When the LCS tumor is readily accessible, the best treatment method is usually surgical removal. There is, however, an observed increased risk of tumor relapse following surgical resection. Both chemotherapy and radiation therapy are useful as treatments, either as monotherapy or in conjunction with other treatments. Chemotherapy is currently the most common treatment method, either alone or in combination. The most common chemotherapeutic regimen consisted of cyclophosphamide, doxorubicin, vincristine, and prednisolone (otherwise known as CHOP therapy). Other common chemotherapeutic regimens include MAID (mesna, doxorubicin, ifosfamide, and dacarbazine), ESHAP (etoposide, carboplatin, cytarabine, and methylprednisolone), EPIG (etoposide, cisplatin, ifosfamide, mesna, and gemcitabine), and AIM (doxorubicin, ifosfamide, and mesna). Other regimens have been used with mixed levels of success. In an effort to prevent recurrence, sometimes combination therapy is used. Following surgical resection, additional radiation therapy or chemotherapy may be performed to prevent relapse. Patients receiving combined chemotherapy and radiation therapy has only shown limited benefit.There are a variety of other therapies under research with varying levels of reported success. These include high-dose chemotherapy, bone marrow transplants,  and somatostatin analogues.When there is only one site of LCS involvement, monotherapy may be sufficient treatment. But when the disease has multiple sites or has metastasized, combination therapy will be necessary to achieve any level of adequate treatment. Prognosis
Overall prognosis of patients with LCS depends heavily on the bodily organ involved and the extent of involvement of the tumor. Organs associated with a poor prognosis include the liver, lungs, and bone marrow.
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.