Langerhans' cell histiocytosis presenting with a para-aortic lesion and heart failure

Langerhans' cell histiocytosis (LCH) is an uncommon disease with variable m anifestations. We report a case of LCH with the unusual initial presentatio ns of chest pain and progressive heart failure in a 5-year-old boy. Chest r adiography revealed a wide mediastinum with cardiomegaly. Electrocardiograp hy showed first-degree atrioventricular block and an inverted T wave over V 4-V6. Echocardiography, computed tomography, and magnetic resonance imaging of the chest all showed an infiltrating lesion that enveloped the entire h eart, great vessels, and coronary arteries. Pathologic examination of the b iopsy specimen revealed LCH. Chemotherapy, which included prednisolone, vin cristine, methotrexate, and 6-mercaptopurine, had only a minimal effect on the tumor. After the addition of etoposide, die lesion decreased in size, a nd the symptoms and signs of heart failure and chest pain were ameliorated.