Correct!
5. All of the above
The patient was diagnosed with pulmonary Langerhans cell histiocytosis (PLCH, also known by older terminology as eosinophilic granuloma or histiocytosis X). The classic diagnosis can be made on the basis of compatible imaging showing small nodules and cysts in the upper lungs with bronchoalveolar lavage showing high macrophage counts (1). When patients do not have classic presentation, video-assisted thorascopic surgical (VATS) biopsy is recommended taken from a region with an abundance of nodules. Wedge biopsies of early lesions show multiple well-demarcated grey-white to tan-white irregular, stellate nodules ranging from less than 1 cm to about 2 cm (2). With evolution of PLCH, lesions show increasing amounts of stellate fibrosis and cyst formation. Pathologically, early PLCH lesions consist of discrete bronchiolocentric stellate nodules. Early lesions are more cellular and less fibrotic than more mature lesions and consist of a variable mix of Langerhans cells, lymphocytes, eosinophils, and plasma cells with a background of generally mild fibrosis. Fibrosis replaces the cellular nodules as disease progresses, and less cellular stellate nodules are formed. Langerhans cells usually are immunopositive with CD1a, Langerin, E-cadherin, and S100. Birbeck granules, also termed Langerhans cell granules, pentalaminar rod-shaped cytoplasmic organelles with a racket- or rod-shaped appearance, are found ultrastructurally.
What treatment is most appropriate for this patient with PLCH? (Click on the correct answer to proceed to the fifth and last panel)