Cardiac myxomas: clinical presentation, diagnosis and management

Abstract

Cardiac myxomas (CM) are the most common type of primary cardiac tumours in adults, which have an approximate incidence of up to 0.2% in some autopsy series. The purpose of this review is to summarise the literature on CM, including clinical presentation, differential diagnosis, work-up including imaging modalities and histopathology, management, and prognosis. CM are benign neoplasms developed from multipotent mesenchyme and usually present as an undifferentiated atrial mass. They are typically pedunculated and attached at the fossa ovalis, on the left side of the atrial septum. Potentially life-threatening, the presence of CM calls for prompt diagnosis and surgical resection. Infrequently asymptomatic, patients with CM exhibit various manifestations, ranging from influenza-like symptoms, heart failure and stroke, to sudden death. Although non-specific, a classic triad for CM involves constitutional, embolic, and obstructive or cardiac symptoms. CM may be purposefully characterised or incidentally diagnosed on an echocardiogram, CT scan or cardiac MRI, all of which can help to differentiate CM from other differentials. Echocardiogram is the first-line imaging technique; however, it is fallible, potentially resulting in uncommonly situated CM being overlooked. The diagnosis of CM can often be established based on clinical, imaging and histopathology features. Definitive diagnosis requires macroscopic and histopathological assessment, including positivity for endothelial cell markers such as CD31 and CD34. Their prognosis is excellent when treated with prompt surgical resection, with postsurgical survival rates analogous to overall survival in the age-matched general population.

Surgery for Primary Cardiac Tumors in Children: Successful Management of Large Fibromas

“…We have a case of myxofibrosarcoma with multiple metastases soon after surgical resection mimicking myxoma both preoperatively and intraoperatively ( Supplementary Figure 3 ). It may be difficult to differentiate myxomas from malignant tumors or thrombus preoperatively ( 26 , 27 ). PET imaging is sensitive in ruling out malignant tumors, which is costly ( 28 ).…”

“…PET imaging is sensitive in ruling out malignant tumors, which is costly ( 28 ). Although it is histologically benign, cardiac myxomas have a reported recurrence rate of 1–6% ( 27 ). In this study, 2 of the 13 (15.4%) myxomas recurred postoperatively, which may attribute to the higher recurrence rate in younger patients ( 29 ).…”

Heart Failure: From Typical Clinical Manifestations to the Surprising Final Diagnosis

“…Its clinical manifestations are diverse, ranging from incidentally detected masses in asymptomatic individuals to symptoms related to cardiac obstruction, systemic embolization mostly to the central nervous system, direct invasion of the myocardium or adjacent lung parenchyma, or even constitutional signs and symptoms [2,[4][5][6]. The latter is justified by the release of pro-inflammatory factors by the tumor and includes fever, weight loss, and myalgias, among others, also explaining the possible increase in CRP, leukocytosis, thrombocytosis/thrombocytopenia and anemia [2][3][4][5].…”

“…Their clinical spectrum varies regarding size, location, and mobility, with the vast majority developing in the left atrium. The difficulty to detect them correlates with the lack of specific signs and symptoms; from asymptomatic patients to potentially fatal complications [2][3][4], these benign tumors require an early diagnosis and referral for urgent surgical treatment.…”

Myxoma in patients with hypertrophic obstructive cardiomyopathy. Retrospective single-center analysis

“…Atrial myxomas are much rarer although the most frequent ones are benign cardiac tumors. The surgical incidence of atrial myxomas is 0.5/million population/year [3], and the approximate prevalence of cardiac tumors in adults is up to 0.2% in some autopsy series [4][5][6]. Most atrial myxomas are located in the left atrium.…”