Disseminated juvenile xanthogranuloma

This neoplasm is not reportable.
Name
Disseminated juvenile xanthogranuloma
Not Reportable
This neoplasm is not reportable
Alternate Names
Benign cephalic histiocytosis
Deep JXG (if soft tissue involvement)
Generalized (non-lipidemic) eruptive histiocytosis
Progressive nodular histiocytosis
Xanthoma disseminatum (if skin and mucosal lesions)
Definition
This disease is characterized by a proliferation of histiocytes similar to those of the dermal JXG, commonly having a foamy (xanthomatous) component with Touton-type giant cells.

Solitary dermal (skin) JXG is more common then other forms and normally does not progress to disseminated forms. The majority of the disseminated forms occur by the age of 10 years, over half within the first 6 months of life.

For the adult form of this disease, see Erdheim-Chester disease.
Abstractor Notes
This is a benign disease that can have multiple lesions throughout the body. Lesions within the brain, dura or pituitary can be life threatening.

There is known associations with Neurofibromatosis type 1 (NF1). If patient has both JXG and NFI, they are at a higher risk of juvenile myelomonocytic leukemia (JMML).

Treatments used for LCH are usually used.