Ann Thorac Surg 2007;83:1199-1201
© 2007 The Society of Thoracic Surgeons
Case Reports
Successful Steroid Treatment for Fibrosing Mediastinitis and Sclerosing Cervicitis
Koei Ikeda, MD, PhD,
Hiroaki Nomori, MD, PhD*,
Takeshi Mori, MD,
Hironori Kobayashi, MD,
Kazunori Iwatani, MD,
Kentaro Yoshimoto, MD,
Masakazu Yoshioka, MD
Department of Thoracic Surgery, Graduate School of Medicine, Kumamoto University, Kumamoto, Japan
Accepted for publication September 5, 2006.
* Address correspondence to Dr Nomori, Department of Thoracic Surgery, Graduate School of Medicine, Kumamoto University, Honjo 1-1-1, Kumamoto 860-8556, Japan (Email: hnomori{at}qk9.so-net.ne.jp).
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Abstract
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The use of steroids to successfully treat a 75-year-old woman with fibrosing mediastinitis and sclerosing cervicitis causing a stricture of the left common carotid artery is reported. Biopsy specimens showed collagenous fibers and fibroblasts with moderate infiltration of lymphocytes. The mediastinal and neck lesions were significantly reduced, with almost complete resolution of arterial stricture, 3 months after initiating administration of prednisolone at 20 mg/d.
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Introduction
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It has been reported that steroids, immunosuppressive agents, and tamoxifen have been used in the medical treatment of fibrosing mediastinitis and similar kinds of disorders at other sites [1–4]. Although several reports have showed tamoxifen to be effective, the effectiveness of steroid therapy has been controversial. Here, we present a patient with fibrosing mediastinitis and sclerosing cervicitis causing a stricture of left common carotid artery, who was successfully treated with steroid therapy. A review of the English literature shows that this is the second case of the disease successfully treated by steroid therapy alone.
The patient was a 75-year-old woman. In June 2004, she had a right upper lobectomy for pathologic T1N0M0 lung adenocarcinoma when there were no abnormal findings in the mediastinum on computed tomography (CT). In June 2005, she complained of a mass situated at the left upper neck, which was not reduced by antibiotics. There were no other symptoms. A laboratory examination showed no findings of inflammation. A neck CT showed a mass 20 x 18 mm in size around the left common carotid artery. A chest CT also showed a mass 35 x 30 cm in size at the anterior mediastinum above the aortic arch, which caused a conspicuous stricture of the left common carotid artery (Fig 1A). Fluorine-18 fluoro-2-deoxy-D-glucose positron emission tomography (FDG-PET) revealed a high accumulation of FDG at the left neck and the mediastinum (Fig 2A).
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Fig 1. (A) Chest computed tomography at the first medical examination shows the anterior mediastinal mass stricturing the left common carotid artery. (B) After steroid therapy, the mediastinal mass is significantly reduced with almost complete resolution of the stricture of left common carotid artery.
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Fig 2. (A) Fluorodeoxyglucose (FDG) positron emission tomography at the first medical examination shows accumulation of FDG at the left neck and mediastinum. (B) After steroid therapy, the prior FDG accumulation at the left neck and mediastinum has disappeared.
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A surgical biopsy under general anesthesia showed a lesion firmly adhered to the left common carotid artery. The biopsy specimen showed collagenous fibers and fibroblasts with moderate infiltration of lymphocytes. Both the polymerase chain reaction and histologic stains showed neither fungi nor tuberculosis.
Because of the stricture of the left common carotid artery by the mediastinal lesion, the patient was treated with prednisolone (20 mg/d) after surgery. Three months after the initiation of steroid therapy, CT showed a conspicuous reduction in the size of the mediastinal lesion, with almost complete resolution of stricture of the left common carotid artery (Fig 1B). FDG-PET findings became negative for both the neck and mediastinum (Fig 2B).
