Treatment of Intravenous Leiomyomatosis with Cardiac Extension following Incomplete Resection

Abstract

Aim. Intravenous leiomyomatosis (IVL) with cardiac extension (CE) is a rare variant of benign uterine leiomyoma. Incomplete resection has a recurrence rate of over 30%. Different hormonal treatments have been described following incomplete resection; however no standard therapy currently exists. We review the literature for medical treatments options following incomplete resection of IVL with CE. Methods. Electronic databases were searched for all studies reporting IVL with CE. These studies were then searched for reports of patients with inoperable or incomplete resection and any further medical treatments. Our database was searched for patients with medical therapy following incomplete resection of IVL with CE and their results were included. Results. All studies were either case reports or case series. Five literature reviews confirm that surgery is the only treatment to achieve cure. The uses of progesterone, estrogen modulation, gonadotropin-releasing hormone antagonism, and aromatase inhibition have been described following incomplete resection. Currently no studies have reviewed the outcomes of these treatments. Conclusions. Complete surgical resection is the only means of cure for IVL with CE, while multiple hormonal therapies have been used with varying results following incomplete resection. Aromatase inhibitors are the only reported treatment to prevent tumor progression or recurrence in patients with incompletely resected IVL with CE.

Figure 1

(a) Hypodense filling defect in the IVC on CT and (b) free-floating echodensity at the level of the hepatic veins, both consistent with intracaval thrombus.

Figure 2

(a) Right and left pulmonary artery filling defects on CTPA and (b) TOE demonstrating the head of the mass protruding from the IVC into the RA. The TV was not damaged although the mass protruded through the TV into the RV during diastole. IVC: inferior vena cava; RA: right atrium; TV: tricuspid valve; RV: right ventricle.

Figure 3

(a) A thin, white mass in the RA with its attachment extending down the IVC and (b) the gross specimen of the intracardiac tumor. (c) Smooth muscle actin antibody stain positive in the tumor specimen.

Figure 4

(a) CT axial and (b) coronal views of the IVL in the right common iliac vein (red arrows). (c) Axial and (d) coronal views of the pelvic component of the tumor (red arrows), abutting the dilated common iliac vein (yellow arrow). (e) Axial T2-weighted sequence on MRI identifying the intraluminal caval tumor with (f) associated pelvic mass on coronal image.

Figure 5

(a) Gross specimen of the tumor resected from the IVC venotomy during second-stage procedure and (b) pelvic mass with venous attachment site (yellow arrow) with (c) strongly positive staining of IVC tumor specimen for estrogen receptors.