We present a case of a young female with stroke symptoms who underwent valve sparing resection of a presumed fibroelastoma based on echocardiographic findings. After confirming embolic stroke, she underwent excision of the lesion, which on pathology revealed a nonbacterial thrombus. Ultimately, this led to a more extensive work-up leading to the discovery of a papillary serous ovarian carcinoma, the underlying cause of her hypercoagulable state. The initial echocardiographic findings painted the clear picture of a papillary tumor on the aortic valve which was likely the source of the emboli resulting in ischemic stroke. This unique case presentation illustrates that imaging, including echocardiography, may not always coincide with the clinical diagnosis. Thus, understanding the differential diagnoses of cardiac masses is of vital clinical significance. The distinction of fibroelastoma versus the much less common finding of aortic thrombus may lead to early diagnosis of malignancy and prevention of life threatening events due to stroke or undiagnosed disease.
The incidence of primary cardiac tumors is rare with the top three most common tumors consisting of myxomas, lipomas, and fibroelastomas (in order of respective occurrence) [
Echocardiography remains the cornerstone for identification of cardiac tumors, but the diagnosis based on imaging is not always definitive. Cardiac masses have variations in shape and size making it difficult to diagnose a tumor versus a thrombus based on imaging findings and clinical presentation. The sensitivity and specificity have been estimated to be near 90% when using transthoracic echocardiography in diagnosis of papillary fibroelastoma if tumor size is greater than 2 mm [
A 26-year-old previously healthy female presented to emergency department with complaints of headaches for two weeks associated with right sided numbness, weakness, diplopia, facial droop, dysarthria, and ataxia. The patient underwent work-up for a cerebral vascular accident. MRI confirmed the diagnosis of acute stroke involving the left posterior cerebral artery territory with smaller foci of ischemia in various cerebral territories, consistent with embolic phenomena. Transesophageal echocardiography revealed a large, papillary, solid, fixed mass, measuring 7 × 7 mm on the left coronary cusp (Figure
Transesophageal echocardiograph illustrating large, papillary, solid, fixed mass, measuring 7 × 7 mm on the left coronary cusp, on the left ventricular aspect of the aortic valve.
Mass surgically excised from left coronary cusp of aortic valve, approximately 9 × 5 mm. Pathology consistent with thrombus.
Four months after the initial surgery she presented with complaints of left lower quadrant abdominal pain, described as sharp and stabbing in nature. CT scan of the abdomen and pelvis revealed massive ascites. Recommended correlation study with transvaginal ultrasound was conducted and revealed a complex mass of mixed echogenicity with mildly increased vascularity measuring 7.6 × 7.2 × 9.0 cm, located posterior to the uterus and extending to the right adnexa. Biopsy of the lesion revealed adenocarcinoma. She went on to undergo bilateral salpingooophorectomy, omentectomy, lysis of adhesions, and peritoneal biopsies confirming a final diagnosis of papillary serous ovarian carcinoma, Stage IIc.
Stroke-like symptoms in otherwise healthy young adults can be very alarming. Initial assessment involves multiple brain imaging modalities before a suspected diagnosis is made, as strokes can be ischemic or hemorrhagic in nature. Cardiac emboli are a common source of ischemic stroke and may be the result of bacterial or nonbacterial thrombi, or cardiac tumors. Due to the vast differential of cardiac masses, assessment and diagnosis pose a challenging task for physicians, especially cardiac tumors. Nonneoplastic processes such as thrombus and valvular vegetations can mimic tumors; therefore physicians should consider them as part of the differential diagnosis.
Cardiac tumors can be categorized into primary cardiac neoplasms or metastatic deposits from other remote primary neoplasms; most commonly breast, lung, lymphomas, melanomas, and renal tumors [
The occurrence of primary cardiac neoplasms is rare and diagnosed in about 0.001–0.03% of echocardiographic studies, reported by Sutsch et al., 1991, based an analysis of 20,305 echocardiographs [
Treatment of cardiac masses normally depends on the clinical presentation and type of tumor. There is no defined protocol nor evidence based approach to treating these patients; thus clinical judgement in coordination with imagery must be utilized. Benign tumors, including papillary fibroelastomas, are commonly surgically excised due to the high risk of embolic phenomena [
Our patient presented with stroke due to thromboembolic disease from aortic valve vegetation that was believed to be a papillary fibroelastoma due to its papillary structure on echocardiography and patient’s lack of clinical evidence of bacterial endocarditis. TTE and TEE studies revealed findings consistent with a large papillary lesion. Due to the acute risk of further embolic phenomena valve sparing excision was performed revealing a nonbacterial thrombus. Even with the use of contrast echocardiography or cardiac MRI, we would not have been able to differentiate between thrombus and papillary fibroelastoma [
Echocardiographic imaging can only provide limited information on the characteristic morphology of cardiac masses, with further contribution to pathologic etiology improved with use of contrast echocardiography or cardiac MRI (limited availability at most institutions). Despite clinical objective evidence supporting benign cardiac tumor etiology (i.e., TEE study consistent with papillary fibroelastoma), one must take the entire clinical picture into account before pursuing invasive surgical procedures. This unique case provides an example that medicine and surgery is not always “textbook” in nature. Even though the echocardiographic evidence supported our clinical suspicion, pathologic analysis did not coincide. Further investigative research must be done to help clinicians and surgeons diagnostically differentiate benign cardiac tumors (myxomas, papillary fibroelastomas, and lipomas) from the less commonly occurring native aortic thrombus. We also recommend that when aortic thrombi are suspected or discovered on imaging, an age-appropriate malignancy work-up should be performed as approximately 15% of cancer patients may have thromboembolic events throughout their disease process [
Transthoracic echocardiograph
Transesophageal echocardiograph
Magnetic Resonance Imaging.
The authors declare that there are no competing interests regarding the publication of this paper.