Endometrial stromal sarcoma of the small bowel: a case report and review of literature

Kim, Lucia; Choi, Suk Jin; Park, In Suh; Han, Jee Young; Kim, Joon Mee; Chu, Young Chae; Kim, Kyung Rae

doi:10.1016/j.anndiagpath.2006.03.020

« Previous Next »Annals of Diagnostic Pathology
Volume 12, Issue 2 , Pages 128-133, April 2008

Endometrial stromal sarcoma of the small bowel: a case report and review of literature☆

Lucia Kim, MD
- Department of Pathology, Inha Research Institute for Medical Science, Inha University Hospital, Inha University College of Medicine, Incheon 400-711, South Korea
Suk Jin Choi, MD
- Department of Pathology, Inha Research Institute for Medical Science, Inha University Hospital, Inha University College of Medicine, Incheon 400-711, South Korea
In Suh Park, MD
- Department of Pathology, Inha Research Institute for Medical Science, Inha University Hospital, Inha University College of Medicine, Incheon 400-711, South Korea
Jee Young Han, MD
- Department of Pathology, Inha Research Institute for Medical Science, Inha University Hospital, Inha University College of Medicine, Incheon 400-711, South Korea
Joon Mee Kim, MD
- Department of Pathology, Inha Research Institute for Medical Science, Inha University Hospital, Inha University College of Medicine, Incheon 400-711, South Korea
- Corresponding author. Tel.: +82 32 890 3983; fax: +82 32 890 3464.
Young Chae Chu, MD
- Department of Pathology, Inha Research Institute for Medical Science, Inha University Hospital, Inha University College of Medicine, Incheon 400-711, South Korea
Kyung Rae Kim, MD
- Department of Surgery, Inha University Hospital, Inha University College of Medicine, Incheon 400-711, South Korea

Endometrial stromal sarcoma (ESS) is a rare mesenchymal neoplasm of the uterus, which is predominantly composed of endometrial stromal cells. When this feature is encountered in the extragenital area, the diagnosis is sometimes difficult especially if endometriosis is not present. We report a case of ESS arising in the small bowel without associated endometriosis in a 75-year-old woman and review the literatures for 16 cases of extrauterine extraovarian ESS. The most common site of the extrauterine extraovarian ESS is the gastrointestinal tract (8/16 cases). It is intimately associated with endometriosis (12/16 cases) as the case of ovarian ESS. Most ESSs were immunoreactive for CD10 (5/5 cases), progesterone receptor (10/10 cases), and estrogen receptor (9/11 cases), and negative for CD34 (0/7 cases). It may have a higher tendency for dissemination beyond its site of origin (12/16 cases) than its uterine counterpart. In conclusion, a careful morphological examination combined with immunohistochemical studies and consideration of ESS in the differential diagnosis would help in obtaining an accurate diagnosis in these rare circumstances.

Keywords: Endometrial stromal sarcoma, Small bowel, CD10