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Weekly Chest CasesArchive of Old Cases

Case No : 717 Date 2011-07-26

  • Courtesy of Hyun Ju Seon, Yun-Hyeon Kim, Song Choi / Chonnam National University Hospital
  • Age/Sex 46 / F
  • Chief ComplaintCough, sputum (D: 2months), current smoker (0.5 pack x 20 years)
  • Figure 1
  • Figure 2
  • Figure 3
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  • Figure 5

Figure 1

Diagnosis With Brief Discussion

Diagnosis
Pulmonary Langerhans cell histiocytosis (LCH)
Radiologic Findings
Initial chest radiograph shows diffuse coarse reticulonodular opacities in both lungs with upper lung predominance and relative sparing of both costophrenic angle areas.Chest CT shows wide-spreading solid and cavitary nodules and cysts with variable size and upper lung predominance in both lungs and nodules are located in centrilobular areas with relative sparing of both cardiophrenic angle areas.
Brief Review
LCH was formerly known by various names such as histiocytosis X, eosinophilic granuloma, Letterer-Siwe disease, and Hand-Schu¨ ller-Christian syndrome. LCH is an abnormal nonmalignant
proliferation of monoclonal Langerhans cells within one or multiple organ systems. Although the etiology of LCH is unknown, theories include viral infection, antigen exposure, and somatic mutation. 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. Pulmonary LCH appears to begin in the third to fourth decade of life, but a patient with mild to moderate disease may not receive a diagnosis until later. The most common clinical symptoms include dyspnea, cough, and fatigue, although patients may present with chest pain caused by a pneumothorax. For most patients, symptom stabilization occurs with smoking cessation alone.
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. On HRCT, many cysts appear round, they can also have bizarre shapes, being bilobed, cloverleaf shaped, or branching in appearance. These unusual shapes are postulated to occur because of fusion of several cysts, perhaps because the cysts sometimes represent ecstatic and thick-walled bronchi. In some patients, cysts are the only abnormality visible on HRCT, small nodule are also present. They were usually smaller than 5mm in diameter. Larger nodules, sometimes exceeding 1cm, may also be seen, but they are less common. The margins of nodules are often irregular, particularly when there is surrounding cystic or reticular disease. Many nodules can be seen to be peribronchial or peribronchiolar and therefore centrilobular in location. The nodules are usually homogenous in appearance, but some nodules, particulary those large than 1cm in diameter, may show lucent centers, presumably corresponding to small cavities. HRCT image classically shows upper to mid lung zone predominance in size and number of nodules or cysts with sparing area of costophrenic angles. Phenotypically, LCs express surface CD1a glycoprotein and cytoplasmic S-100 protein, although the latter is not specific.
References
1. Danny L. Leatherwood, Darel E. Heitkamp, Robert E. Emerson. Best Cases from the AFIP Pulmonary Langerhans Cell Histiocytosis

