Isolated Langerhans cell histiocytosis of the spleen

Abstract

Langerhans cell histiocytosis (LCH) is a relatively rare disorder characterized by the proliferation of abnormal Langerhans cells. Splenic involvement usually presents in children with multisystem LCH, and isolated LCH of the spleen is a very rare disease. Herein, we report a case of isolated splenic LCH in an adult man. We report a case of isolated splenic LCH that first manifested with recurrent left upper abdominal pain. Unenhanced CT revealed multiple nodular lesions located at the spleen. Magnetic resonance imaging speculated that these lesions were lymphomas. On the basis of histopathological and immunohistochemical findings, the diagnosis of isolated splenic LCH was confirmed. The patient underwent splenectomy. Histopathologic examination revealed the proliferation of Langerhans cell. Immunohistochemical staining revealed that cells of the tumor were positive for S-100, CD1a, CD45RO, and Vimentin. The patient is alive without recurrence 9 years after operation. Isolated LCH of the spleen may have a favorable prognosis and splenectomy is an effective therapeutic method.