Cystic appearance of low-grade endometrial stromal sarcoma in the right atrium: case report

Abstract

A 71-year-old woman presented with a right adnexal solid mass invading the right gonadal vein and inferior vena cava up to the hepatic veins revealed by CT and confirmed by MRI. A thin-walled cyst and a solid mass were unexpectedly found in the right atrium by transesophageal echocardiography (TEE) in the operating room. Using color Doppler and air bubbles as contrast material a circumscribed cyst was confirmed and localized close to the IVC. The cyst was connected to the mass in the inferior vena cava. The tumor, including the cyst, was removed without using cardiopulmonary bypass and described as a low-grade endometrial stromal sarcoma, a rare slowly growing tumor. This is the first TEE description of endometrial stromal sarcoma manifesting as a right atrial cyst.

Figure 1

Visualization of the IVC tumor using contrast enhanced axial CT. Panel A: Dynamic contrast-enhanced axial CT scan through the suprahepatic IVC demonstrates central filling defect consistent with tumor or thrombus. Panel B: At an adjacent axial level, CT scan demonstrates a negative filling defect within the right atrium, suggesting the presence of an intra-atrial mass but the image is not conclusive for a cyst in the right atrium especially in comparison with the TEE images. IVC: inferior vena cava, RA: right atrium.

Figure 2

In a modified midesophageal bicaval view, a cyst was seen in the right atrium. Panel A: A cystic structure in the RA with a thin wall resulting in difficult visualization. Panel B: Color Doppler was used to confirm the existence of the cyst close to the IVC. Panel C: Bubble study, used to exclude PFO, confirmed the borders of the cyst and its position close to the IVC intersection. Panel D: A completely empty RA after the removal of the tumor. RA: right atrium, LA: left atrium, IVC: inferior vena cava.

Figure 3

A low-grade endometrial stromal sarcoma tumor divided into a solid and a tubular part. The solid part ended in a solid head and the tubular part ended in a cyst. Utilizing TEE, each individual part of the tumor can be seen on Panel A, B and C located in the IVC. Panel A: A solid tumor can be seen in the IVC. Panel B: The tumor started dividing into two parts. The origin of the solid (blue asterisk) and tubular (red asterisk) part can be seen. Panel C: Close to the IVC-RA intersection the tumor divided into a solid (blue asterisk) and a tubular (red asterisk) part. Panel D: The removed tumor divided into a solid (blue asterisk) and tubular (red asterisk) neck and a solid and cystic head. IVC: inferior vena cava, IVC-RA: inferior vena cava-right atrium.