A Rare Case of Synchronous Double Cardiac Leiomyosarcoma in the Left Atrium

Abstract

Primary cardiac tumors are rare and leiomyosarcoma is an even rarer entity with a reported incidence of <0.25% of all cardiac tumors. We demonstrate the need to include leiomyosarcoma on the differential when presented with an atrial mass on echocardiogram, which may guide intraoperative decision making. (Level of Difficulty: Intermediate.)

Primary cardiac tumors are rare and leiomyosarcoma is an even rarer entity with a reported incidence of <0.25% of all cardiac tumors…

A 49-year-old female presented to the emergency room with complaints of a chronic cough, 3 days of progressive dyspnea, and new-onset orthopnea. Her vital signs were unremarkable. A chest X-ray revealed vascular congestion suggestive of pulmonary edema. A transthoracic echocardiogram demonstrated a large echogenic mass in the left atrium measuring 5.5 × 4.3 cm. The stalk and origin of the tumor were not clearly visualized. Additionally, the mass prolapsed across the mitral annulus causing severe mitral stenosis with a mean pressure gradient of 22 mm Hg (Video 1). The patient was taken to the operating room. The left atrium was opened and 2 well-circumscribed masses were identified (Supplemental Figure). One mass had a stalk originating from the lateral wall and the second mass had a separate stalk in the medial wall. Both masses, including the stalks and bases, were resected completely and sent to pathology for further evaluation (Supplemental Figure). The patient recovered well. Histopathologic evaluation revealed low-grade leiomyosarcomas. She was referred to an oncologist and decision was made for surveillance given that further imaging did not show any residual lesion. She was contacted 4 months after surgery and has no evidence of recurrence.

Online Video 1.

Download video file ^{(10.9MB, mp4)}

Transthoracic echocardiogram with a large mass in the left atrium.

Primary cardiac leiomyosarcomas are rare and even fewer synchronous lesions have been reported. The incidence of primary cardiac tumors is 0.02%, and primary cardiac leiomyosarcomas represent <0.25% of all cardiac tumors (1,2). Although complete resection is not always feasible, surgical intervention is required for definitive tissue diagnosis, symptomatic relief, and prevention of related sequela such as embolic events. Synchronous cardiac leiomyosarcoma are rarely present in available reports. Although more than 1 tumor is unusual, this should be taken into preoperative consideration and resection of all tumors confers better outcomes.