Atrial Myxoma Workup
- Author: Gyanendra K Sharma, MD, FACC, FASE; Chief Editor: Park W Willis IV, MD more...
Laboratory Studies
Lab studies are nonspecific and nondiagnostic. If present, abnormalities may include the following:
- Elevated erythrocyte sedimentation rate (ESR) and elevated C-reactive protein and serum gamma globulin levels
- Leukocytosis
- Anemia may be normochromic or hypochromic. Hemolytic anemia may occur because of the mechanical destruction of erythrocytes by the tumor.
- Serum interleukin-6 levels may be raised and can be used as a marker of recurrence.
Imaging Studies
- Chest radiography
- Abnormal cardiac silhouette, mimicking mitral stenosis
- Unusual intracardiac tumor calcification
- Pulmonary edema
- Echocardiography
- Although transesophageal echocardiography is more sensitive, 2-dimensional echocardiography is usually adequate for diagnosis.
- Tumor location, size, attachment, and mobility can be assessed with this technique.
- Because tumors may be in multiple locations, all 4 chambers should be visualized.
- An atrial myxoma must be differentiated from a left atrial thrombus. The thrombus is usually situated in the posterior portion of the atrium and has a layered appearance. Presence of a stalk and mobility favors atrial myxoma.
- Doppler echocardiography can show the hemodynamic consequences of atrial myxoma. The findings are consistent with mitral stenosis or regurgitation.
- Transesophageal echocardiography
- Better specificity and 100% sensitivity compared to transthoracic echocardiography
- Good resolution of both atria and the atrial septum
- Better visualization of anatomic details of the tumor and stalk
- Reveals smaller (1-3 mm in diameter) vegetations or tumors
- Visualizes atrial appendages
- Detects shunting
- Advisable for myxoma syndrome - Multiple less common sites
Transesophageal echocardiography.
- MRI
- MRI provides useful information about size, shape, and surface characteristics on T1-weighted images. Cine MR gradient echo (GRE) images can demonstrate the mobility of a tumor. Point of attachment is best visualized by MRI with a postsurgical correlation of 83%. In a small series, MRI was superior to CT scan, which showed only 30% correlation for the site of attachment.
- Information about tissue composition can be used to differentiate a tumor and a thrombus.
- CT scan
- CT scan can be used to differentiate the characteristics of the myxoma from intracardiac thrombus. Myxomas are larger than a thrombus and have typical site of origin, shape, mobility, and occurrence of prolapse.[15]
- Degree of attenuation or presence of calcification is not useful to differentiate atrial myxoma from a thrombus.
Other Tests
- If petechiae are present, a skin biopsy may reveal the presence of elongated or spindle-shaped, myxomatous, endothelial-like cells with round or oval nuclei and prominent nucleoli.
- Electrocardiography may show left atrial enlargement, atrial fibrillation, atrial flutter, or conduction disturbances.
- In suspected cases of Carney complex, molecular genetic testing for PRKAR1A should be performed to confirm the diagnosis. The family members of the PRKAR1A -positive patients should undergo genetic screening and evaluation for cardiac and extracardiac manifestations of the disease.[16]
Procedures
Cardiac catheterization
- Preoperative cardiac catheterization and angiography may be of value to evaluate for neovascularization.[3]
- Cardiac catheterization is usually performed to exclude coexistent coronary artery disease in patients older than 40 years.
- Atrial myxoma appears as an intracardiac filling defect on angiography. This was the pre-echocardiography method of diagnosis. With current imaging modalities, there is no need for angiography.
Histologic Findings
Histologic studies are characterized by the presence of lipidic cells embedded in a vascular myxoid stroma.
- The cells are polygonal to stellate in shape with scant eosinophilic cytoplasm.
- Tumor necrosis is present in approximately 8% of patients; calcification is present in 10-20%.
- A variable degree of hemorrhage may be present, and mitoses are typically absent.
- In a series of 37 cases, 74% of tumors showed immunohistochemical expression of interleukin-6 while 17% had abnormal DNA content.[17]
Larsson S, Lepore V, Kennergren C. Atrial myxomas: results of 25 years' experience and review of the literature. Surgery. Jun 1989;105(6):695-8. [Medline].
Ha JW, Kang WC, Chung N. Echocardiographic and morphologic characteristics of left atrial myxoma and their relation to systemic embolism. Am J Cardiol. 1999;83:1579-1582. [Medline].
Hasdemir H, Alper AT, Arslan Y, Erdinler I. [Left atrial myxoma with severe neovascularization: role of preoperative coronary angiography]. Turk Kardiyol Dern Ars. Mar 2011;39(2):163-5. [Medline].
Park J, Song JM, Shin E, Jung SH, Kim DH, Kang DH, et al. Cystic cardiac mass in the left atrium: hemorrhage in myxoma. Circulation. Mar 15 2011;123(10):e368-9. [Medline].
Sakamoto H, Sakamaki T, Kanda T, Tsuchiya Y, Sato M, Sato H. Vascular endothelial growth factor is an autocrine growth factor for cardiac myxoma cells. Circ J. May 2004;68(5):488-93. [Medline].
Mendoza CE, Rosado MF, Bernal L. The role of interleukin-6 in cases of cardiac myxoma. Clinical features, immunologic abnormalities, and a possible role in recurrence. Tex Heart Inst J. 2001;28(1):3-7. [Medline].
