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Weekly Chest CasesArchive of Old Cases

Case No : 1259 Date 2021-12-06

  • Courtesy of Yuna Lee / Seoul National University Hospital
  • Age/Sex 31 / M
  • Chief ComplaintChest discomfort (1 month ago)
  • Figure 1
  • Figure 2
  • Figure 3
  • Figure 4
  • Figure 5

Diagnosis With Brief Discussion

Diagnosis
Langerhans cell histiocytosis
Radiologic Findings
Fig 1. Chest PA shows multiple small cystic lesions in the upper and middle lungs.
Fig 2-5. Chest CT scans reveal multiple variable-sized thin-walled cystic and cavitary nodules with irregular shape in both lungs with upper lung predominance and relative sparing of both costophrenic angle areas. There are no pneumothorax, pleural effusion, or mediastinal lymphadenopathy.
Brief Review
Clinical course of the patient
The patient underwent VATS lung biopsy of the right upper lobe and was finally diagnosed with pulmonary Langerhans cell histiocytosis. On pathologic specimens, the tissue was composed of multinodular fibrosis with many eosinophils and Langerhans cell infiltration. Langerhans cell aggregates were highlighted by positive immunohistochemical staining for CD1a.

Brief Discussion
Langerhans cell histiocytosis (LCH) is a rare disease of unknown etiology, characterized by organ infiltration with specialized myeloid cells, which share morphological and surface receptor markers with epidermal Langerhans cells. Pulmonary LCH refers to LCH isolated to the respiratory system, notably the lungs. The prevalence of pulmonary LCH is unknown but may account for about 3-5% of all adult diffuse lung diseases. In adults, pulmonary LCH occurs predominantly in young smokers on ex-smokers, with a peak incidence between the ages of 20 and 40 years. Pulmonary LCH occurs with equal frequency in both genders.
The radiologic findings of pulmonary LCH vary depending on the stage of the disease at diagnosis. Chest x-ray often shows bilateral, and generally symmetric reticulo-micronodular changes, in which cysts may sometimes be identified, predominantly involving the upper and middle lung fields. Chest CT characteristically demonstrates a combination of nodules, cavitating nodules measuring 1-10 mm in diameter and thick-walled or thin-walled cysts. Cysts vary in size and may coalesce to form irregular shapes. Lesions are predominantly located in the upper and middle lung fields with relative sparing of the basal lungs. In the early stage of the disease, nodules and cavitary nodules are more numerous than lung cysts, while more advanced disease is often cystic in appearance.
Patients with pulmonary LCH show 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 the continued cystic replacement of lung parenchyma that may progress to end-stage lung disease.
Please refer to
Case 1217, Case 980, Case 904, Case 849, Case 717, Case 680, Case 654, Case 587, Case 422, Case 367, Case 226,
KSTR imaging conference 2018 Summer  Case 6 ,
KSTR Imaging Conference 2015 Spring  Case 2 ,
KSTR Imaging conference 2009 Summer  Case 9 ,
KSTR Imaging Conference 2002 Summer  Case 5 ,
KSTR Imaging Conference 2000  Case 4,
References
1. Vassallo R, Harari S, Tazi A. Current understanding and management of pulmonary Langerhans cell histiocytosis. Thorax 2017; 72:937
2. Leatherwood D. et al. Pulmonary Langerhans Cell Histiocytosis. Radiographics. 2007;27:265-8
Keywords

