[3] Dendritic cell neoplasms are classified by the World Health Organization as follows:[4] The exact incidence of LCS is unknown due to the rarity of the condition.
The related condition, Langerhans Cell Histiocytosis (LCH) is estimated to have an incidence of around 5 cases per 1 million people per year.
[5] In another systematic review, the median age of presentation was 50 years, but with a slight predilection for males.
[5] 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.
[3][8] The related condition, LCH, presents with various clinical features depending on the bodily organs involved.
LCS tumors may present with much larger sizes and aggressive features, even causing some bleeding and necrosis of the surrounding tissue.
[5] 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.
[5] Histologically, Langerhans cells characteristically display Birbeck granules and nuclei with a longitudinal groove.
[5][6] Birbeck granules are currently a poorly understood cellular structure, but are commonly used for cell-type identification.
[8] Treatment for this rare disease consists of a variety of approaches, with none displaying any increased efficacy over another.
[3] The most common chemotherapeutic regimen consisted of cyclophosphamide, doxorubicin, vincristine, and prednisolone (otherwise known as CHOP therapy).
But when the disease has multiple sites or has metastasized, combination therapy will be necessary to achieve any level of adequate treatment.
[4] Evidence of metastatic disease or relapse from a previously treated LCS usually signifies a worsened prognosis.
[4] Treatment of these metastatic lesions or relapsed tumors may improve the patient's prognosis, but there is limited evidence as to a preferred therapy.