Yano Yoko, Yamasaki Yui, Yamanaka Keitaro, Nishimoto Masashi, Nagamata Satoshi, Terai Yoshito
Department of Obstetrics and Gynecology, Kobe University Graduate School of Medicine, 7-5-1 Kusunoki-cho, Chuo-ku, Kobe, Hyogo 650-0017, Japan.
Department of Obstetrics and Gynecology, Kobe University Graduate School of Medicine, 7-5-1 Kusunoki-cho, Chuo-ku, Kobe, Hyogo 650-0017, Japan.
Int J Surg Case Rep. 2023 Oct;111:108857. doi: 10.1016/j.ijscr.2023.108857. Epub 2023 Sep 21.
A case of Low-grade endometrial stromal sarcoma (LG-ESS) invading the great vessels is rare.
A 34-year-old female who had no past history presented to a previous hospital with abdominal distension. Magnetic resonance imaging revealed a 15 cm pelvic mass beside the uterus, and only the pelvic mass was removed at the surgery. The tumor was judged to be a LG-ESS. The patient chose to be observed to preserve her fertility, and no adjuvant treatment was undertaken. Two years later, she was referred to our hospital due to recurrence of the pelvic mass. Enhanced computed tomography revealed a large tumor in the vena cava which extended from the left internal iliac vein and which originated from the pelvic tumor. An operation was performed by a multidisciplinary team. Complete resection of the tumor was achieved with a radical hysterectomy, bilateral salpingo-oophorectomy, removal of recurrent pelvic masses and the intravascular tumor. We diagnosed a recurrence of LG-ESS. She received a postoperative adjuvant therapy of LG-ESS.
Patients with fertility-sparing treatment had higher recurrence rates. In cases of tumor intravenous extension, we should make every effort to extract the tumor to avoid sudden death.
This case highlights the importance of a multidisciplinary approach in treating this rare tumor with intravascular extension. In particular, patients with LG-ESS who receive fertility-sparing surgery should undertake postoperative chemotherapy or radiotherapy in order to reduce the risk of recurrence, as was in this case.
低级别子宫内膜间质肉瘤（LG-ESS）侵犯大血管的病例罕见。
一名既往无病史的34岁女性因腹胀就诊于之前的医院。磁共振成像显示子宫旁有一个15厘米的盆腔肿块，手术仅切除了盆腔肿块。肿瘤被判定为LG-ESS。患者选择观察以保留生育能力，未进行辅助治疗。两年后，因盆腔肿块复发转诊至我院。增强计算机断层扫描显示下腔静脉有一个大肿瘤，该肿瘤从左髂内静脉延伸而来，起源于盆腔肿瘤。由多学科团队进行了手术。通过根治性子宫切除术、双侧输卵管卵巢切除术、切除复发性盆腔肿块和血管内肿瘤，实现了肿瘤的完全切除。我们诊断为LG-ESS复发。她接受了LG-ESS的术后辅助治疗。
保留生育功能治疗的患者复发率较高。对于肿瘤静脉内扩展的病例，我们应尽一切努力摘除肿瘤以避免猝死。
本病例凸显了多学科方法在治疗这种伴有血管内扩展的罕见肿瘤中的重要性。特别是，接受保留生育功能手术的LG-ESS患者应进行术后化疗或放疗，以降低复发风险，本病例即是如此。
