Abstract
We present a case of a 46-year-old female presenting with syncope. Echocardiography initially showed a right atrial mass. Further evaluation revealed a mass arising from the fundus of the uterus, with a tumor thrombus in the left gonadal vein, extending into the left renal vein and through the inferior vena cava (IVC) into the right heart across the tricuspid valve. She was managed with surgical resection, and postoperative pathology was consistent with intravenous leiomyomatosis (IVL). IVL is a rare uterine smooth muscle cell neoplasm which extends into the venous system. Gynecological tumors are often overlooked in differential diagnosis for atrial masses. A benign tumor like fibroid, in rare circumstances, can extend into the right side of the heart causing dynamic obstruction to outflow tract, thus increasing mortality. The objective of this article is to present such a case and highlight the broad differentials of atrial masses, including IVL.
Highlights
Primary cardiac tumors are rare, with an incidence of less than 0.1% [1]
An echocardiogram revealed a mobile echogenic mass in the right atrium extending from the inferior vena cava (IVC) and moving across the tricuspid valve, causing dynamic obstruction of the right atrial and tricuspid flow (Figure 1)
intravenous leiomyomatosis (IVL) is a rare uterine neoplasm comprised of benign smooth muscle cells, which by extension grows within the intrauterine and extrauterine venous system [3]
Summary
Primary cardiac tumors are rare, with an incidence of less than 0.1% [1]. They include benign tumors like myxoma, lipoma, and papillary fibroelastoma as well as malignant tumors like sarcoma, rhabdomyosarcoma, lymphoma, and pericardial mesothelioma. An echocardiogram revealed a mobile echogenic mass in the right atrium extending from the inferior vena cava (IVC) and moving across the tricuspid valve, causing dynamic obstruction of the right atrial and tricuspid flow (Figure 1). She was promptly started on intravenous heparin for possible thrombus. Pre-surgical differential diagnosis included atypical leiomyoma, sarcoma, or metastatic ovarian tumor She was transferred to a higher center for surgery, and underwent a single-stage operation which included exploratory laparotomy with total abdominal hysterectomy/bilateral salpingo-oophorectomy by gynecology, left gonadal vein resection, and tumor thrombectomy with vascular reconstruction per urologyoncology, and removal of the right atrial and ventricular tumor along with IVC tumor with cardiopulmonary bypass by cardiothoracic surgery. She remains disease-free for a year and a half since diagnosis
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