대한흉부영상의학회 Weekly Chest Cases

Case No : 980 Date 2016-08-08

Courtesy of Geewon Lee, Ji-Won Lee / Pusan National University Hospital
Age/Sex 59 / M
Chief ComplaintCough for 2 months

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Diagnosis With Brief Discussion

Diagnosis: Pulmonary Langerhans cell histiocytosis
Radiologic Findings: Posteroanterior chest radiographs reveal diffuse reticular opacities and small nodules throughout the lungs.
Axial CT scans in lung window setting demonstrate widespread small, bizarre shaped lung cysts with resultant parenchymal destruction. All lobes were involved with relative sparing of the costophrenic angles.
Video-assisted thoracoscopic surgery was performed to obtain diagnostic wedge biopsy specimens of the right upper and right lower lobes. Pathologic findings were consistent with pulmonary Langerhans cell histiocytosis (LCH).
Brief Review: Pulmonary LCH refers to LCH isolated to the respiratory system, notably the lungs. This rare disease is found almost exclusively in cigarette smokers, which supports the theory of antigen exposure, since cigarette smoke contains thousands of known antigens. The radiologic findings of pulmonary LCH vary depending on the stage of the disease at diagnosis. Diffuse bilateral ill-defined nodules will be seen in early stages. Because the disease has an inhalational component, the middle and upper lung zones are involved to a greater extent than the lung bases. It is thought that these nodules undergo cystic degeneration as the disease progresses, and so a reticular pattern begins to predominate on chest radiographs as the numerous cystic walls are superimposed on one another. The cysts and residual parenchyma can undergo fibrosis over time and eventually lead to changes of honeycombing. High-resolution CT plays an important role in the evaluation of pulmonary LCH. The diagnosis can be easily made with high-resolution CT by showing both ill-defined nodules and cysts in a heavy smoker. However, the diagnostic accuracy of high-resolution CT falls short when only nodules or cysts alone are present. Most of these cases are confirmed with lung biopsy.
Patients with pulmonary LCH have variable and uncertain clinical courses. Up to one-half will show clinical and radiographic stability, while up to 25% will demonstrate spontaneous regression. The remaining 25% can have continued cystic replacement of parenchyma that may progress to end-stage lung disease.
References: Leatherwood D. et al. Radiographics. 2007;27:265-8 Pulmonary Langerhans Cell Histiocytosis.
Keywords: Lung, Interstitial lung disease, smoking related ILD,

No. of Applicants : 85

▶ Correct Answer : 45/85, 52.9%

- The University of Tokyo Hospital , Japan Akifumi Hagiwara
- radiologist, aditya imaging centre , India vivek patel
- Yokohama-asahi-chuo-general hospital , Japan Kyoko Nagai
- Seoul medical center , Korea (South) Tae-ran Ahn
- Asan Medical Center, Ulsan University , Korea (South) Mi Young Kim
- Korea University Guro Hospital , Korea (South) Sun Hong
- Oita University, Faculty of Medicine , Japan Fumito Okada
- Military service , Korea (South) Dong Jang
- The first affiliated Hospital of Xiamen University , China Qing Qiang Guo
- Kyoto University , Japan Akihiko Sakata
- Korea University Guro Hospital , Korea (South) Bit Na Park
- samsung medical center , Korea (South) Dongyoung Jeong
- Samsung Medical Center , Korea (South) Boda Nam
- Policlinico Umberto I , Italy Paolo Baldassari
- Onomichi municipal hospital , Japan Hirofumi Mifune
- Seirei Hamamatsu General Hospital , Japan Kenichi Mizuki
- University of Tsukuba, Dept of Radiology , Japan Manabu Minami
- The University of Tokyo Hospital , Japan Kaoru Sumida
- Korea University Guro Hospital , Korea (South) Yoonmi Choi
- Saitama-Sekishinkai Hosptal , Japan Mihoko Yamazaki
- Korea University Guro Hospital , Korea (South) Jeong Woo Kim
- Ichinomiya Nishi Hospital , Japan Takao Kiguchi
- university of montreal , Canada Andrei Gorgos I
- Yan Bian University hospital , China ZHANG YING
- The University of Tokyo Hospital , Japan Yusuke Watanabe
- Korea university medical center, guro hospital , Korea (South) JINWOO HAN
- Department of Radiology, Seoul National University Bundang Hospital , Korea (South) Sung Hyun Yoon
- Gifu University Hospital , Japan Yo Kaneko
- Kizawa Memorial Hospital , Japan Tomohiro Ando
- Asan Medical Center , Korea (South) Hyung Jung Koo
- Ewha womans university , Korea (South) kyoungmin lee
- McGill University Health Center , Canada Alexandre Semionov
- Seoul national university hospital , Korea (South) Kumju Chae
- Amagasaki General Medical Center , Japan Genki Fukumoto
- Otsu Municipal Hospital , Japan Noriatsu Ichiba
- the first affiliatited hospital of nanjing medical univercity , China Hai Xu
- IRSA LA ROCHELLE , France, Metropolitan BIGOT
- Konkuk University Medical Center , Korea (South) Yong Kwon
- Private sector , Greece Vasilios Tzilas
- CHU Poitiers , France CHAN paul
- CLINIQUE STE CLOTILDE , Reunion patrick MASCAREL
- ZIGONG TCM HOSPITAL OF CHINA , China Cao Cunyou
- Nasaret Hospital , Korea (South) Hee Seok Choi
- Asan Medical Center , Korea (South) , Korea (South) Han Na Lee
- Hyogo Prefectural Amagasaki General Medical Center , Japan Tomoaki Otani

▶ Correct Answer as Differential Diagnosis : 7/85, 8.2%

- The University of Tokyo Hospital , Japan Toshihiro Furuta
- GHICL , France manuel toledano
- SAISEIKAI KURIHASHI HOSPITAL , Japan YASUO OOKUBO
- the First Affiliated Hospital of NanJing Medical University , China Aiping Chen
- Shiga University of Medical Science , Japan Akitoshi Inoue
- Kanto Rosai Hospital , Japan Taku Tajima
- Centre Hospitalier Regional de Lanaudiere , Canada Matthieu Storme

대한흉부영상의학회 Korean Society of Thoracic Radiology

Archive of Old Cases

Case No : 980 Date 2016-08-08

Discussion

No. of Applicants : 85