Erdheim-Chester disease

This neoplasm is not reportable.
Name
Erdheim-Chester disease
Not Reportable
This neoplasm is not reportable
Definition
This is a rare form of non-Langerhans histiocytosis. This disease produces excessive amounts of histiocytes, which are white blood cells.

Average onset of this disease is in the mid 50's. It is similar to langerhans in that the most common sites are the bones; however, it can occur in other sites as well.

Source: Histiocytosis Association, Inc.
http://www.histio.org/page.aspx?pid=403#.VH3NKskhCo0
Abstractor Notes
Erdheim-Chester disease can be mistaken for Langerhans cell histiocytosis. Pathologic confirmation of the disease will need to be done to determine between Langerhans cell histiocytosis and Erdheim-Chester disease.

Although no treatment plans have been established, treatments for this disease include: steroids, immunotherapy, chemotherapy, radiation and surgery.