No. of Applicants : 102

▶ Correct Answer : 87/102,  85.3%
  • - Ishikawa Matto Central Hospital , Japan MANABU AKIMOTO
  • - Saitama-Sekishinkai Hosptal , Japan MIHOKO YAMAZAKI
  • - , Japan HIROAKI ARAKAWA
  • - Korea University Guro Hospital , Korea (South) SEOKYOUNG LEE
  • - ZIGONG TCM HOSPITAL OF CHINA , China CAO CUN YOU
  • - Kinki University Faculty of Medicine, , Japan MITSURU MATSUKI
  • - Showa University Fujigaoka Hospital , Japan KYOKO NAGAI
  • - Niigata University , Japan ATSUSHI UEHARA
  • - Ulsan University Hospital , Korea (South) SOYEOUN LIM
  • - Mayo Clinic , United States AKITOSHI INOUE
  • - Chungbuk National University Hospital , Korea (South) YEONGTAE PARK
  • - Other , Korea (South) HYEJIN YANG
  • - Dhq hospital faisalabad , Pakistan AMMAR AHMED
  • - Affilitated Hospital of Jining Medical college , China JIANG SHENG HUA
  • - , United Kingdom SAMSON KADE
  • - Other , Korea (South) MINGI SHIN
  • - McGill University Health Center , Canada ALEXANDRE SEMIONOV
  • - CHCB , France LE GUEN RAPHAEL
  • - Osaka Red Cross Hospital , Japan YUSUKE UTSUNOMIYA
  • - NIMS, HYDERABAD , India BHASKAR K
  • - National Center of Neurology and Psychiatry , Japan MOTO NAKAYA
  • - Az Osp Santa Maria Terni , Italy ANGELO CARLONI
  • - , Italy PAOLO BALDASSARI
  • - Tiger Gate Hospital , Japan SHIN-ICHI CHO
  • - Ichinomiya Nishi Hospital , Japan CHIHIRO KIGUCHI
  • - Chungbuk National University Hospital , Korea (South) JIYUN KANG
  • - Vita Hospital , Brazil DIOGO LAGO PINHEIRO
  • - Other , Korea (South) SEONGSU KANG
  • - , India SHASHANK SOOD
  • - , Korea (South) JIN YOUNG LEE
  • - , China FANG YING GEN
  • - university of montreal , Canada Andrei Bogdan Gorgos I
  • - Chonbuk National University Hospital , Korea (South) JUNGHWAN KIM
  • - Osaka University , Japan AKINORI HATA
  • - Ajou University Hospital , Korea (South) HAEIN LEE
  • - Shimada General Medical Center , Japan HAYATO NOZAWA
  • - National Center for Global Health and Medicine , Japan MASATOSHI HOTTA
  • - , Korea (South) HYEWON CHOI
  • - The University of Tokyo Hospital , Japan TOSHIHIRO FURUTA
  • - Ajou University Hospital , Korea (South) MINJI KIM
  • - HHS , Canada S LEE
  • - Seoul National University Bundang Hospital , Korea (South) SUNG HYUN YOON
  • - Inje University Pusan Paik Hospital , Korea (South) JIYEON HAN
  • - Kyeongpook National University Hospital , Korea (South) HYEONJUN JANG
  • - Yeungnam University Medical Center , Korea (South) JONGSOO PARK
  • - Gifu University Hospital , Japan Yo Kaneko
  • - McGill , Canada MUTAZ ADNAN KHAIRO
  • - Chonbuk National University Hospital , Korea (South) HYUNSOO BAE
  • - Kyoto University Hospital , Japan YASUHISA KURATA
  • - The University of Tokyo Hospital , Japan RYO KUROKAWA
  • - , Korea (South) YOU NA KIM
  • - , Korea (South) EUNJIN LEE
  • - , Korea (South) YEONG UK HWANG
  • - , United States HIROAKI TAKAHASHI
  • - Osaka City General Hospital , Japan SHU MATSUSHITA
  • - Chungbuk National University Hospital , Korea (South) MIHYEON PARK
  • - Other , Korea (South) MIN A LEE
  • - Other , Korea (South) MINSU KIM
  • - Centre Hospitalier Regional de Lanaudiere , Canada MATTHIEU STORME
  • - AIMS , India NITHYA HARIDAS MALIYAM
  • - Osaka city university Hospital , Japan TATSUSHI OURA
  • - CHU Poitiers , France PAUL RHEINART
  • - Other , Korea (South) SEONG WON JANG
  • - Dokkyo Medical University , Japan HIROAKI ARAKAWA
  • - Columbia asia hospital Hebbal ,Bengaluru , India PRAVIN KUMAR M
  • - Other , Korea (South) KYU-CHONG LEE
  • - Kyoto University Hospital , Japan SATOSHI IKEDA
  • - Paras hospital, Panchkula , India SHALEEN RANA
  • - Nishida Hospital , Japan SHOJI OKUDA
  • - University of Tsukuba Hospital , Japan SODAI HOSHIAI
  • - Chonbuk National University Hospital , Korea (South) MINJEE KIM
  • - Toranomon Hospital , Japan KAORU SUMIDA
  • - Chonbuk National University Hospital , Korea (South) DONG HAN SHIN
  • - Ichinomiya Nishi Hospital , Japan Takao Kiguchi
  • - , Japan KANA TANAKA
  • - The University of Tokyo Hospital , Japan HIROSHI TAKUMIDA
  • - , Japan KAZUMA TERAUCHI
  • - , China WANG DR
  • - Private sector , Greece VASILIOS TZILAS
  • - Rasoul Akram generel hospital of Iran university of medical science , Iran MOHAMAD REZA AYOUBPOUR
  • - College of Medicine, CHA University , Korea (South) PARK JONG WON
  • - , Japan YUKI HAYASHI
  • - , Korea (South) WONJU HONG
  • - the first affiliatited hospital of nanjing medical univercity , China HAI XU
  • - Kyemyung University Dongsan Hospital , Korea (South) DONG HYEON KIM
  • - Korea University Ansan Hospital , Korea (South) CHERRY KIM
  • - Jiangsu province hospital , China WANGJIAN ZHA
▶ Correct Answer as Differential Diagnosis : 4/102,  3.9%
  • - Mallinckrodt Institute of Radiology , United States NAGANATHAN MANI
  • - Avrasya Hospital/ISTANBUL , Turkey MURAT ULUSOY
  • - , Japan SOTA MASUOKA
  • - Diagnose.me (BV) / Royal Perth Hospital , Australia YURANGA WEERAKKODY
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Current Editor : Sang Young Oh, M.D., Ph.D Email : sangyoung.oh@gmail.com

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