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.
By definition, Langerhans Cell Sarcoma (LCS) is a cancerous disease caused by the uncontrolled overproliferation of Langerhans cells.Because Langerhans cells are most commonly found in the mucosa and the skin, LCS is thought to usually begin here with further spread to other areas of the body via the lymphatic system.Langerhans cell sarcoma can occur de novo, or can occur as a malignant transformation of Langerhans cell histiocytosis. Clinical Manifestations
The related condition, LCH, presents with various clinical features depending on the bodily organs involved. LCS shares most of these clinical presentations. Most commonly, these patients will present with generalized signs and symptoms such as fever and weight loss. Blood tests will commonly show pancytopenia, an overall reduction in blood cell counts. Additionally, these patients may display lymphadenopathy, hepatosplenomegaly, skin lesions, bone lesions, and lung lesions. Most common site presentations are the skin and lymphatics. Other common sites of involvement include the lung, liver, and spleen.LCS will present with a varied gross appearance. When involving the skin, LCS will present as a patches of erythema, with nodules and ulceration present as well. Skin lesions will most commonly involve the trunk, scalp, and legs. Tumor size may range from 1-6 cm, but may be larger. Usually the tumor is well circumscribed with a surface covered in small nodules or protuberances. Most are of a solid consistency  and are of a pink, white, or tan color. LCS tumors may present with much larger sizes and aggressive features, even causing some bleeding and necrosis of the surrounding tissue.