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Weekly Chest CasesArchive of Old Cases

Case No : 680 Date 2010-11-08

  • Courtesy of Eil Seong Lee / Hallym University College of Medicine
  • Age/Sex 37 / F
  • Chief ComplaintSystemic multiple lymph node enlargements, hypothyroidism, pituitary tumor, No chest symptom
  • Figure 1
  • Figure 2
  • Figure 3
  • Figure 4
  • Figure 5

Diagnosis With Brief Discussion

Diagnosis
Multisystem Langerhans cell histiocytosis
Radiologic Findings
Fig 1. Chest PA shows multiple variable shaped nodules less than 1.0 cm in both upper and middle lung zones.
Fig 2-a to 2-c. High-resolution CT images shows variable shaped nodules (few mm to 1.5cmm) in both upper lungs.
Fig 2-d. Chest CT image show osteolytic lesion in vertebral body of thoracic spine.
Brief Review
Langerhans cell histiocytosis (LCH) is a rare disease of uncertain aetiology and is well recognized in children. Functionally, Langerhans cells are antigen-presenting cells normally distributed in the skin, lymph nodes, bronchial mucosa, and thymus. Patients with LCH are currently categorized as (a) those with single system disease affecting a single site; (b) single system disease affecting multiple sites; (c) multisystem disease. In addition, isolated pulmonary LCH affecting adults was categorized as an LCH variant [1, 2].
Multisystem LCH is characterized by granulomatous infiltration of the lung, bone, skin, lymph nodes, liver, spleen, brain, kidneys, and endocrine system. Children and adult patients share several features, though the proportion with lung involvement is higher in adults, which may be explained by cigarette smoking. It is currently recommended that staging evaluation of LCH should include a bone scan, skeletal X-ray of suspicious bone lesions, a chest X-ray, and an abdominal ultrasound. This case was confirmed with neck lymph node biopsy.
Pulmonary involvement may occur as part of multisystem LCH or isolated pulmonary LCH (PLCH), which accounts for approximately 20% of adult LCH patients [1, 3]. . In PLCH, the nodules are non-clonal, which partly accounts for its classification as variant LCH with up to 95% of adults with PLCH cigarette smokers. Symptoms may include cough, dyspnea and chest pain with pneumothorax a recognized complication but up to 20% of patients may be asymptomatic. Reduced DLCO is found in up to 90% of affected patients. Imaging findings include small nodules (1
References
1. Dolye DJ, Weisbrod GL, Downey GP. Imaging of multisystem Langerhans cell histiocytosis in an adult. Eur J Radiol 2007:109-117.
2. Favara BE, Feller AC, Pauli M, et al. Contemporary classification of histiocytic disorders. The WHO committee on histiocytic/reticulum cell proliferations. Reclassification working group of the histiocyte society. Med Pediatr Oncol 1997; 29:157-166.
3. Abbott GF, Rosado-de-Christenson ML, Franks TJ, Frazier AA, Galvin JR. From the archives of the AFIP Pulmonary Langerhan's cell histiocytosis. RadioGraphics 2004; 24:821-841.
Keywords
Multiple organ, Interstitial lung disease, LCH, smoking related ILD,

No. of Applicants : 79

▶ Correct Answer : 18/79,  22.8%
  • - Hangang sacred heart hospital , Korea (South) You Mie Han
  • - Osaka University , Japan Osamu Honda
  • - Montreal , Canada D J
  • - James Paget Hospital , U.K , United Kingdom nabil mahmood
  • - Teleradiology solutions , India harish bhambore
  • - seoul national university hospital , Korea (South) Ijin Joo
  • - Onomichi municipal hospital , Japan Junya Itakura
  • - Assam Medical college , India samudra borah
  • - McGill University Health Center , Canada Alexandre Semionov
  • - APOLLO BSR ,BHILAI , India girish verma
  • - Univ. of Miami/Jackson Memorial , United States mantosh rattan
  • - chonnam university hospital , Korea (South) shin hyo hyun
  • - Toyama University Hospital, Laboratory of Pathology , Japan TOMONORI TANAKA
  • - Chonnam university hospital , Korea (South) Minyoung Jung
  • - IRCCS Istituto Oncologico - Bari , Italy Carlo Florio
  • - clinique de SAVOIE , France, Metropolitan gay-depassier philippe
  • - NASA SCANS , India RAKESH BHATIA
  • - Yokohama-asahi-chuo-general hospital , Japan Kyoko Nagai
▶ Correct Answer as Differential Diagnosis : 12/79,  15.2%
  • - Oita University, Faculty of Medicine , Japan Fumito Okada
  • - Onomichi municipal hospital , Japan Hirofumi Mifune
  • - McGill University , Canada Ben Smith
  • - Auckland hospital , New Zealand (Aotearoa) Yuranga Weerakkody
  • - Siirt Devlet Hastanesi , Turkey Armagan Sarac
  • - Dongnam Institute of Radiological and Medical Sciences , Korea (South) Dae Wook Yeh
  • - Fukuyama daiichi Hospital , Japan Mototsugu Saeki
  • - All India Institute of medical sciences , India Justin Moses
  • - Mallinckrodt Institute of Radiology , United States Naganathan Mani
  • - Hospital Sotiria, Athens, Greece , Greece Vasilios Tzilas
  • - Beaulieu clinic Geneva , Switzerland gilles GENIN
  • - vims , India akhila d
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