Discussion
Diagnosis With Brief Discussion
- Diagnosis
- Langerhans cell histiocytosis
- Radiologic Findings
- Chest radiograph shows ill-defined small nodular opacities in both lungs with upper lung zone predominance. Chest CT scans demonstrate numerous thick- and thin-walled small lung cysts and micronodules in both lungs with upper lung predominance. He underwent VATS wedge resection of right upper lobe and finally diagnosed as pulmonary Langerhans cell histiocytosis. His medical record revealed that he was a 30 pack-year current smoker.
- Brief Review
- Langerhans cell histiocytosis is a rare disease of unknown etiology, however, 90% or more of the patients are cigarette smokers. In the early stages, HRCT findings include centrilobular opacities and small nodules with bilateral and symmetric distributions. Nodules have irregular borders and are predominantly distributed in the upper, middle lung zones with sparing of lung bases near costophrenic angles. Early stage of the disease is characterized by the abundance of nodules and cavitated nodules, whereas cysts are few and do not tend to be confluent. The differential diagnosis of early stage of this disease includes pulmonary papillomatosis and cavitary metastasis.
Advanced disease is characterized by a small number of nodules, whereas cysts have increased in number and size and are confluent. The following sequence of abnormalities seen with CT corresponds to the evolution of the disease; nodules, cavitated nodules, thick-walled cysts, cysts, and confluent cysts, rupture or collapse of cysts with or without pneumothorax. Although many cysts appear round, they can also have bizarre shapes, being bilobed, clover-leaf shaped, or branching in appearance. The cystic manifestation can mimic that of bullous emphysema or lymphangioleiomyomatosis.
- Please refer to
Case 32, Case 226, Case 367, Case 422, Case 654, Case 680, Case 849, -
- References
- Keywords
- Lung, Interstitial lung disease, smoking related ILD,