Abstract
<jats:p>Langerhans cell sarcoma (LCS) is a rare high-grade malignant tumor consisting of Langerhans cells and characterized by aggressive course and high mortality. The incidence rate is 2 cases per 100 million population. As opposed to Langerhans cell histiocytosis (LCH), LCS is marked by pronounced cell atypia and tendency towards rapid metastasis. Clinical manifestations vary depending on the lesion location and involve skin, lymph nodes, bones, lungs, liver, spleen, and bone marrow. Due to the rarity of this disease, there are no standardized treatment protocols. The approaches used in LCH therapy are not effective in the treatment of LCS. The present paper reports a case of LCS with intensified chemotherapy regimens similar to those used in highly aggressive lymphomas including subsequent maintenance therapy which is commonly administered to high-risk LCH patients. This therapeutic strategy resulted in the complete LCS remission which has sustained for over 10 years.</jats:p>