Erdheim-Chester disease - orbits

Case contributed by Assoc Prof Frank Gaillard

Orbits

CT

Intraconal, well-defined, and enhanced masses. 

Orbits

MRI

Intraconal well-defined masses: hypointense signal on both T1 and T2-weighted images and intense enhancement on T1 post contrast. 

Case Discussion

Unusual case of Erdheim-Chester Disease localized to the orbits. It has remained stable over more than 18months.

Histology Final Diagnosis:

These biopsies show xanthogranulomatous inflammation. There is no evidence of necrotizing granulomatous inflammation or lymphoma. Xanthogranulomatous inflammation of the orbit is noteworthy in that it may be seen in association with underlying hematolymphoid neoplasia.

Immunoperoxidase stains were done on the specimen there and showed the following results: L-26 (B cells) -focal staining of a lymphoid follicle, CD3 (T cells) -moderate number scattered throughout specimen, CD68 (histiocytes) -large number throughout specimen, CD1a -negative.

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