Discussion
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
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KSTR Imaging Conference 2015 Spring Case 2
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KSTR Imaging conference 2009 Summer Case 9
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KSTR Imaging Conference 2002 Summer Case 5
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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