Diagnosis
Diagnosis of Langerhans Cell Sarcoma is predominantly a pathologic diagnosis. Cells from these tumor samples will display features characteristic of Langerhans cells, but with additional signs of malignancy, such as increased mitoses, cellular atypia, and pleomorphic nuclei.CD-207 is a cellular marker associated with Birbeck granules than can be used for confirmation of cell type. LCS will also stain positive for CD-68, CD-68R, CD-21, CD-35, S-100, CD1a, lysozyme, HLA-DR, CD4, fascin, Factor XIIIa, and cyclin D1, which are cellular markers typically used in the characterization of various pathology specimens. Positive staining with CD1a and S-100 are required for diagnosis of LCS. Positive staining with CD-207 (Langerin) is an additional confirmation marker of malignancy in LCS. Other cellular markers have been reported to be positive in LCS, but have are not as routinely used.Histologically, Langerhans cells characteristically display Birbeck granules and nuclei with a longitudinal groove. Birbeck granules are currently a poorly understood cellular structure, but are commonly used for cell-type identification. Previously, the presence of Birbeck granules was necessary for diagnosis, but this is no longer considered diagnostic.Currently, there appears to be no relation of LCS with chromosomal abnormalities.The diagnosis of LCS can be difficult, especially due to its rarity. It has previously been mistaken with metastatic melanoma and metastatic large-cell carcinoma. Treatment
Treatment for this rare disease consists of a variety of approaches, with none displaying any increased efficacy over another. There are currently three broad treatment strategies for LCS: surgery, chemotherapy, and combination with radiation therapy.
Pulmonary vein stenosis is a rare cardiovascular disorder. It is recognized as being the stenosis of one or more of the four pulmonary veins that return blood from the lungs to the left atrium of the heart. In congenital cases, it is associated with poor prognosis and high mortality rate. In some people, pulmonary vein stenosis occurs after pulmonary vein ablation for the treatment of atrial fibrillation. Some recent research has indicated that it may be genetically linked in congenital cases. == References ==