Discussion
Diagnosis With Brief Discussion
- Diagnosis
- Langerhans cell histiocytosis
- Radiologic Findings
- Plain chest radiograph shows multiple ill-defined nodular opacities with subtle reticular opacities in both lungs.
Chest CT scan images show multiple tiny nodules, ill-defined cavitary nodules and air cysts in both lungs, with upper and mid- lung zone predominance.
VATS biopsy was done and pulmonary Langerhans cell histiocytosis was confirmed.
- 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. Patterns of the nodules or cysts may represent longevity of the lesions. 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. 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 717, Case 587, Case 422, -
- References
- 1. Abbott GF, Rosado-de-Christenson ML, Franks TJ, Frazier AA, Galvin JR. From the archives of the AFIP Pulmonary langerhans cell histiocytosis. Radiographics. 2004 May-Jun;24(3):821-41
2. Galvin, Jeffrey R, Franks, Teri J. Smoking related lung diseases. J Thorac Imag. 2009; 24(4):274-284
3. Brauner MW, Grenier P, Mouelhi MM, Mompoint D, Lenoir S. Pulmonary histiocytosis X: evaluation with HRCT. Radiology 1989;172:255-258
4. Moor ADA, Godwin DJ, Muller NL, et al. Pulmonary histiocytosis X: comparison of radiographic and CT findings. Radiology 1989;172:249-254
5. Webb WR, Muller NL, Naidich DP. High-resolution CT of the lung. 3rd. Lippincott Williams & Wilkins, 2001:421-429
- Keywords
- Lung, Interstitial lung disease, LCH, smoking related ILD,