracic Surgery, Bristol Royal Infirmary, Marlborough Street, Bristol BS2 8HW, UK
Received 3 December 2003;
received in revised form 19 March 2004;
accepted 23 March 2004.
* Corresponding author. Tel.: +44-117-928-4211; fax: +44-117-928-3522
e-mail: anthony.morgan{at}ubht.swest.nhs.uk
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Abstract
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Fibrosing mediastinitis is a rare, chronic inflammatory process that can cause superior vena cava syndrome, and can mimic malignancy. We present two cases of this disease where surgical resection was not possible and review the treatment options.
Key Words: Fibrosing mediastinitis • Superior vena cava syndrome • Thoracic surgery
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1. Introduction
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Fibrosing mediastinitis is a benign, slowly progressing, inflammatory process causing sclerosis around any of the mediastinal structures, but most commonly the superior vena cava (SVC). The clinical picture can mimic that of a malignant process, which must be excluded. Histologically, it consists of abundant collagen containing cellular fascicles of fibroblasts associated with a chronic inflammatory infiltrate. We present two cases of fibrosing mediastinitis causing SVC obstruction, and discuss the surgical management.
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2. Patients
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2.1. Case 1
A 48-year-old lady presented with a cough and swollen, dilated veins of the face and upper extremities. The cough had been present for 2 years, but the venous obstruction had occurred over the course of a couple of weeks. She had normal lung function tests, and normal blood tests. At bronchoscopy, there was compression in the anterior segment of the right upper lobe, although there was no endobronchial lesion. A CT scan confirmed a mass in the mediastinum (Fig. 1)
, and a biopsy taken at the same time showed the presence of T and B lymphocytes, appearing more an inflammatory lesion than a neoplasm. A mediastinoscopy was normal, and a repeat CT guided biopsy gave the same result as before. A right anterior mediastinotomy showed a large mediastinal mass, and in view of previous diagnostic problems large biopsies (2 cmx2 cm) were taken under direct vision, as deep as the incision would allow. Histology showed dense hyaline collagen with blood vessels surrounded by a mild lymphocytic inflammation with no evidence of malignancy. Fibrosing mediastinitis was diagnosed. She was commenced on steroids for symptom palliation, which has halted the progression of the disease to date.
2.2. Case 2
A 60-year-old female was admitted with increasing shortness of breath over 3 years and dilated veins over her chest. A chest X-ray revealed a mass in the right upper mediastinum with compression and deviation of the trachea. A CT scan confirmed the presence of the mass, with central calcification, and occlusion of the brachiocephalic veins. The tumour extended around the arch of the aorta but did not invade it. Surgery was planned to resect the tumour and establish the diagnosis. At operation the tumour was found to be densely adherent to the aorta, proximal superior vena cava and brachiocephalic artery, and hence resection was abandoned. Two separate samples of the tissue were taken (approximately 2 cmx3 cm) as close to the centre of the mass as possible, and histological examination showed predominantly fibrofatty tissue with extensive lamellar hyalinised fibrosis. Dense collagen bundles were separated by focal areas of chronic non-specific inflammatory cells, with negative stains for fungi and acid-fast bacilli. There was no evidence of malignancy, and fibrosing mediastinitis was diagnosed. She was commenced on dexamethasone, which achieved symptom relief for several months until the dyspnoea became worse. At this point tamoxifen was added to her drug regime, which has considerably improved her symptoms.
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3. Discussion
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Fibrosing mediastinitis is a rare, chronic inflammatory process resulting in the deposition of dense fibrous tissue throughout the visceral compartment of the mediastinum [1] and is a disease process similar to retroperitoneal fibrosis. Aggregates of lymphocytes and plasma cells are found within a hyalinised fibrous connective tissue. The fibroblasts may show some proliferative activity. A venulitis is frequently present and elastic stains are useful in demonstrating this process. It is important to exclude an underlying neoplastic process, in particular Hodgkin's disease and a high-grade B-cell sclerosing mediastinal lymphoma. An adequate biopsy is necessary and immunochemistry is of great help in excluding these conditions.
The exact pathogenesis of the mediastinal fibrosis is unclear, although tuberculosis, syphilis, histoplasmosis and blastomycosis have all been suggested as aetiological factors [2], as well as immune reactions, drugs, trauma and malignancy.
In 40% of patients the disease is discovered incidentally, but the rest have symptoms of compression of mediastinal structures [1,3] the superior vena cava being the most common clinically, and it may mimic a malignant process. The disease is exceptionally rare in Europe, with most cases reported originating from the United States, where it is often associated with fungal infections, particularly histoplasmosis [1,3,4].
The indications for surgery are, in most cases, in order to establish diagnosis. In both reported cases, the initial likely clinical diagnosis was that of malignancy. Even in cases where the original biopsies reveal benign tissue, then malignancy would still have to be considered. In the first patient, this was the case and subsequent larger, more representative, biopsies were taken—all of these showed the same result, but the fact that the final one was taken at mediastinotomy meant that a larger sample was taken, and hence the histology was more accurate.
In cases where surgery is required to relieve severe compression syndromes, although reconstruction measures using allografts or prosthetic grafts are not currently routine procedures, superior vena cava obstruction can be relieved with resection and prosthetic replacement. The long-term patency with spiral vein grafts [5,6] externally stented as well as unsupported PTFE grafts are satisfactory to justify their use in patients with bad collateral venous circulation. Good symptomatic relief has also been reported after balloon angioplasty and stent insertion [7,8]. Resection of fibrotic tissue has been successful for tracheal stenosis with reconstitution of the tracheal lumen [9], although there is a high operative risk associated with tracheal resection. Where surgery is feasible, it appears to be curative in those cases not related to histoplasmosis.
Symptomatic tumours due to involvement of important mediastinal viscera often cannot be simply resected. Even in some severe compression syndromes surgery cannot be performed, as in case 2, as it would be too high an operative risk. Once the diagnosis has been obtained appropriate therapy can be commenced. Tamoxifen and steroids have been shown to lead to dramatic improvement in symptoms, although trials are necessary to judge long-term outcome [10]. In both of our cases reported the patients had some degree of symptomatic relief with dexamethasone, but case 2 did need further treatment with tamoxifen.
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References
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Top
Abstract
1. Introduction
