Kim Lucia, Choi Suk Jin, Park In Suh, Han Jee Young, Kim Joon Mee, Chu Young Chae, Kim Kyung Rae
Department of Pathology, Inha Research Institute for Medical Science, Inha University Hospital, Inha University College of Medicine, Incheon 400-711, South Korea.
Ann Diagn Pathol. 2008 Apr;12(2):128-33. doi: 10.1016/j.anndiagpath.2006.03.020.
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.
子宫内膜间质肉瘤（ESS）是一种罕见的子宫间叶肿瘤，主要由子宫内膜间质细胞组成。当在生殖器外区域遇到这种特征时，诊断有时会很困难，尤其是在不存在子宫内膜异位症的情况下。我们报告一例75岁女性小肠发生的ESS且无相关子宫内膜异位症的病例，并回顾了16例子宫外卵巢外ESS的文献。子宫外卵巢外ESS最常见的部位是胃肠道（8/16例）。与卵巢ESS的情况一样，它与子宫内膜异位症密切相关（12/16例）。大多数ESS对CD10（5/5例）、孕激素受体（10/10例）和雌激素受体（9/11例）呈免疫反应，对CD34呈阴性（0/7例）。与子宫内的ESS相比，它可能有更高的扩散到其起源部位以外的倾向（12/16例）。总之，在这些罕见情况下，仔细的形态学检查结合免疫组织化学研究并在鉴别诊断中考虑ESS将有助于获得准确的诊断。
