Surgical treatment strategies for extra-pelvic intravenous leiomyomatosis

Abstract

Background: Extra-pelvic intravenous leiomyomatosis (IVL) extending into inferior vena cava (IVC) or heart (i.e. intracardiac leiomyomatosis, ICL) is an extremely rare benign disease. No consensus has been reached on the optimal surgical strategy. The aim of this study is to introduce four types of one-stage surgical strategies including less invasive options and a guideline to select patient-specific strategy for this disease. Methods: Twenty-four patients of extra-pelvic IVLs receiving one-stage resections at the Zhongshan Hospital from July 2011 to November 2019 were reviewed retrospectively. Base on the initial experiences of the indiscriminate choices of tumor thrombectomies through sterno-laparotomy under cardiopulmonary bypass (CPB) in 6 ICLs, an anatomy-based guideline for four types of surgical strategies was developed and applied for the next 18 patients. Results: Under the direction of guideline, tumor thrombectomies through single laparotomy were applied without CPB in 2 ICLs and 4 IVLs confined in IVC, or with CPB in 7 ICLs. Guideline-directed double-incisions with CPB were applied in only 5 ICLs, including 1 receiving mini-thoracotomy and 4 receiving sternotomy because of tumor adherences with right atriums in 2 and with pulmonary arteries in 2. All 24 patients accomplished one-stage panhysterectomy, bilateral adnexectomy and complete resections of intracaval and intracardiac tumors. For residual pelvic intravenous tumors in 19 patients, 17 received macroscopically complete resections while the other 2 failed because of high risk of hemorrhage. Intraoperative blood losses, operation time and hospitalization expense in the single-laparotomy non-CPB group were significantly lesser than the other groups. In CPB groups, inpatient stay and hospitalization expense in the single-incision group were significantly lesser than the double-incisions group. All patients were alive and free of recurrences during a mean follow-up of 35.4 ± 27.2 months (range, 1-100 months). The pelvic tumor residues in 2 patients remained unchanged for 51 and 52 months since operation, respectively.

Unraveling the challenges of intravenous leiomyomatosis: a retrospective analysis of 11 cases

“…However, it’s worth noting that pelvic ultrasound can only assess the pelvic veins, and its accuracy often depends on the experience of the sonographer. Enhanced CT or MRI is more helpful in evaluating the internal iliac-common iliac-inferior vena cava venous system, assessing the extent of the lesion, and determining operative procedures [ 14 , 15 ]. Clinicians can interpret these images themselves, eliminating the need to rely solely on the sonographer’s diagnosis.…”

Intravenous Leiomyomatosis Complicated by Arteriovenous Fistula: Case Series and Literature Review

“…This discrepancy could be attributable to the bias introduced by the short sample size. Nevertheless, the ligation of the bilateral internal iliac vein and ovarian vein is helpful to prevent the recurrence or shedding of residual tumor embolus ( 26 ). Patients who have a pelvic tumor resection are more likely to experience complications than patients who do not have a pelvic tumor resection ( Table 3 ), which could be owing to the abundance of the pelvic vascular bed, or possibly the creation of tumor-related arteriovenous malformations.…”

One-stage resection of intravascular leiomyomatosis involving the right heart chamber through a single laparotomy

“…Intravenous leiomyomatosis (IVL) is a rare benign leiomyoma and a specific type of uterine leiomyoma (UM). It usually originates in the uterus and extends to the extrauterine venous system or even the heart and pulmonary arterial system ( 1 , 2 ). IVL extending into the cardiac cavity is called intracardiac leiomyomatosis (ICL).…”

“…The current understanding of ICL is mainly based on case reports and case series; its clinical presentation is non-specific and includes right heart obstruction, pulmonary embolism and even sudden death ( 3 ). Surgical resection of extrapelvic IVL is the current gold standard treatment, and different surgical centers have reported various surgical strategies ( 2 ). At present, consensus on the best surgical strategy for IVL, which simultaneously involves the extrauterine vein, inferior vena cava (IVC) and intracardiac cavity, is lacking.…”