Tuberc Respir Dis.  2007 Oct;63(4):387-391.

Localized Fibrosing Mediastinitis with Superior Vena Caval(SVC) Syndrome

Affiliations
  • 1Department of Internal Medicine, National Health Insurance Corporation Ilsan Hospital, Koyang, Korea. kom2d@netsgo.com
  • 2Department of Thoracic surgery, National Health Insurance Corporation Ilsan Hospital, Koyang, Korea.
  • 3Department of Pathology, National Health Insurance Corporation Ilsan Hospital, Koyang, Korea.
  • 4Department of Radiology, National Health Insurance Corporation Ilsan Hospital, Koyang, Korea.
  • 5Department of Internal Medicine, Yonsei University College of Medicine, Seoul, Korea

Abstract

Fibrosing mediastinitis is a rare disease that is characterized by the proliferation of dense fibrous tissue of the mediastinum. The pathogenesis of fibrosing mediastinitis is unknown in most cases. However, histoplasmosis, tuberculosis, autoimmune disease, radiation therapy, and other idiopathic fibroinflammatory diseases have been implicated in some cases. Most clinical features are related to an obstruction or compression of the mediastinal structure. Fibrosing mediastinitis is often progressive and occurs diffusely throughout the mediastinum. We encountered a case of fibrosing mediastinitis of a very focal lesion without evidence of mediastinal involvement. The condition was confirmed by biopsy and graft bypass surgery was performed because of SVC syndrome.

Keyword

Fibrosing mediastinitis; Superior vena caval syndrome

MeSH Terms

Autoimmune Diseases
Biopsy
Histoplasmosis
Mediastinitis*
Mediastinum
Rare Diseases
Transplants
Tuberculosis

Figure

  • Figure 1 Engorged external jugular vein (A) and collateral vessels on the anterior chest wall and abdomen (B). After graft bypass, vein engorgements were improved (C).

  • Figure 2 Initial chest PA shows cicatricial atelectasis and multiple cavities on left upper lung.

  • Figure 3 In initial CT scans, about 3.5 × 2 cm sized ill defined infiltrative lesion was observed on SVC. SVC was totally obstructed by this lesion(arrow).

  • Figure 4 Focal dense fibrous tissue compressed SVC at the junction of right innominate vein (4A, arrow). Within right inominate vein and right atrium, a 10 mm Gore-tex® graft used to bypass the obstruction site (4B).

  • Figure 5 Dense collagenized fibrosis containing several chronic inflammatory cells and dark anthracotic pigmentation. Hematoxylin and Eosin stain. (5A: ×100, 5B: ×200).


Reference

1. Rossi SE, McAdams HP, Rosado-de-Christenson ML, Franks TJ, Galvin JR. Fibrosing mediastinitis. Radiographics. 2001. 21:737–757.
2. Loyd JE, Tillman BF, Atkinson JB, Des Prez RM. Mediastinal fibrosis complicating histoplasmosis. Medicine (Baltimore). 1988. 67:295–310.
3. Park SK, Jee HO, Park YK, Kim KH. Superior vena caval syndrome: report of a case. Korean J Thorac Cardiovasc Surg. 1979. 12:140–144.
4. Ko WO, Kim GH, Kim YS, Kim SW, Park SK, Lee DP, et al. A case case of chronic chronic sclerosing sclerosing mediastinitismediastinitis. Tuberc Respir Dis. 1995. 42:231–237.
5. Kim JH, Kim JH, Hur GY, Hur GY, Lee SH, Lee SHY, et al. Idiopathic Fibrosing fibrosing Mediastinitis mediastinitis Causing causing Extensive extensive Fibrotic fibrotic Venoveno- occlusion with Minimal minimal Mediastinal mediastinal Involvementinvolvement. Tuberc Respir Dis. 2002. 52:278–282.
6. Peebles RS, Carpenter CT, Dupont WD, Loyd JE. Mediastinal fibrosis is associated with human leukocyte antigen-A2. Chest. 2000. 117:482–485.
7. Baslaim G, deVarennes B. Localized idiopathic fibrosing mediastinitis as a cause of superior vena cava syndrome: a case report. Can J Surg. 1998. 41:68–71.
8. Flieder DB, Suster S, Moran CA. Idiopathic fibroinflammatory (fibrosing/sclerosing) lesions of the mediastinum: a study of 30 cases with emphasis on morphologic heterogeneity. Mod Pathol. 1999. 12:257–264.
9. Mole TM, Glover J, Sheppard MN. Sclerosing mediastinitis: a report on 18 cases. Thorax. 1995. 50:280–283.
10. Dunn EJ, Ulicny KS Jr, Wright CB, Gottesman L. Surgical implications of sclerosing mediastinitis. A report of six cases and review of the literature. Chest. 1990. 97:338–346.
11. Doty DB, Doty JR, Jones KW. Bypass of superior vena cava. Fifteen years' experience with spiral vein graft for obstruction of superior vena cava caused by benign disease. J Thorac Cardiovasc Surg. 1990. 99:889–895. discussion 95-6.
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