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Weekly Chest CasesArchive of Old Cases

Case No : 1217 Date 2021-02-16

  • Courtesy of Ki-Nam Lee, Eun-Ju Kang, Hyun Jin Kim / Dong-a University Hospital
  • Age/Sex 38 / M
  • Chief ComplaintAsymptomatic, abnormal finding on f/u chest CT Rectal neuroendocrine tumor (NET) patient
  • Figure 1
  • Figure 2
  • Figure 3
  • Figure 4

Diagnosis With Brief Discussion

Diagnosis
Langerhans cell histiocytosis (LCH)
Radiologic Findings
Chest radiograph shows multiple small nodules and thin-walled cystic lesions in upper and middle lungs. Chest CT images show multiple small cystic or cavitary nodules in both lungs, especially upper lobes while relatively sparing of lung base. There are no pneumothorax, pleural effusion, and thoracic lymphadenopathy. On chest CT 1 year ago, there was no lesion.
In this case, the patient has been following up for rectal neuroendocrine tumor (NET), and multiple cystic nodules were newly developed when compared with previous chest CT 1 year ago. The patient was asymptomatic. His medical record revealed that he was a 30 pack-year current smoker. Through this, we could think of pulmonary LCH and cystic metastasis as a differential diagnosis.
Brief Review
He underwent VATS wedge resection of the right upper and lower lobe and was finally diagnosed with pulmonary Langerhans cell histiocytosis. On pathologic specimens, the tissue was composed of a densely cellular infiltrate of histiocytes and other inflammatory cells within the interstitium and air space. Langerhans cell aggregates were highlighted by positive immunohistochemical staining for CD1a. At higher magnification, sheets of histiocytes (arrows) with abundant pale, eosinophilic cytoplasm (arrowhead) were apparent.

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 (LCs). Pulmonary LCH refers to LCH isolated to the respiratory system, notably the lungs. In adults, PLCH occurs predominantly in young smokers or ex-smokers (>90% of cases), with a peak incidence between the ages of 20 and 40 years. PLCH occurs with equal frequency in both genders.
There are for key elements concerning the pathogenesis of PLCH: (1) the mechanisms of accumulation of large numbers of CD1a+ cells in bronchiolocentric loosely formed granulomas; (2) the capacity of these granulomatous lesions to destroy and remodel surrounding tissues; (3) the reactive versus clonal/neoplastic nature of the disease; and (4) the role of smoking in adult PLCH.
The radiologic findings of pulmonary LCH vary depending on the stage of the disease at diagnosis.
The radiographic findings of LCH include reticular, nodular, and reticulonodular patterns, often in combination; a cystic appearance may mimic honeycombing. Abnormalities usually are bilateral, predominantly involving the middle and upper lung zones, with relative sparing of costophrenic angles.
In the early stage of the disease, only small nodules (usually smaller than 10 mm) may be seen, with a peribronchiolar distribution. Cavitation may be present in as many as 10% of patients. The classic CT manifestation includes cysts of varying size and thickness, with scattered peribronchiolar nodules. As the disease progress, cysts predominate and appear thin-walled, an appearance very similar to that of emphysema.
Histologically, PLCH may have different appearances depending on the stage of the disease. In the early stages, lesions are predominantly cellular, with a high concentration of Langerhans cells, eosinophils, and various inflammatory cells. Langerhans cell nuclei have a typical folded appearance and are positive for CD1, S-100, and E-cadherin stains. Langerhans cells also contain Birbeck granules, which appear as pentalaminar rods or racquet-shaped inclusions at electron microscopy. As the disease progresses, the cellular component decreases and is replaced by collagen, leading to fibrosis and the “burned-out” lesion typically described as a peribronchiolar stellate scar.
Smoking cessation is of paramount importance in treating PLCH, with stabilization occurring in most patients and regression occurring in 10%–15% of patients who quit smoking. For those in whom the disease progresses, corticosteroid and immunosuppressive therapy may be of use.
Please refer to
Case 980, Case 904, Case 849, Case 717, Case 587, Case 422,
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. Verssallo R, Harari S, Tazi A. Current understanding and management of pulmonary Langerhans cell histiocytosis. Thorax 2017; 72:937–945.
2. Danny L, Darel E, Robert El. Best cases from the AFIP, Pulmonary Langerhans Cell Histiocytosis. RadioGraphics 2007; 27:265–268.
3. Alison C, Miller K, Farver C, et al. AIRP Best Cases in Radiologic Pathologic Correlation: Pulmonary Langerhans Cell Histiocytosis. RadioGraphics 2012; 32:987–990.
Keywords

