Case report of an intracaval leiomyomatosis 10 months after complete hysterectomy

INTRODUCTION: Intravenous leiomyomatosis (IVL) is a rare smooth muscle tumor, usually found in women with tumors of the reproductive organs, such as uterus myomatosous. Surgically, this case belies the call for sternotomy and two-stage surgery in caval IVL extending to the right atrium: we suggest one-stage median laparotomy as a minimal procedure with maximal benefit. PRESENTATION OF CASE: We present the case of a 60-year-old postmenopausal woman with suspected intravenous leiomyomatosis of the right internal iliac vein. The patient had undergone hysterectomy and bilateral adnexectomy for uterus myomatosous in September 2015, where an IVL limited to the veins of the uterus and the right adnex had been diagnosed. No further medical treatment had been implemented. IVL of the inferior vena cava was diagnosed when a CT scan of the abdomen was performed due to an infected abdominal seroma in June 2016. Although histologically benign, we found this case of IVL to be clinically aggressive because of its expansion to the heart. This may lead to thromboembolic complications (e.g. pulmonary embolism) or signs of right sided cardiac failure. The patient was asymptomatic, but because of the extension of the intracaval thrombus to the heart, we decided to operate and performed thrombectomy via a median laparotomy. The patient left the hospital shortly after on newly started oral anticoagulation. DISCUSSION: For caval IVL without intracardiac attachment, the extraction via laparotomy without sternotomy is the treatment of choice. It calls for an interdisciplinary approach and careful surgical planning. CONCLUSION: There is no inherent need for sternotomy in IVL extending to the right atrium. A one-year follow-up with sonographic control is advised. Medium term oral anticoagulation should be considered. This work has been reported in line with the SCARE criteria Agha et al. (2016). The SCARE Statement: Consensus-based surgical case report guidelines. Agha RA, Fowler AJ, Saeta A, Barai I, Rajmohan S, Orgill DP; SCARE Group. Int J Surg. 2016 Oct;34:180-186 [1].