Intravenous Endometrial Stromal Sarcoma: a case report

Reprint requests to: Dr. Wen-Ko Su Department of Radiology, Hsinchu Mackay Memorial Hospital. No. 690, Sec. 2, Guang Fu Road, Hsinchu 30071, Taiwan, R.O.C. Endometrial stromal sarcoma (ESS) is a rare mesenchymal neoplasm that usuallyoccurs as a primary tumor of the uterus. Endometrial stromal sarcomas may occasionally arise in other sites, such as the ovary, mesentery, and vagina. We report an endometr ial stromal sarcoma arising from the left ovarian vein after a hysterectomy 29 years earlier because of uterine myomas. To the best of our knowledge, it is extremely rare for endometrial stromal sarcoma to grow in the great vessels, especially the left ovarian vein. Even though this tumor is rare, it should be included in the differential diagnosis when a longitudinal mass is seen in the left lower abdomen of a female patient. Therefore, the correct preoperative diagnosis can be established. Endometrial stromal sarcoma (ESS) is a rare malignant mesenchymal tumor that usually develops in the uterus and occasionally arises at various extrauterine sites [1, 2]. The overall five-year survival rate for ESS exceeds eighty percent. Approximately fifty percent of the patients, however, have tumor recurrence, mostly after a long latency period. Our case is a female patient who has undergone a hysterectomy 29 years earlier and an extrauterine ESS in the left ovarian vein is found. It is diff icult to determine whether the tumor is a primary extrauterine ESS or a late recurrence of ESS. We report this case and review of the literature.