No. of Applicants : 86

▶ Correct Answer : 58/86,  67.4%
  • - Saitama-Sekishinkai Hosptal , Japan MIHOKO YAMAZAKI
  • - , Japan HIROAKI ARAKAWA
  • - Korea University Guro Hospital , Korea (South) LEE SEOKYOUNG
  • - 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
  • - Chungbuk National University Hospital , Korea (South) YEONGTAE PARK
  • - Other , Korea (South) HYEJIN YANG
  • - Chonbuk National University Hospital , Korea (South) MINGI SHIN
  • - McGill University Health Center , Canada ALEXANDRE SEMIONOV
  • - Centre Hospitalier Boulogne-sur-Mer , France BENJAMIN DAMAREY
  • - , Italy PAOLO BALDASSARI
  • - Tiger Gate Hospital , Japan SHIN-ICHI CHO
  • - Kyeongpook National University Hospital , Korea (South) CHANGGUN KIM
  • - Ichinomiya Nishi Hospital , Japan CHIHIRO KIGUCHI
  • - Asan Medical Center , Korea (South) WOOIL KIM
  • - Vita Hospital , Brazil DIOGO LAGO PINHEIRO
  • - Other , Korea (South) SEONGSU KANG
  • - , Korea (South) JIN YOUNG LEE
  • - university of montreal , Canada Andrei Bogdan Gorgos I
  • - National Center for Global Health and Medicine , Japan MASATOSHI HOTTA
  • - , Korea (South) HYEWON CHOI
  • - Other , Korea (South) YEJI SHIN
  • - HHS , Canada S LEE
  • - Seoul National University Bundang Hospital , Korea (South) SUNG HYUN YOON
  • - Kyeongpook National University Hospital , Korea (South) HYEONJUN JANG
  • - Yeungnam University Medical Center , Korea (South) JONGSOO PARK
  • - Gifu University Hospital , Japan Yo Kaneko
  • - , Japan KANAE TAKAHASHI
  • - McGill , Canada MUTAZ ADNAN KHAIRO
  • - The University of Tokyo Hospital , Japan RYO KUROKAWA
  • - Kangwon National University Hospital , Korea (South) SEONGJAE BAE
  • - Soonchunhyang University Hospital Bucheon , Korea (South) SEONGHWAN BYUN
  • - Osaka City General Hospital , Japan SHU MATSUSHITA
  • - Other , Korea (South) MINSU KIM
  • - University of Tsukuba, Dept of Radiology , Japan MANABU MINAMI
  • - AIMS , India NITHYA HARIDAS MALIYAM
  • - Chonbuk National University Hospital , Korea (South) KUM JU CHAE
  • - the third hospital of datong ,shanxi province , China GAO ZHI XU
  • - , Japan HIROAKI ARAKAWA
  • - Kyeongpook National University Hospital , Korea (South) PARK BYUNG GEON
  • - TB centre kasaragod. , India rikhy krishnan
  • - Kitano Hospital , Japan SATOSHI IKEDA
  • - Pusan National University Yangsan Hospital , Korea (South) JI HWAN KIM
  • - Toranomon Hospital , Japan KAORU SUMIDA
  • - Ichinomiya Nishi Hospital , Japan Takao Kiguchi
  • - National Center for Global Health and Medicine , Japan HIROSHI TAKUMIDA
  • - , Japan KAZUMA TERAUCHI
  • - Seoul National University Bundang Hospital , Korea (South) JUNWOO KIM
  • - , Japan TOMOKI IMOKAWA
  • - Tokyo Metropolitan Bokutoh Hospital , Japan TOMOKI WADA
  • - Private sector , Greece VASILIOS TZILAS
  • - College of Medicine, CHA University , Korea (South) PARK JONG WON
  • - , Korea (South) WONJU HONG
  • - Kyoto university , Japan AKIHIKO SAKATA
  • - the first affiliatited hospital of nanjing medical univercity , China HAI XU
  • - Jiangsu province hospital , China WANGJIAN ZHA
▶ Correct Answer as Differential Diagnosis : 9/86,  10.5%
  • - Ishikawa Matto Central Hospital , Japan MANABU AKIMOTO
  • - TWMU Medical Center East , Japan MASAFUMI KAIUME
  • - Mayo Clinic , United States AKITOSHI INOUE
  • - Chonnam National University Hospital , Korea (South) MOON GYEONG IL
  • - , Korea (South) EUNJIN LEE
  • - Other , Korea (South) DONGYOUNG JEONG
  • - Other , Korea (South) KYU-CHONG LEE
  • - Diagnose.me (BV) / Royal Perth Hospital , Australia YURANGA WEERAKKODY
  • - Hanyang University Hospital , Korea (South) SEUNG JIN YOO
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