Very rare. Xanthogranulomatous tumor that is the result of systemic histiocytosis not originating from Langerhans cells. Onset usually occurs in adulthood, but pediatric cases have been reported. Oligoclonal proliferation of abnormal CD68+/CD1a histiocytes  with lipidic overload. Histology shows giant cells known as Touton's cells, with a pathognomonic foamy cytoplasm. Elevated levels of interferon α, IL-7 and IL-12. Probably due to mutations of the BRAF gene

The locations can be multiple: 

-         bones: lytic tumors, pathognomonic appearance of symmetric cortical osteosclerosis  of the long bones with  bone pain

-         skin: papulonodular lesions, xanthelasma 

-         central nervous system: retro-orbital tumor, dura mater infiltration, diabetes insipidus and /or pituitary insufficiency or hyperprolactinemia, 

-         digestive system: retroperitoneal infiltration that can result in hydronephrosis ('hairy kidneys')

-         cardiovascular system: pericarditis, renovascular hypertension

-         respiratory system: lung fibrosis with progressive dyspnea.

Treatment: corticosteroids, interferon, infliximab, vemurafenib, chemo-and/or radiotherapy.

Anesthetic implications: 

depending on tumor location; check blood electrolytes in case of diabetes insipidus; side effects of the treatments.

References : 

-         Hariharan U, Goel AV, Sharma D. 
Erdheim-Chester disease: clinical pearls for the anesthesiologist. 
Indian J Anaesth 2014; 30: 297-8.

-        Tran T-A, Fabre M, Pariente D, Craiu I, Haroche J et al.
Erdheim-Chester disease in childhood: a challenging diagnosis and treatment.
J Pediatr Hematol Oncol 2009 ; 31 : 782-6.

Updated: December 2016