Langerhans' cell histiocytosis presenting with a para-aortic lesion and heart failure
Journal
Journal of the Formosan Medical Association
Journal Volume
100
Journal Issue
2
Pages
127-130
Date Issued
2001
Author(s)
Abstract
Langerhans' cell histiocytosis (LCH) is an uncommon disease with variable manifestations. We report a case of LCH with the unusual initial presentation of chest pain and progressive heart failure in a 5-year-old boy. Chest radiography revealed a wide mediastinum with cardiomegaly. Electrocardiography showed first-degree atrioventricular block and an inverted T wave over V4-V6. Echocardiography, computed tomography, and magnetic resonance imaging of the chest all showed an infiltrating lesion that enveloped the entire heart, great vessels, and coronary arteries. Pathologic examination of the biopsy specimen revealed LCH. Chemotherapy, which included prednisolone, vincristine, methotrexate, and 6-mercaptopurine, had only a minimal effect on the tumor. After the addition of etoposide, the lesion decreased in size, and the symptoms and signs of heart failure and chest pain were ameliorated.
SDGs
Other Subjects
etoposide; mercaptopurine; methotrexate; prednisolone; vincristine; angina pectoris; article; atrioventricular block; cardiomegaly; case report; child; clinical feature; computer assisted tomography; coronary artery; drug response; echocardiography; electrocardiography; great blood vessel; heart failure; histiocytosis; histopathology; human; Langerhans cell; male; mediastinum; nuclear magnetic resonance imaging; symptomatology; T wave; thorax radiography; tumor biopsy; tumor volume; Aortic Diseases; Chest Pain; Child, Preschool; Heart Failure, Congestive; Histiocytosis, Langerhans-Cell; Humans; Male
Type
journal article