Endometrial stromal sarcoma of the small bowel arising in a 75-year-old woman : A case report

초록

Endometrial stromal sarcomas arising in the ovary or in extraovarian sites in the absence of a primary uterine lesion are extremely rare, and about 70 cases are reported in the literature. Most of the extrauterine endometrial stromal sarcoma originates from endometriosis and very small proportion of the tumor arises from the metaplasia of the pelvic peritoneal surface. We report a case of endometrial stromal sarcoma arising in the small bowel without associated endometriosis in a 75 year-old-woman. The patient was 75 year-old-female who complained of abdominal pain and palpable abdominal mass detected before the one year. She had undergone a total hysterectomy for uterine myoma 38 years ago. On operation, a polypoid mass protruding into the lumen and a large serosal mass were present in the jejunum and multiple nodular masses were found in the omentum and mesentery of small bowel, It showed partly encapsulated gray-white, lobulated, and soft cut surface with multiple small daughter nodules invading into the fibrous capsule and small bowel wall. Endometriosis was not identified in or adjacent to the masses although thorough examination. Microscopically, the masses were composed of sheets of closely packed uniform cells forming short fascicles with a tongue-like nodular growth at the periphery of the large mass. Numerous small thick-walled blood vessels resembling spiral arterioles of late secretory endometrium were uniformly distributed among the stromal cells, On immunohistochemical staining, the tumor cells showed diffuse immunoreactivity for CD10, estrogen receptor and progesterone receptor, focal positivity for CD34, and negative staining for cytokeratin, CD117 and S-100 protein. Limited smooth muscle differentiation was occasionally present in the large mass, and was positive for desmin and smooth muscle actin. The diagnosis of endometrial stromal sarcoma arising in the small bowel was made.