대한흉부영상의학회 Weekly Chest Cases

Case No : 882 Date 2014-09-22

Courtesy of Shin Kyung Min, Jae Kwang Lim, Yang Bo Ra / KyungPook National University Hospital
Age/Sex 50 / M
Chief ComplaintHoarseness for 2 weeks

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Diagnosis With Brief Discussion

Diagnosis: Fibrosing Mediastinitis
Radiologic Findings: Chest PA shows widening of superior mediastinum.
Chest CT scan shows enhanced soft tissue attenuation mass surrounding left side of aortic arch, orifice of left carotid artery and subclavian artery.
PET CT shows increasd uptake in the left side of aortic arch(mSUV 4.2).

Mediastinoscopic biopsy reveals inflammatory fibrous tissue containing a few plasma cells and lymphocyte infiltration and that were not stained for IgG4.
Brief Review: Fibrosing mediastinitis (chronic sclerosing mediastinitis, granulomatous mediastinitis, or idiopathic mediastinal fibrosis) is a rare condition characterized by chronic inflammation and fibrosis of mediastinal soft tissues. The clinical, radiological and pathological presentation of fibrosing mediastitis within the mediastinum is highly compatible with the disease of IgG4 related disease in other organ such as the pancreas and the salivary glands. But the immunological characteristics are not consistent with IgG4 related disease in this case.
The exact pathogenesis of the mediastinal fibrosis is unclear, although tuberculosis, syphilis, histoplasmosis and blastomycosis have all been suggested as aetiological factors, as well as immune reactions, drugs, trauma and malignancy. 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
Most patients present with signs or symptoms related to obstruction or compression of vital mediastinal structures such as the central airways, superior vena cava, pulmonary veins, pulmonary arteries and esophagus. The most common presenting complaints include cough, dyspnea, recurrent pulmonary infection, hempotysis, and pleuritic chest pain.
Sherrick et al identified two patterns of involvement on CT scans: a focal pattern and a diffuse pattern. The focal pattern, seen in 82% of cases, manfests as a mass of soft-tissue attenuation that is frequently calcified (63% of cases) and is usually located in the fight paratracheal or subcarinal regions or in the hila. This type of fibrosing mediastinitis is, in all probability, caused by histoplasmosis in patients from the United States. The diffuse pattern, seen in 18% of cases, manifests as a diffusely infiltrating, noncalcified mass that affects multiple mediastinal compartments. The diffuse pattern is probaly not related to histopsmosis but often occurs in the setting of other idiopathic fibrosing disorders such as retoperitoneal fibrosis.
Fibrosing mediastinitis typically manifests on CT scans as an infiltrative mass of soft-tissue attenuation obliterating normal mediastinal fat planes and encasing or invading adjacent structures. Fibrosing mediastinitis most commonly affects the middle mediastinal compartments, including both the paratracheal and subcarinal regions, and the hila.
References: 1. Sherrick AD, Brown LR, Harms GF, Myers JL. The radiographic findings of fibrosing mediastinitis. Chest 1994; 106:484-489
2. Santiago E. Rossi, MD, H. Page McAdams, MD, Melissa L. Rosado-de-Christenson, Col, USAF, MC, Teri J. Franks, MD and Jeffrey R. Galvin, MD. Fibrosing Mediastinitis. Radiographics 2001; 21:737-757
3. Fraser RS, Muller NL, Colman N, Pare PD. Fraser and Pare's Diagnosis of diseases of the chest. 4th ed., W.B. Saunders company, Philadelphia. 1999:2856-2863
4. Rossi SE, McAdams HP, Rosado-de-Christenson ML, Franks TJ, Galvin JR. Fibrosing mediastinitis. Radiographics. 2001;21:737-757
Keywords: Mediastinum, Non-infectious inflammation,

No. of Applicants : 73

▶ Correct Answer : 10/73, 13.7%

- chp st martin , France Mariotte benoit
- McGill University Health Center , Canada Alexandre Semionov
- Kizawa Memorial Hospital , Japan Yo Kaneko
- Hanyang universitiy, radiology department , Korea (South) HyunGyu Lee
- Oita University, Faculty of Medicine , Japan Fumito Okada
- Asan Medical Center, Ulsan University , Korea (South) Mi Young Kim
- university of montreal , Canada Andrei Gorgos I
- IRSA La Rochelle , France Denis Chabassiere
- Fortis hospital , Mohali , India Shaleen Rana
- National Center hospital of Neurology and Psychiatry , Japan Kaoru Sumida

▶ Correct Answer as Differential Diagnosis : 13/73, 17.8%

- The University of Tokyo Hospital , Japan Akifumi Hagiwara
- Niigata City General Hospital , Japan Takao Kiguchi
- Avrasya Hospital , Turkey Murat Ulusoy
- The University of Tokyo Hospital , Japan Takeyuki Watadani
- Seirei Hamamatsu General Hospital , Japan Kenichi Mizuki
- Yokohama-asahi-chuo-general hospital , Japan Kyoko Nagai
- The University of Tokyo Hospital , Japan Toshihiro Furuta
- CNUH , Korea (South) Kim Soo Hyun
- sahyadri speciality hospital , India kothareddy dileepreddy
- DAYA General Hospital,Thrissur,Kerala , India Raveendran TK
- Pusan National University Yangan Hospital , Korea (South) yoonyoung choi
- SNUH , Korea (South) Eui Jin Hwang
- Medicheck health care , Korea (South) Chae Lim

▶ Semi-Correct Answer : 7/73, 9.6%

- Seoul National University Hospital , Korea (South) Sang Min Lee
- Shiga University of Medical Science , Japan Akitoshi Inoue
- Ibaraki-gazou-shindan , Japan Shoichi Katoh
- National Hospital Organization Okinawa Hospital , Japan Yasuji Oshiro
- Onomichi municipal hospital , Japan Hirofumi Mifune
- Kobe City Medical Center General Hospital , Japan Yasuhisa Kurata
- Pneumologia Universitaria, Policlinico di Bari , Italy Mario Damiani

대한흉부영상의학회 Korean Society of Thoracic Radiology

Archive of Old Cases

Case No : 882 Date 2014-09-22

Discussion

No. of Applicants : 73