Minerva medica
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Langerhans cell histiocytosis, once called histiocytosis X, is a rare disease. Usually it can occur in children and is characterized by granulomas (eosinophilic granuloma and Hand-Schueller-Christian disease) or by a extensive involvement of various organs (Letterer-Siwe disease). The etiology remains uncertain and could be related to undefined immunologic disturbance. Lesions can involve bone marrow, skin, oral mucosa, retro-orbital tissue, central nervous system, lymph nodes, spleen, liver, lung, and gastroenteric tract. Surgery, radiotherapy and chemotherapy can be employed as treatment. Prognosis is different in relation to the extension of the disease. ⋯ The usual treatment of Langerhans cell histiocytosis is surgery and eventually radiotherapy for localized disease and chemotherapy for extended disease. The prognosis is related to the number of involved organs: usually favorable with only one site of disease and unfavorable when more organs are involved. Other unfavorable prognostic factors are the age < 2 years, the presence of anemia, liver and spleen involvement and respiratory failure. In our case only one site of disease was evident and the clinical behavior has been quite favorable. The main peculiarities are the radiologic aspect and the slow, partial regression after the treatment; this fact could be related to the presence of wide calcifications inside the lesion. After 5 years follow-up it is possible to consider the absence of progression as a response to the treatment.