Bilateral adrenal infiltration in Erdheim-Chester disease. Report of seven cases and literature review.
BACKGROUND
Erdheim-Chester disease (ECD) is a rare non-Langerhans form of histiocytosis characterized by xanthomatous tissue infiltration with CD68(+) CD1a(-) foamy histiocytes. Endocrine problems, such as diabetes insipidus and hypogonadotropic hypogonadism, frequently occur in ECD, but bilateral adrenal infiltration has rarely been reported in this disease.


OBJECTIVE
The aim of the study was to describe adrenal and periadrenal infiltration in ECD.


METHODS
A total of 22 patients with ECD undergoing systematic computed tomography to search for signs of adrenal enlargement participated in the study.


RESULTS
Of the 22 patients with ECD, seven (31.8%) displayed adrenal infiltration on computed tomography. In one case, autopsy confirmed that the adrenal enlargement was due to foamy histiocyte infiltration in the adrenal glands. Possible adrenal insufficiency was assessed in five of the seven patients. One developed signs of adrenal insufficiency, which was confirmed by adrenocorticotropin stimulation tests. Adrenal involvement was reported in only 15 of the 240 ECD cases published up to May 2006. This frequency is significantly lower than that in our series (P = 0.0008; Fisher's exact test).


CONCLUSIONS
Physicians should be aware of ECD as a possible cause of morphological changes in adrenal size and infiltration.
Haroche
Julien
J
Service de Médecine Interne, Hôpital Pitié-Salpêtrière, 47-83 Bld de l'Hôpital, Paris, France. julien.haroche@psl.aphp.fr
Amoura
Zahir
Z
Touraine
Philippe
P
Seilhean
Danielle
D
Graef
Claire
C
Birmelé
Béatrice
B
Wechsler
Bertrand
B
Cluzel
Philippe
P
Grenier
Philippe A
PA
Piette
Jean-Charles
JC
D000307:Adrenal Gland Diseases / Q000000981:diagnostic imaging* / Q000473:pathology*; D000311:Adrenal Glands / Q000000981:diagnostic imaging / Q000473:pathology; D000328:Adult; D000368:Aged; D031249:Erdheim-Chester Disease / Q000000981:diagnostic imaging* / Q000473:pathology*; D017809:Fatal Outcome; D005260:Female; D006644:Histiocytes / Q000473:pathology; D006801:Humans; D008297:Male; D008875:Middle Aged; D014057:Tomography, X-Ray Computed