Kono T, Koide N, Hama Y, et al. Expression of vascular endothelial growth factor and angiogenesis in cardiac myxoma: a study of fifteen patients. J Thorac Cardiovasc Surg. Jan 2000;119(1):101-7. [Medline].
Aggarwal SK, Barik R, Sarma TC, Iyer VR, Sai V, Mishra J. Clinical presentation and investigation findings in cardiac myxomas: new insights from the developing world. Am Heart J. Dec 2007;154(6):1102-7. [Medline].
Lee VH, Connolly HM, Brown RD Jr. Central nervous system manifestations of cardiac myxoma. Arch Neurol. Aug 2007;64(8):1115-20. [Medline].
Smith MJ, Chaudhry MA, Humphrey MB, Lozano PM. Atrial myxoma and bone changes: a paraneoplastic syndrome?. J Card Surg. Jul 2011;26(4):375-7. [Medline].
Furukawa A, Kishi S, Aoki J. Large Infected Atrial Myxoma With Vegetations. Rev Esp Cardiol. Jul 21 2011;[Medline].
Basson CT, MacRae CA, Korf B. Genetic heterogeneity of familial atrial myxoma syndromes (Carney complex). Am J Cardiol. Apr 1 1997;79(7):994-5. [Medline].
Ryou KS, Lee SH, Park SH, Park J, Hwang SK, Hamm IS. Multiple fusiform myxomatous cerebral aneurysms in a patient with Carney complex. J Neurosurg. Aug 2008;109(2):318-20. [Medline].
Imai Y, Taketani T, Maemura K, Takeda N, Harada T, Nojiri T. Genetic analysis in a patient with recurrent cardiac myxoma and endocrinopathy. Circ J. Aug 2005;69(8):994-5. [Medline].
Scheffel H, Baumueller S, Stolzmann P, Leschka S, Plass A, Alkadhi H. Atrial myxomas and thrombi: comparison of imaging features on CT. AJR Am J Roentgenol. Mar 2009;192(3):639-45. [Medline].
Hajj-Chahine J, Jayle C, Houmaida H, Corbi P. Utility of genetic testing in multisite myxoma to rule out Carney complex. Interact Cardiovasc Thorac Surg. Apr 2011;12(4):624. [Medline].
Acebo E, Val-Bernal JF, Gomez-Roman JJ, Revuelta JM. Clinicopathologic study and DNA analysis of 37 cardiac myxomas: a 28-year experience. Chest. May 2003;123(5):1379-85. [Medline].
Owers CE, Vaughan P, Braidley PC, Wilkinson GA, Locke TJ, Cooper GJ, et al. Atrial myxomas: a single unit's experience in the modern era. Heart Surg Forum. Apr 1 2011;14(2):E105-9. [Medline].
Pinede L, Duhaut P, Loire R. Clinical presentation of left atrial cardiac myxoma. A series of 112 consecutive cases. Medicine (Baltimore). May 2001;80(3):159-72. [Medline].
Deshpande RP, Casselman F, Bakir I, Cammu G, Wellens F, De Geest R. Endoscopic cardiac tumor resection. Ann Thorac Surg. Jun 2007;83(6):2142-6. [Medline].
Patil NP, Dutta N, Satyarthy S, Geelani MA, Kumar Satsangi D, Banerjee A. Cardiac myxomas: experience over one decade. J Card Surg. Jul 2011;26(4):355-9. [Medline].
Colucci WS, Schoen FJ, Braunwald E. Primary tumors of the heart. In: Heart disease: A textbook of cardiovascular medicine. 5th ed. 1997:1464-77.
Ekinci EI, Donnan GA. Neurological manifestations of cardiac myxoma: a review of the literature and report of cases. Intern Med J. May 2004;34(5):243-9. [Medline].
Grebenc ML, Rosado-de-Christenson ML, Green CE, et al. Cardiac myxoma: imaging features in 83 patients. Radiographics. May-Jun 2002;22(3):673-89. [Medline].
Jelic J, Milicic D, Alfirevic I. Cardiac myxoma: diagnostic approach, surgical treatment and follow-up. A twenty years experience. J Cardiovasc Surg (Torino). Dec 1996;37(6 Suppl 1):113-7. [Medline].
Kang G, Bhullar P, Kang M. New diagnosis of left atrial myxoma in a 93-year-old woman. Am J Geriatr Cardiol. May-Jun 2005;14(3):148-53. [Medline].
Karlof E, Salzberg SP, Anyanwu AC, Steinbock B, Filsoufi F. How fast does an atrial myxoma grow?. Ann Thorac Surg. Oct 2006;82(4):1510-2. [Medline].
Reynen K. Cardiac Myxomas. N Eng J Med. 1995;333:1610-1617. [Medline].
Reynen K. Frequency of primary tumors of the heart. Am J Cardiol. Jan 1 1996;77(1):107.
Roberts WC. Primary and secondary neoplasms of the heart. Am J Cardiol. 1997;80:671-682. [Medline].
Scott N, Veinot JP, Chan KL. Symptoms in cardiac myxoma. Chest. Dec 2003;124(6):2408. [Medline].
Tazelaar HD, Locke TJ, McGregor CG. Pathology of surgically excised primary cardiac tumors. Mayo Clin Proc. Oct 1992;67(10):957-65. [Medline].

