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Weekly Chest CasesArchive of Old Cases

Case No : 985 Date 2016-09-12

  • Courtesy of Kum Ju Chae, Soon Ho Yoon, Hyun Ju Lee, Jin Mo Goo / Seoul National University Hospital
  • Age/Sex 53 / M
  • Chief ComplaintIncidental abnormality found on chest CT
  • Figure 1
  • Figure 2
  • Figure 3

Diagnosis With Brief Discussion

Diagnosis
Amyloidosis
Radiologic Findings
Contrast-enhanced chest CT demonstrates a soft tissue mass with multiple nodular calcifications in the anterior mediastinum. The patient has a prior diagnosis of a monoclonal gammopathy of undetermined significance on the immunoelectrophoresis. PET-CT shows mild FDG uptake in the anterior mediastinum (SUV max 2.87). Robot-assisted total thymectomy was performed for making a confirming the diagnosis. The Congo-red stained specimen showed the apple-green birefringence under polarizing microscopy. The pathologic diagnosis of the biopsy specimen demonstrated amyloidosis.

Congo-red stain

Bipolarized microscopy

Brief Review
Amyloidosis is a disease characterized by the abnormal extracellular deposition and accumulation of protein called amyloid accumulates. Clinical and radiologic manifestations of amyloidosis are variable and often nonspecific. Amyloidosis can be classified on the basis of its anatomic distribution into systemic (involving multiple organ systems) or localized (involving a single organ such as the lungs or the heart). It can also be classified on the basis of the structure of the protein deposits. The fibrillar proteins amyloid light chain (AL) and serum amyloid A (AA) are present in the vast majority of cases. Although thoracic involvement of amyloidosis is relatively rare, lungs, mediastinum, pleura, and heart may be involved in amyloidosis. The isolated tumefactive lesion, so called amyloidoma, may occur in the mediastinum, and it may manifest with various patterns of calcifications such as a punctate, diffuse, or eggshell calcification.
Characteristically, the amyloid deposit shows apple-green birefringence under polarized light on Congo-red staining. Cytotoxic chemotherapy is required in most patients with AL systemic amyloidosis who are associated with plasma cell dyscrasias, and the overall median survival in those patients who received chemotherapy was 5.1 years. In AA amyloidosis, the treatment goal is to manage the underlying disease, and successful treatment often leads to partial regression of the amyloid deposits.
References
1. Czeyda-Pommersheim F, Hwang M, Chen SS, Strollo D, Fuhrman C, Bhalla S. Amyloidosis: Modern Cross-sectional Imaging. Radiographics. 2015 Sep-Oct;35(5):1381-92.
2. Georgiades CS, Neyman EG, Barish MA, Fishman EK. Amyloidosis: review and CT manifestations. Radiographics. 2004 Mar-Apr;24(2):405-16.
3. Fiorelli A, Accardo M, Ciancia G, Pettinato G, Santini M. Isolated mediastinal amyloidosis mimicking a neoplastic lesion. Gen Thorac Cardiovasc Surg. 2014 May;62(5):324-6.
Keywords
Mediastinum, Metabolic and storage lung disesae,

No. of Applicants : 68

▶ Correct Answer : 17/68,  25.0%
  • - staten island univ hops , United States Joseph Lowry
  • - Kinki University Faculty of Medicine, , Japan Mitsuru Matsuki
  • - Shiga University of Medical Science , Japan Akitoshi Inoue
  • - King Abdulaziz University Hospital , Saudi Arabia Amr Ajlan
  • - Kashan University of Medical Sciences, kashan , Iran Ebrahim Razi
  • - Oita University, Faculty of Medicine , Japan Fumito Okada
  • - university of montreal , Canada Andrei Gorgos I
  • - Seoul National University Hospital , Korea (South) Hyoung-In Choi
  • - Narayana Multispeciality Hospital Jaipur Raj , India JAINENDRA JAIN
  • - Gifu University Hospital , Japan Yo Kaneko
  • - SNUH , Korea (South) TAE HYUNG KIM
  • - chp st martin , France Mariotte benoit
  • - Samsung Medical Center , Korea (South) Boda Nam
  • - Kanto Rosai Hospital , Japan Taku Tajima
  • - Maternity & child hospital , Saudi Arabia Sania ALBlushi
  • - Private sector , Greece Vasilios Tzilas
  • - SNUH , Korea (South) Juil Park
▶ Correct Answer as Differential Diagnosis : 17/68,  25.0%
  • - Saitama-Sekishinkai Hosptal , Japan Mihoko Yamazaki
  • - Northern Yokohama Showa university , Japan Kota Watanabe
  • - The University of Tokyo Hospital , Japan Akifumi Hagiwara
  • - Freelance resident in Reunion Island , Reunion Fabien HO
  • - The first affiliated Hospital of Xiamen University , China Qing Qiang Guo
  • - The University of Tokyo Hospital , Japan Toshihiro Furuta
  • - Department of Radiology, Seoul National University Bundang Hospital , Korea (South) Sung Hyun Yoon
  • - Diskapi Yildirim Beyazit Hospital, Ankara , Turkey Meric Tuzun
  • - Asan Medical Center, Ulsan University , Korea (South) Mi Young Kim
  • - University of Tsukuba, Dept of Radiology , Japan Manabu Minami
  • - GHICL , France manuel toledano
  • - Saket City Hospital , India Rajesh Gothi
  • - Fortis hospital , Mohali , India Shaleen Rana
  • - Kizawa Memorial Hospital , Japan Shoji Okuda
  • - Ichinomiya Nishi Hospital , Japan Takao Kiguchi
  • - Jangsung public clinic , Korea (South) Yongsub Song
  • - the first affiliatited hospital of nanjing medical univercity , China Hai Xu
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