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

Abstract

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.

Multiple Nervous System Involvement of Erdheim-Chester Disease With Responsive Perinephritic Hypermetabolism

“…2,3 18 F-FDG PET/CT is the method of choice for the determination of the lesions of Erdheim-Chester disease (ECD) and the discrimination of the ECD with its related form including Langerhans cell histiocytosis. 5 In addition, 18 F-FDG PET/CT has proven to be feasible in response evaluation of the lesions of ECD. 6 This is a report of a case with multiple central nervous system and peripheral nervous system involvement as well as perinephritic specific sign.…”

“…However, some of the central nervous system lesions and most of the perinephritic lesions do not show FDG uptake 2,3 . 18 F-FDG PET/CT is the method of choice for the determination of the lesions of Erdheim-Chester disease (ECD) and the discrimination of the ECD with its related form including Langerhans cell histiocytosis 5 . In addition, 18 F-FDG PET/CT has proven to be feasible in response evaluation of the lesions of ECD 6 .…”