2. Aubry MC, Wright JL, Myers JL. The pathology of smoking-related diseases.
Keywords
lung, lymphoproliferative disease,

No. of Applicants : 118

▶ Correct Answer : 94/118,  79.7%
  • - Suncheonhyang university buchoen hospital , Korea (South) Jeong hye
  • - Deptt of Radiodiagnosis & Imaging, PGIMER chandigarh , India Ram Galwa
  • - Onomichi municipal hospital , Japan Hirofumi Mifune
  • - Oita University, Faculty of Medicine , Japan Fumito Okada
  • - Youngdong Hospital , Korea (South) Sang Min Lee
  • - Hangang sacred heart hospital , Korea (South) You Mie Han
  • - Okayama University , Japan Akihiro Tada
  • - China Medical University ,Taiwan,R.O.C. , Taiwan Jun Jun Yeh
  • - Yokohama-asahi-chuo-general hospital , Japan Kyoko Nagai
  • - Mackay Memorial Hospital, Taipei , Taiwan Sheng-Yeh Shen
  • - Saga University , Japan Tetsuya Kondo
  • - CHU NANCY , France, Metropolitan LEGOU Francois
  • - radiologist, aditya imaging centre , India vivek patel
  • - sunder lal jain hospital , India sanjeev vachher
  • - Showa general hospital , Japan Ai Sugano
  • - chp st martin , France Mariotte benoit
  • - James Paget Hospital , U.K , United Kingdom nabil mahmood
  • - DongA University hospital , Korea (South) Jiyoung Kang
  • - Virgin Mary Hospital Burgas , Bulgaria VLADISLAV RUSINOV
  • - stanley medical college , India arivudai nambi
  • - Kohka Public Hospital , Japan Akitoshi Inoue
  • - EWHA WOMANS MOKDONG UNIVERSITY HOSPITAL , Korea (South) Sang Min Lee
  • - Fr Mullers Medical College , India Deepu Thomas
  • - Mallinckrodt Institute of Radiology , United States Travis Henry
  • - Medical College Chest Hospital,Thrissur,Kerala , India Raveendran TK
  • - DrRP GMEDICAL COLLEGE TANDA , India DINESH SOOD
  • - Diskapi Yildirim Beyazit Hospital, Ankara , Turkey Meric Tuzun
  • - clinique de SAVOIE , France, Metropolitan gay-depassier philippe
  • - Chaeil Clinic , Korea (South) Sunkyung Lim
  • - Osaka University , Japan Hiromitsu Sumikawa
  • - snuh , Korea (South) Choo jiyung
  • - SNUH , Korea (South) Lee Nyoung Keun
  • - Chuncheon Hallym sacred heart hospital , Korea (South) heerok jeong
  • - University of British Columbia , Canada Amr Ajlan
  • - IHL lyon , France BEZZA Riadh
  • - National Jewish Health , United States Jonathan Chung
  • - Hongik Hospital , Korea (South) Pyeong Guk Kang
  • - IMS, BHU,VARANASI , India Sriram Patwari
  • - Chuncheon Sacred Heart Hospital , Korea (South) Pak Su-young
  • - Sunchun university hospital, Bucheon , Korea (South) So Young Bae
  • - Dongnam Institute of Radiological and Medical Sciences , Korea (South) Dae-Wook Yeh
  • - Montreal , Canada D J
  • - Chonbuk National University Hospital , Korea (South) Sungkwan Kim
  • - Dong-A university hospital , Korea (South) Woo-jeong Kim
  • - donga university medical center , Korea (South) Dong Kim
  • - Seoul National University Hospital , Korea (South) YongSub Song
  • - Univ. of Miami/Jackson Memorial , United States mantosh rattan
  • - Toyama University Hospital, Laboratory of Pathology , Japan TOMONORI TANAKA
  • - Onomichi municipal hospital , Japan Ryotaro Kishi
  • - Hallym University College of Medicine , Korea (South) Miyeon Yie
  • - NZOZ Diagnostica , Poland Krzysztof Owsianny
  • - KASH , Saudi Arabia Elbagir Nasser
  • - Hangang Sacred Heart Hospital , Korea (South) Eil Seong Lee
  • - Ewha Womans University Hospital , Korea (South) YOOKYUNG KIM
  • - SMC , Korea (South) Yi Kyung Kim
  • - CHRU lille , France manuel toledano
  • - Toranomon Hospital , Japan Takana Yamakawa
  • - Inha University Hospital , Korea (South) Ju Won Lee
  • - assam medical college , India shantiranjan sanyal
  • - jaslok hospital & research centre mumbai , India JAINENDRA JAIN
  • - HSC , Brazil Diogo Pinheiro
  • - radiology , Korea (South) Lee Sanghee
  • - McGill University Health Center , Canada Alexandre Semionov
  • - KASTURBA MEDICAL COLLEGE , India NAVEEN KULKARNI
  • - Chonnam National University Hospital , Korea (South) Jung hyun Kim
  • - Az Osp Santa Maria Terni , Italy angelo carloni
  • - Hospital Sotiria, Athens, Greece , Greece Vasilios Tzilas
  • - Fukuyama daiichi Hospital , Japan Mototsugu Saeki
  • - Chonnam National University Hospital , Korea (South) Daun Lee
  • - Kosin university Gospel hospital , Korea (South) Hee Kang
  • - Okayama university, Department of Pathology , Japan Junya Itakura
  • - newmasan hospital , Korea (South) park ji hoon
  • - Hopital Louis Pradel LYON , France matthieu ROUSSET
  • - IRSA La Rochelle France , France Denis Chabassiere
  • - Chuncheon Sacred Heart Hospital , Korea (South) Kyung bum Nam
  • - CHA Bundang hospital , Korea (South) Hyerin Kim
  • - Assam Medical college , India samudra borah
  • - Cairo University , Egypt Mamdouh Eissa
  • - royal melbourne hospital , Australia kimchi kings
  • - Chungnam National University hospital , Korea (South) sunkyoung you
  • - Radiologie Brabois adultes , France ROPION helene
  • - hopital louis pradel , France dementhon maud
  • - Saga University , Japan Ryoko Egashira
  • - McGill University , Canada Sean Carr
  • - IRCCS Istituto Oncologico - Bari , Italy Carlo Florio
  • - Kizawa Memorial Hospital , Japan Yo Kaneko
  • - NDMVP Nashik , India Imran Jindani
  • - IRSA , France jean-luc BIGOT
  • - All India Institute of medical sciences , India Justin Moses
  • - Vital Imaging Centre, Mumbai,India , India Ganesh Agrawal
  • - Saint Malo , France jean-baptiste Noel
  • - KUMC ansan , Korea (South) kihwan kim
  • - Okayama university , Japan Mayu Uka
  • - Kangnam Sacred Heart Hospital , Korea (South) Jaewon Kim
▶ Correct Answer as Differential Diagnosis : 2/118,  1.7%
  • - Korea University Guro hospital , Korea (South) Boram Jeon
  • - FORTIS HOSPITAL KOLKATA , India SALIM PARVEZ
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