Pulmonary Langerhans cell histiocytosis
Updates to Case Attributes
In the presence of pulmonary cystic images, the differential diagnosis includes lymphangioleiomyomatosis and lymphocytic interstitial pneumonia. In addition, history such as immunodeficiency and tobacco use should be investigated.
Lymphangioleiomyomatosis also presents with multiple cysts, possibly coalescent, with thin walls, but with diffuse homogeneous distribution but with a predominant apical distribution. There may be centrilobular nodules.
In Langerhans cell histiocytosis, on the other hand, cavitating nodules, initially thick-walled and spherical in morphology, occur in combination with centrilobular nodules. They evolve into thin-walled cysts, to show coalescence in advanced stages, acquiring tubular morphology or irregular contour partly due to traction due to the presence of fibrosis, and must be differentiated from bullous emphysema. In the final stages, a “burned” lung is described, very similar to emphysema with increased volume, plus a reticular pattern due to fibrosis.
An important characteristic is that this evolution is upward.
In the histopathological study of the piece obtained by transbronchial biopsy, there were cells with a histiocytic appearance and occasional multinucleated giant cells were found. Immunohistochemistry was positive for S100, CD1a and CD68.
The images of this patient were evaluated together with Dr. Agustina Regallo, a colleague belonging to the Diagnostic Imaging Service and Dr. Javier Abdala Pulmonary Specialist.
-<p>In the presence of pulmonary cystic images, the differential diagnosis includes lymphangioleiomyomatosis and lymphocytic interstitial pneumonia. In addition, history such as immunodeficiency and tobacco use should be investigated.</p><p>Lymphangioleiomyomatosis also presents with multiple cysts, possibly coalescent, with thin walls, but with diffuse homogeneous distribution but with a predominant apical distribution. There may be centrilobular nodules.</p><p><br>In Langerhans cell histiocytosis, on the other hand, cavitating nodules, initially thick-walled and spherical in morphology, occur in combination with centrilobular nodules. They evolve into thin-walled cysts, to show coalescence in advanced stages, acquiring tubular morphology or irregular contour partly due to traction due to the presence of fibrosis, and must be differentiated from bullous emphysema. In the final stages, a “burned” lung is described, very similar to emphysema with increased volume, plus a reticular pattern due to fibrosis.</p><p><br>An important characteristic is that this evolution is upward.</p><p>In the histopathological study of the piece obtained by transbronchial biopsy, there were cells with a histiocytic appearance and occasional multinucleated giant cells were found. Immunohistochemistry was positive for S100, CD1a and CD68.</p><p>The images of this patient were evaluated together with Dr. Agustina Regallo, a colleague belonging to the Diagnostic Imaging Service and Dr. Javier Abdala Pulmonary Specialist.</p>- +<p>In the presence of pulmonary cystic images, the differential diagnosis includes lymphangioleiomyomatosis and lymphocytic interstitial pneumonia. In addition, history such as immunodeficiency and tobacco use should be investigated.</p><p>Lymphangioleiomyomatosis also presents with multiple cysts, possibly coalescent, with thin walls, but with diffuse homogeneous distribution but with a predominant apical distribution. There may be centrilobular nodules. In Langerhans cell histiocytosis, on the other hand, cavitating nodules, initially thick-walled and spherical in morphology, occur in combination with centrilobular nodules. They evolve into thin-walled cysts, to show coalescence in advanced stages, acquiring tubular morphology or irregular contour partly due to traction due to the presence of fibrosis, and must be differentiated from bullous emphysema. In the final stages, a “burned” lung is described, very similar to emphysema with increased volume, plus a reticular pattern due to fibrosis. An important characteristic is that this evolution is upward.</p><p>In the histopathological study of the piece obtained by transbronchial biopsy, there were cells with a histiocytic appearance and occasional multinucleated giant cells were found. Immunohistochemistry was positive for S100, CD1a and CD68.</p><p>The images of this patient were evaluated together with Dr. Agustina Regallo, a colleague belonging to the Diagnostic Imaging Service and Dr. Javier Abdala Pulmonary Specialist.</p>