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Comment
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Comings and colleagues [2] originally reported multifocal fibrosclerosis, including 5 disorders consisting of idiopathic fibrosing mediastinitis, Riedel’s thyroiditis, idiopathic retroperitoneal fibrosis, sclerosing cholangitis, and pseudotumor of the orbit. A similar disorder occurring in the neck has subsequently been reported and is referred to as sclerosing cervicitis [5]. Symptoms of these disorders are often progressive and cause pain or stricture of the mediastinal vessels and the esophagus. The cause of the disease has been reported to be histoplasma, mycobacterium, Nocardia, Hodgkin’s disease, sarcoidosis, autoimmune disorders, trauma, and prior surgery, but most cases of the disease are considered to be idiopathic [6].
Our patient had both the fibrosing mediastinitis and sclerosing cervicitis. Although our patient had a history of right upper lobectomy for lung cancer 1 year before the present disease developed, the disease occurred at the anterior mediastinum and the left neck, both of which were completely separate from the site of the prior surgery. We therefore believe that the fibrosing mediastinitis and sclerosing cervicitis in this patient were most probably idiopathic and not caused by the prior surgery.
It has been reported that steroids, immunosuppressive agents, and tamoxifen have been used in the medical treatment of fibrosing mediastinitis and similar kinds of disorders at other sites [1–4]. Several reports have shown tamoxifen to be effective, but the effectiveness of steroid therapy has been controversial. The effectiveness of tamoxifen was first reported by Clark and colleagues [3], who tried this drug in 2 patients with retroperitoneal fibrosis and found dramatic improvement in a small-bowel obstruction and obstructive ureter. Savelli and colleagues [7] reported a young woman with fibrosing mediastinitis and sclerosing cervicitis. She recovered after tamoxifen and steroid treatment, but had a recurrence after cessation of tamoxifen therapy. They therefore concluded that tamoxifen had more significant role in the treatment of the disease than steroids. Bays and colleagues [8] reported 2 patients with fibrosing mediastinitis who were treated with steroids. One patient recovered as a result of steroid-only treatment but the other patient needed tamoxifen as well to bring about an improvement in symptoms [8]. Our patient showed a significant reduction in the mediastinal and neck lesions with significant resolution of the stricture of the left common carotid artery by steroid therapy alone.
Although there have been several reports of surgical treatment for fibrosing mediastinitis where it caused strictures of the great vessels, esophagus, and airway [9], we believe that idiopathic fibrosing mediastinitis should first be treated by steroids or tamoxifen rather than by surgical treatment if the symptoms do not need immediate relief.
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References
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- Meredith SD, Madison J, Fechner RE, Levine PA. Cervical manifestations of fibrosing mediastinitis: a diagnostic and therapeutic dilemma Head Neck 1993;15:561-565.[Medline]
- Comings DE, Skubi KB, Van Eyes J, Motulsky AG. Familial multifocal fibrosclerosisFindings suggesting that retroperitoneal fibrosis, mediastinal fibrosis, sclerosing cholangitis, Riedel’s thyroiditis, and pseudotumor of the orbit may be different manifestations of a single disease. Ann Intern Med 1967;66:884-892.[Abstract/Free Full Text]
- Clark CP, Vanderpool D, Preskitt JT. The response of retroperitoneal fibrosis to tamoxifen Surgery 1991;109:502-506.[Medline]
- Ahsan N, Choudhury AA, Berger A. Retroperitoneal fibrosis Am Fam Physician 1990;41:1775-1780.[Medline]
- Rice DH, Batsakis JG, Coulthard SW. Sclerosing cervicitis: homologue of sclerosing retroperitonitis and mediastinitis Arch Surg 1975;110:120-122.[Abstract/Free Full Text]
- Dehner LP, Coffin CM. Idiopathic fibrosclerotic disorders and other inflammatory pseudotumors Semin Diagn Pathol 1998;15:161-173.[Medline]
- Savelli BA, Parshley M, Morganroth ML. Successful treatment of sclerosing cervicitis and fibrosing mediastinitis with tamoxifen Chest 1997;111:1137-1140.
- Bays S, Rajakaruna C, Sheffield E, Morgan A. Fibrosing mediastinitis as a cause of superior vena cava syndrome Eur J Cardiothorac Surg 2004;26:453-455.[Abstract/Free Full Text]
- Parish JM, Rosenow EC. Mediastinal granuloma and mediastinal fibrosis Semin Respir Crit Care Med 2002;23:135-143.[Medline]
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Abstract
Introduction
