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This version published online on April 3, 2007
Journal of Clinical Endocrinology & Metabolism, doi:10.1210/jc.2006-2018
A more recent version of this article appeared on June 1, 2007
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*Adrenal Gland Disorders
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Submitted on September 14, 2006
Accepted on March 27, 2007

Bilateral Adrenal Infiltration in Erdheim-Chester Disease. Report of Seven Cases and Literature Review

Julien Haroche*, Zahir Amoura, Philippe Touraine, Danielle Seilhean, Claire Graef, Béatrice Birmelé, Bertrand Wechsler, Philippe Cluzel, Philippe A. Grenier, and Jean-Charles Piette

From Service de Médecine Interne, Service d'Endocrinologie et Médecine de la Reproduction, Service de Neuropathologie, and Service de Radiologie Hôpital Pitié-Salpêtrière, 47-83 Bld de l'Hôpital, 75013 Paris, France; Service de Néphrologie - Immunologie Clinique, Hôpital Bretonneau, 2 Boulevard Tonnelé, 37044 Tours, France

* To whom correspondence should be addressed. E-mail: julien.haroche{at}psl.aphp.fr.

Clinical case seminar

Context: 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: To describe adrenal and periadrenal infiltration in ECD.

Patients: 22 patients with ECD undergoing systematic computed tomography (CT) scan to search for signs of adrenal enlargement.

Results: Seven of the 22 (31.8%) patients with ECD displayed adrenal infiltration on CT scan. 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.






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