Retroperitoneal Erdheim-Chester disease without skeletal bone involvement mimicking uterine sarcoma with multiple organ involvement
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 vagin...
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| Format: | Article |
| Language: | English |
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Korean Society of Obstetrics and Gynecology
2020-07-01
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| Series: | Obstetrics & Gynecology Science |
| Subjects: | |
| Online Access: | http://www.ogscience.org/upload/pdf/ogs-19232.pdf |
| _version_ | 1819049181808951296 |
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| author | Hae Min Kim Gun Oh Chong Min Ju Kim Ji Young Park Yoon Hee Lee |
| author_facet | Hae Min Kim Gun Oh Chong Min Ju Kim Ji Young Park Yoon Hee Lee |
| author_sort | Hae Min Kim |
| collection | DOAJ |
| description | 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. |
| first_indexed | 2024-12-21T11:28:04Z |
| format | Article |
| id | doaj.art-21ab5d5ea1ea4785a276c914fc6de69d |
| institution | Directory Open Access Journal |
| issn | 2287-8572 2287-8580 |
| language | English |
| last_indexed | 2024-12-21T11:28:04Z |
| publishDate | 2020-07-01 |
| publisher | Korean Society of Obstetrics and Gynecology |
| record_format | Article |
| series | Obstetrics & Gynecology Science |
| spelling | doaj.art-21ab5d5ea1ea4785a276c914fc6de69d2022-12-21T19:05:37ZengKorean Society of Obstetrics and GynecologyObstetrics & Gynecology Science2287-85722287-85802020-07-0163453453710.5468/ogs.192328558Retroperitoneal Erdheim-Chester disease without skeletal bone involvement mimicking uterine sarcoma with multiple organ involvementHae Min Kim0Gun Oh Chong1Min Ju Kim2Ji Young Park3Yoon Hee Lee4 Department of Obstetrics and Gynecology, School of Medicine, Kyungpook National University, Daegu, Korea Department of Obstetrics and Gynecology, School of Medicine, Kyungpook National University, Daegu, Korea Department of Obstetrics and Gynecology, School of Medicine, Kyungpook National University, Daegu, Korea Department of Pathology, School of Medicine, Kyungpook National University, Daegu, Korea Department of Obstetrics and Gynecology, School of Medicine, Kyungpook National University, Daegu, KoreaErdheim-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.http://www.ogscience.org/upload/pdf/ogs-19232.pdferdheim-chester diseaseuterusureter |
| spellingShingle | Hae Min Kim Gun Oh Chong Min Ju Kim Ji Young Park Yoon Hee Lee Retroperitoneal Erdheim-Chester disease without skeletal bone involvement mimicking uterine sarcoma with multiple organ involvement Obstetrics & Gynecology Science erdheim-chester disease uterus ureter |
| title | Retroperitoneal Erdheim-Chester disease without skeletal bone involvement mimicking uterine sarcoma with multiple organ involvement |
| title_full | Retroperitoneal Erdheim-Chester disease without skeletal bone involvement mimicking uterine sarcoma with multiple organ involvement |
| title_fullStr | Retroperitoneal Erdheim-Chester disease without skeletal bone involvement mimicking uterine sarcoma with multiple organ involvement |
| title_full_unstemmed | Retroperitoneal Erdheim-Chester disease without skeletal bone involvement mimicking uterine sarcoma with multiple organ involvement |
| title_short | Retroperitoneal Erdheim-Chester disease without skeletal bone involvement mimicking uterine sarcoma with multiple organ involvement |
| title_sort | retroperitoneal erdheim chester disease without skeletal bone involvement mimicking uterine sarcoma with multiple organ involvement |
| topic | erdheim-chester disease uterus ureter |
| url | http://www.ogscience.org/upload/pdf/ogs-19232.pdf |
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