대한흉부영상의학회 Weekly Chest Cases

Case No : 509 Date 2007-07-30

Courtesy of Yang Sin Park, MD, Yu Mi Jang, MD, Eun Jin Chae, MD, Kyung-Hyun Do, MD. / Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
Age/Sex 54 / F
Chief ComplaintDyspnea (1 MA)

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Diagnosis With Brief Discussion

Diagnosis: Systemic Amyloidosis
Radiologic Findings: Chest radiograph shows prominent both hilum and mild cardiomegaly with LV hypertrophy but no abnormal parenchymal opacity in both lung fields.
Enhanced chest CT scans with mediastinal window setting show multiple enlarged lymph nodes in both axillary area and calcified lymph nodes in mediastinum. Chest CT with lung window setting shows no abnormal parenchymal lesion.
Needle aspiration biopsy was performed at axillary lymph node. A polarized micrograph shows apple-green bifringence of amyloid deposition.
His laboratory findings show monoclonal gammopathy of serum, urine, and bone marrow, he was diagnosed as multiple myeloma. Echocardiographic findings suggested infiltrative disease such as amyloidosis and chest CT with concentric low density along the endocardium supported cardiac involvement of systemic amyloidosis.
Brief Review: Systemic disease is due to the deposition of mainly (92%) AL proteins in multiple organs. Respiratory tract involvement is common but rarely produces symptoms of clinical importance, and may be difficult to distinguish from symptoms due to cardiac involvement. Prognosis is poor, with a 5 year survival rate of, 20%, and is worse in those cases associated with multiple myeloma. Systemic amyloidosis is diagnosed by positive staining (with Congo Red) of abdominal fat samples (positive in 80%), bone marrow aspirate (50% positive), or rectal biopsy (80% positive).
Both mediastinal and hilar lymphadenopathy are common in AL-type systemic amyloidosis (75% of patients in the Mayo Clinic series), but rare in localized disease. It is usually associated with parenchymal disease (50%) but may be a solitary finding, and calcification may be present.
It may be possible to distinguish between systemic and localized respiratory tract amyloidosis based on the thoracic CT findings alone. Lymphadenopathy and the diffuse alveolar septal pattern of parenchymal disease both suggest systemic disease, as they are rare in the localized form.
The heart is the most commonly involved organ in the chest, usually caused by the AL subtypes, rarely by the AA form. Virtually all parts of the heart can be involved, including not only the myocardium, the interatrial septum and atrial walls but also the valve leaflets. Increased thickness of the myocardial wall, caused by increased interstitial amyloidal deposition, leads to systolic and diastolic dysfunction, and cardiac arrhythmias. Although cardiac biopsy can reliably diagnose cardiac amyloidosis, its procedural risks and uncertainty about sampling errors limit its applications. Currently cardiac ultrasound is considered the non-invasive test of choice to diagnose cardiac amyloidosis. Due to the amyloidal deposition, the myocardium has a granular or speckled appearance on cardiac ultrasound. Recently, several groups, using the inversion-recovery contrast-enhanced MRI technique, have described patterns of myocardial enhancement that seem to be unique for cardiac amyloidosis. Maceira and co-workers described a “zebra pattern” consisting of a strong subendocardial and subepicardial late enhancement, involving both the right and left ventricle.
References: 1. Aylwin AC, Gishen P, Copley SJ. Imaging appearance of thoracic amyloidosis. J Thorac Imaging. 2005;20:41-6.
2. Georgiades CS, Neyman EG, et al. Amyloidosis: review and CT manifestations. Radiographics. 2004;24:405-16.
3. Van Geluwe F, Dymarkowski S, et al. Amyloidosis of the heart and respiratory system. Eur Radiol. 2006;16:2358-65.
Keywords: Multiple organ, Mediastinum, Metabolic and storage lung disesae, Amyloidosis,

No. of Applicants : 41

▶ Correct Answer : 6/41, 14.6%

- Cotia Saede-Seo Paulo, Brazil Jose Luiz Masson
- Osaka University, Japan Osamu Honda
- China Medical University Hospital, Taiwan Jun-Jun Yeh
- Dept. of Radiodiagnosis and Imaging, PGIMER, Chandigarh, India Ram Prakash Galwa
- Dept. of Radiology, Seoul National University Hospital, Korea Kwang Nam Jin
- CHU St Etienne hospital, France Romain Malezieux

▶ Correct Answer as Differential Diagnosis : 12/41, 29.3%

- Annemasse, Polyclinique De Savoie, France Gay-Depassier Philippe
- Vital imaging centre, Mumbai, India Ganesh Agrawal
- Hospital Sotiria, Athens, Greece Vasilios Tzilas
- Mackay Memorial Hospital, Taipei, Taiwan Chia-Fu Tsai
- IRSA La Rochelle, France Jean-Luc BIGOT
- Hangang Sacred Heart Hospital, Korea Eil Seong Lee
- Yongsan Hospital, College of Medicine Chung-Ang University, Korea Jae Seung Seo
- Hospital Sotiria Athens Greece Delaveri kaith
- IRSA, La Rochelle, France Denis Chabassiere
- Ultracare Coimbatore, India Debabrata Das
- Armidale Radiology, Australia Saurabh Khandelwal
- Pittwater Radiology, Australia Julie Arora

▶ Semi-Correct Answer : 18/41, 43.9%

- Dept. of radiology, Inje University Ilsan Paik Hospital, Korea Bae Geun Oh
- Regional Imaging Riverina, Australia Rashid Hashmi
- MA Diagnostic Center, Guntur, India Sunil Almale
- Registrar in Radiology, UHL NHS Trust, UK A. Donuru
- CHU, Grenoble, France MORONI Anne-Line
- Mumbai , India Rahul B Deshmukh
- NUH, nottingham, UK Rohit Kochhar
- Aarthi Advanced CT and MRI scans, Tirunelveli, India Sivasubramanian Srinivasan
- Pohang Naval Hospital, Korea Jinoo Kim
- Jiangsu Province Official Hospital, Nanjing, Jiangsu Province, China Dong Sheng Jin
- Homs National Hospital, Homs - Syria Rami Abou Zalaf
- KAUMS, Kashan, Iran Ebrahim Razi
- Annecy hospital, France Gilles Genin
- Jackson Memorial Hospital, Miami, Florida, USA N.B.S. Mani
- Soonchunhyang University hospital, chunan, Korea Sang-Hyun Park
- Max Hospital, New Delhi, India Vickrant Malhotra
- E-Da hospital, Taiwan Yu-Feng Wei
- CHU Grenoble, France Bing Fabrice

대한흉부영상의학회 Korean Society of Thoracic Radiology

Archive of Old Cases

Case No : 509 Date 2007-07-30

Discussion

No. of Applicants : 41