Corbett Gillian A, O'Gorman Catherine, Kamran Waseem
Department of Gynaecological Surgery, Saint James's Hospital, Dublin, Ireland
Department of Gynaecological Surgery, Saint James's Hospital, Dublin, Ireland.
BMJ Case Rep. 2020 Mar 12;13(3):e233341. doi: 10.1136/bcr-2019-233341.
Intravenous leiomyomatosis is extremely rare. This case describes a 42-year-old woman who presented with abdominal distension, cyclical bloating and urinary retention. Preoperative imaging showed a multilobulated uterine mass. Following multidisciplinary team discussion, a complete staging surgery consisting of midline laparotomy, total hysterectomy and bilateral salpingo-oophrectomy was performed. Intraoperatively, a large multilobulated uterine mass was noted with engorgement of the infundibulopelvic ligaments due to intravascular extension of tumour. On removal of the uterus, the patient desaturated and became hypotensive. Intraoperative transoesophageal echocardiography revealed mass extending from the inferior vena cava (IVC) into the right atrium (RA). The cardiothoracic surgical team retrieved a worm-like mass extending from the IVC into the RA. Histopathological examination diagnosed a large uterine leiomyoma with intravenous leiomyomatosis. The mass from the RA was a bland spindle cell tumour which matched the uterine mass histopathologically. Intravenous leiomyomatosis is a rare variant of uterine leiomyoma. Although intracardiac extension has been described, this is the first case of intraoperative embolisation of pelvic tumour to the RA at hysterectomy.
静脉内平滑肌瘤病极为罕见。本病例描述了一名42岁女性，她出现腹胀、周期性腹部膨隆和尿潴留。术前影像学检查显示子宫有一个多叶状肿块。经过多学科团队讨论后，进行了包括中线剖腹术、全子宫切除术和双侧输卵管卵巢切除术的完整分期手术。术中发现一个大的多叶状子宫肿块，由于肿瘤血管内延伸导致漏斗骨盆韧带充血。切除子宫时，患者出现血氧饱和度下降和低血压。术中经食管超声心动图显示肿块从下腔静脉延伸至右心房。心胸外科团队取出了一个从下腔静脉延伸至右心房的蠕虫状肿块。组织病理学检查诊断为伴有静脉内平滑肌瘤病的大子宫平滑肌瘤。来自右心房的肿块是一个形态温和的梭形细胞瘤，其组织病理学与子宫肿块相符。静脉内平滑肌瘤病是子宫平滑肌瘤的一种罕见变体。尽管已有心脏内延伸的描述，但这是首例在子宫切除术中盆腔肿瘤术中栓塞至右心房的病例。
