Uterine Intravenous Leiomyomatosis with Cardiac Extension: Radiologic Assessment with Surgical and Pathologic Correlation

Abstract

We present the computed tomography (CT) and magnetic resonance (MR) imaging findings of a 71-year-old woman with a cardiac extension of intravenous leiomyoma (IVL) that arose from the uterus, extended to the inferior vena cava (IVC), and reached the right ventricle through the right ovarian vein. Radiologic-pathologic correlation showed that the intravascular cord-like mass originating from the IVC and extending to the right ventricle was composed of degenerated smooth muscle cells with a number of large vessels that were regarded as arteries; moreover, the arteries within the cord-like mass appeared to be looping internally. Given the disappearance of the right ovarian venous wall around the IVL pathologically, extracting the tumor from the ovarian vein during an operation is considered to be impossible retrospectively. Also it was difficult to identify even the intravenous extension of the uterine leiomyoma histopathologically. Therefore, contrast-enhanced CT, in particular arterial phase imaging, provided important information that revealed the mass, range, and path of the lesion, ensuring that an appropriate operative plan could be drawn up and the tumor completely excised.

Benign Metastasizing Leiomyoma of the Heart

“…Previous studies have reported these tumors often stain positively for both estrogen and progesterone receptors [8][9][10][11][12]. Our patient's cardiac mass was 95% and 20% immuno-reactive for estrogen and progesterone, respectively, consistent with a uterine origin.…”

“…However, this study found no significant relationship between the type of primary surgery and BML diagnosis with respect to timing of surgery or site of metastasis. Three out of the four other recorded cases of BML to the heart had multiple lung metastases [7][8][9][10][11][12]. In these case reports, BML was found in the right atrium, interventricular septum, tricuspid valve and right ventricle [8].…”

A Case of Syncope Due to Intracardiac Leiomyomatosis

“…This is followed by CT with angiography, which gives detailed information regarding the complete path of the lesion. This can be seen as worm-like tumor extension through the ovarian or uterine vein, into the IVC, and ultimately into the right heart [ 8 ]. Complete surgical resection, when possible, is the treatment of choice to prevent a recurrence.…”

Intravascular leiomyomatosis with intracardiac extension: a toraco-abdominal approach

“…There are two main theories which attempt to explain the etiology of uterine IVL: mural origin of the tumor from the venous walls and direct vascular invasion by a primary tumor arising from an uterine leiomyoma [ 10 ]; the second theory is favored in the literature for the following reasons: (a) patients have a history of uterine myoma or have undergone myomectomy or hysterectomy; (b) tumor bases were often connected to the uterine wall on pathological and imaging examination; (c) tumor cells tested positive for estrogen- and progesterone-receptors [ 11 ].…”