대한흉부영상의학회 Korean Society of Thoracic Radiology

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Weekly Chest CasesArchive of Old Cases

Case No : 1385 Date 2024-05-07

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  • Courtesy of Pa Hong / Samsung Seoul Hospital
  • Age/Sex 58 / M
  • Chief ComplaintDyspnea
  • Figure 1
  • Figure 2
  • Figure 3
  • Figure 4
  • Figure 5

Diagnosis With Brief Discussion

Diagnosis
Erdheim-Chester disease (cardiopulmonary manifestations of Erdheim-Chester disease)
Radiologic Findings
Fig 1. Chest PA shows enlarged cardiac configuration and septal thickenings in bilateral lower lung zones.
Fig 2-4 Axial enhanced CT scans reveals circumferential periaortic infiltration, pericardial thickening with effusion, and perirenal infiltration.
Fig 5. Axial non-enhanced CT scan with lung window setting shows fissural and interlobular septal thickenings in bilateral lower lung zones.
Brief Review
Erdheim-Chester Disease (ECD) is a rare, systemic non-Langerhans cell histiocytosis. It is characterized by xanthomatous infiltrates of foamy histiocytes and predominantly affects middle-aged and older adults. ECD commonly presents with symmetrical bone pain, especially in the long bones. Extraskeletal involvement occurs in about 50% of patients, affecting organs such as the retroperitoneum, kidneys, lungs, central nervous system, orbit, skin, heart, breast, sinonasal mucosa, and skeletal muscles. Lung involvement is noted in 20%
References
1. Jitesh Ahuja, Jeffrey P. Kanne, Cristopher A. Meyer, Sudhakar N. J. Pipavath, Rodney A. Schmidt, Jonathan O. Swanson, and J. David Godwin. Histiocytic Disorders of the Chest: Imaging Findings. RadioGraphics 2015 35:2, 357-370
Keywords

No. of Applicants : 82

▶ Correct Answer : 60/82,  73.2%
  • - Ishikawa Matto Central Hospital , Japan MANABU AKIMOTO
  • - Saitama-Sekishinkai Hosptal , Japan MIHOKO YAMAZAKI
  • - , Japan SUZUNE TSUKAMOTO
  • - medical scanning , Japan HIROAKI ARAKAWA
  • - The University of Tokyo Hospital , Japan JUN KANZAWA
  • - ZIGONG TCM HOSPITAL OF CHINA , China CAO CUN YOU
  • - Shiga University of Medical Science , Japan AKITOSHI INOUE
  • - Kyoto University , Japan AKIHIKO SAKATA
  • - McGill University Health Center , Canada ALEXANDRE SEMIONOV
  • - Oita university , Japan AYUMI KAMEI
  • - Kyoto City Hospital , Japan YUSUKE UTSUNOMIYA
  • - , Korea (South) DONG-HO BANG
  • - Healthy Longevity Medical Center , Japan SHIN-ICHI CHO
  • - , France LAURA RAMIREZ
  • - Vita Hospital , Brazil DIOGO LAGO PINHEIRO
  • - , Senegal AXEL MBUYU KAYEMBE
  • - Other , Korea (South) SEONGSU KANG
  • - , Korea (South) JIN YOUNG LEE
  • - Secomedic Hospital , Japan FUMINORI MIYOSHI
  • - Oita University, Faculty of Medicine , Japan FUMITO OKADA
  • - , Korea (South) KANGHWI LEE
  • - Uniyersity of Yamanashi , Japan TAKAAKI HASHIMOTO
  • - Osaka University , Japan AKINORI HATA
  • - Mie University , Japan HIKARI FUKUI
  • - University of Yamanashi , Japan HIROYUKI MORISAKA
  • - The University of Tokyo Hospital , Japan TOSHIHIRO FURUTA
  • - , Japan YOSHIKI ISHII
  • - University of Yamanashi , Japan HIROAKI WATANABE
  • - Gifu University Hospital , Japan Yo Kaneko
  • - , Japan KENTARO KOTANI
  • - Kyoto University Hospital , Japan YASUHISA KURATA
  • - Other , Korea (South) CHAEHUN LIM
  • - Osaka Metropolitan University Hospital , Japan SHU MATSUSHITA
  • - , Japan SHUNJIRO NOGUCHI
  • - , Korea (South) HEONSEOK LEE
  • - Jichi Medical University, School of Medicine , Japan MITSURU MATSUKI
  • - University of Tsukuba, Dept of Radiology , Japan MANABU MINAMI
  • - Shiga General Hospital , Japan YUSAKU MORIBATA
  • - Avrasya Hospital/ISTANBUL , Turkey MURAT ULUSOY
  • - Osaka metropolitan university Hospital , Japan TATSUSHI OURA
  • - Mie university , Japan SHIKO OKABE
  • - , Viet Nam PHI THAI PHAM
  • - GHICL , France MANUEL TOLEDANO
  • - Kyoto university , Japan HIROKAZU SAWAMURA
  • - Other , Korea (South) JY LEE
  • - Korea University Anam Hospital , Korea (South) KYU-CHONG LEE
  • - Paras hospital, Panchkula , India SHALEEN RANA
  • - Kyoto University , Japan SHO KOYASU
  • - Hyogo Prefectural Kobe Children , Japan SHUHEI NORIMOTO
  • - Yonsei University,Severance Hospital , Korea (South) SEO BUM CHO
  • - Fukuoka university , Japan KEISUKE SATO
  • - Kantou Rousai Hospital , Japan KAORU SUMIDA
  • - Ichinomiya Nishi Hospital , Japan Takao Kiguchi
  • - Nerimahikarigaoka hospital , Japan TAKANA HAYASHI
  • - Matsunami General Hospital , Japan TARO TAKEDA
  • - Toyota Kosei Hospital , Japan YUKI HAYASHI
  • - the first affiliatited hospital of nanjing medical univercity , China HAI XU
  • - University of Yamanashi , Japan KOJIRO ONOHARA
  • - , Korea (South) JIN WOO YOON
  • - Jiangsu province hospital , China WANGJIAN ZHA
▶ Correct Answer as Differential Diagnosis : 6/82,  7.3%
  • - Other , Korea (South) HYEJOO PARK
  • - Ome Medical Center , Japan MIKIYA FUJII
  • - Mie university , Japan MIYUKO FUJITA
  • - Dokkyo Medical University , Japan HIROAKI ARAKAWA
  • - The Catholic University of Korea Yoeuido St. Mary , Korea (South) CHAWOONG JEON
  • - Juntendo University , Japan YUTAKA IKENOUCHI
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