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

Disseminated juvenile xanthogranuloma (JXG) 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. There is evidence for clonality in some instances.

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

The skin and soft tissues are affected most commonly. Disseminated forms commonly affect the mucosal surfaces, in particular with the upper aerodigestive tract.

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.

For more information, see the Histiocytosis Association website (http://www.histio.org/page.aspx?pid=391)

Immunophenotyping

This data item does not apply
Glossary