FDG PET/CT in Mixed Langerhans Cell Histiocytosis and Erdheim-Chester Disease

The histiocytoses arise from hematopoietic bone marrow stem cells or monocytes. They range from limited to disseminated disease. We report a 31-year-old woman with multisystem Langerhans cell histiocytosis (LCH). The recognition that pericardial disease was rare in LCH prompted review of the initial limited biopsies, which helped establish a second diagnosis of Erdheim-Chester disease. The increasing recognition of mixed histiocytoses requires searching for Erdheim-Chester disease when a patient is diagnosed with LCH and vice versa. FDG PET/CT can help establish the diagnosis or suggest mixed disease based on the organs involved.