This neoplasm is not reportable.

Name

Erdheim-Chester disease

Not Reportable

This neoplasm is not reportable

Alternate Names

ECD
Lipogranulomatosis
Lipoid granulomatosis
Lipid (cholesterol) granulomatosis
Polyostotic sclerosing histiocytosis

Definition

Erdheim-Chester disease (ECD) is a clonal systemic proliferation of histiocytes, commonly having a foamy (xanthomatous) component, and contain Touton giant cells (considered to be a non-Langerhans cell histiocytosis).

Abstractor Notes

Any organ or tissue can be infiltrated by ECD.

Characteristic features on imaging studies are bilateral and symmetrical osteosclerosis of the diaphyseal and metaphyseal parts of the long bones. Osteolytic lesions may also be seen. PET/CT has a high specificity for the diagnosis of bone involvement by ECD.

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.

For more information, see the Histiocytosis Association website (http://www.histio.org/page.aspx?pid=403#.V1cW-UYw0eU)

Definitive Diagnostic Methods

This data item does not apply

Genetics Data

This data item does not apply

Immunophenotyping

This data item does not apply

Corresponding ICD-9 Codes

202.9 Other and unspecified malignant neoplasms

Corresponding ICD-10 Codes

C96.7 Other specified malignant neoplasms of lymphoid, hematopoietic

Corresponding ICD-10-CM Codes (U.S. only)

C96.4 Sarcoma of dendritic cells (accessory cells) (effective October 01, 2015)

Diagnostic Exams

BRAF testing
CT (CAT) scan
PET (positron emission tomography) scan
MRI (magnetic resonance imaging)

Epidemiology and Mortality

Age: 55-60 median age, pediatric cases rare
Incidence: less 1000 cases reported, male predominance
Survival: 32 months average

Sources

Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J (Eds):
WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues (Revised 4th edition)
IARC: Lyon 2017
Section: Histiocytic and dendritic cell neoplasms
Pages: 481-482

National Cancer Institute
Section: General Information About Langerhans Cell Histiocytosis (LCH)
Pages: https://www.cancer.gov/types/langerhans/hp/langerhans-treatment-pdq#section/_178%20or%20http://www.cancer.gov/types/langerhans/patient/langerhans-treatment-pdq
Glossary