Obstet Gynecol Sci.  2020 Jul;63(4):534-537. 10.5468/ogs.19232.

Retroperitoneal Erdheim-Chester disease without skeletal bone involvement mimicking uterine sarcoma with multiple organ involvement

• Kim HM
• Chong GO
• Kim MJ
• Park JY
• Lee YH

• KMID: 2505045
• DOI: http://doi.org/10.5468/ogs.19232

Abstract

Erdheim-Chester disease (ECD) is a rare type of non-Langerhans cell histiocytosis and is characterized by the diffuse histiocytic infiltration of multiple organs. Retroperitoneal ECD, especially with uterine involvement, is extremely rare. We report about a 73-year-old woman who presented with vaginal spotting for a month and experienced abdominal pain along with pus-like urine. Computed tomography revealed an irregular mass-like lesion in the uterus, possibly a uterine sarcoma, invading the ureter, rectosigmoid, and bladder. A tissue biopsy of the retroperitoneal mass revealed typical morphological and immunohistochemical features of ECD. However, clinical features, especially long bone involvement, did not coincide with ECD, and BRAF V600E gene mutation was not detected. We made a diagnosis of atypical retroperitoneal ECD mimicking uterine sarcoma with multiple organ involvement.

Keyword

Erdheim-Chester disease; Uterus; Ureter

Figure

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  Fig. 1. (A) Magnetic resonance image showing a lobulated irregular mass (white arrow) at the left side of the uterus (U: uterus). (B) Magnetic resonance image showing bladder invasion (white arrow) and rectosigmoid invasion (yellow arrow). (C) Computed tomographic image showing severe hydronephrosis and hydroureter (white arrow).

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  Fig. 2. (A) Image of histopathologic features of the uterus demonstrating clear and foamy histiocytes (hematoxylin and eosin stain, ×200). (B) Immunohistochemical staining image demonstrating the strong positive reactivity of histiocytic markers (CD68, ×40).

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