Surgical management of intravascular leiomyomatosis

Abstract

Intravascular leiomyomatosis is a rare entity defined by benign smooth uterine muscle cells that typically originate from the uterus with the potential to spread into veins possibly up to the heart. The diagnosis for patients presenting with cardiac symptoms may be difficult and imaging often interpreted as thrombus or atrial myxoma.

Keywords: Cardiovascular surgery; Gynecology; Heart disease; Intravascular; Leiomyomatosis; Tumor.

Fig 1

(A-C) Axial image of an abdomen and Pelvis computed tomography scan at the level of the right atrium, inferior vena cava (IVC), and right ovarian vein. Intraluminal material visualized within the IVC consistent with intravascular tumor (yellow arrows). (D) Coronal image of abdomen and pelvis CT scan at the level of the IVC. Intravascular leiomyomatosis (IVL) is marked with a yellow arrow.

Fig 2

Intravascular leiomyomatosis (IVL) mass extracted after surgery, 50 cm, with the intracardiac (blue star) and intravascular (blue arrows) parts.

Fig 3

(A) Low magnification showed a spindle cell tumor filling the lumen of a large vein (stain: hematoxylin and eosin; original magnification ×2). (B) The tumor consisted of bland spindle cells without atypia or significant mitotic activity, similar in appearance to those of the vessel's media (stain: hematoxylin and eosin; original magnification ×20). (C) Caldesmone. The smooth muscle marker caldesmone showed staining by immunhistochemistry on both the tumor cell and the media of the vessel, immunohistochemical staining for caldesmone (original magnification ×20). (D) Staining for estrogen receptor (ER) showed positivity in the nuclei of tumoral cells but not those of the vessel's media, immunohistochemical staining for ER (original magnification ×20).

Fig 4

Axial and coronal computed tomography (CT) scans of the patient's inferior vena cava (IVC) and heart chambers at the 9-month follow-up.