Close
New

Medscape is available in 5 Language Editions – Choose your Edition here.

 

Cardiac Myxoma Pathology 

  • Author: Joseph J Maleszewski, MD; Chief Editor: Allen Patrick Burke, MD  more...
 
Updated: Feb 02, 2014
 

Definition

The World Health Organization (WHO) defines a cardiac myxoma as a neoplasm composed of stellate to plump, cytologically bland mesenchymal cells set in a myxoid stroma.[1]

Next

Epidemiology

From an epidemiologic perspective, cardiac myxomas are best divided into the following 2 categories: those that arise in isolation and those that arise in the setting of a syndrome (so-called Carney complex). The former account for about 90-95% of cases, while the latter account for a minority of cases.

Nonsyndromic cardiac myxomas have been described prenatally and in individuals as old as 97 years.[2, 3] Although they can be found anywhere within this very wide age range, these isolated cardiac myxomas are clearly more common among adults, who present at an average age of 50 years; furthermore, nonsyndromic cardiac myxomas occur more commonly in women than in men.[3, 4]

Syndromic cardiac myxomas typically arise as part of the Carney complex (see Molecular/Genetics). Cardiac myxomas occurring in this setting usually develop in combination with 1 or more of the following lesions:

  • Myxomas in other sites (eg, cutaneous, mucosal, breast)
  • Cutaneous lentiginosis
  • Acromegaly
  • Cushing syndrome (primary pigmented nodular adrenocortical disease)
  • Large-cell calcifying Sertoli-cell tumors of the testis
  • Psammomatous melanotic schwannoma

There are important demographic differences between syndromic and nonsyndromic cardiac myxomas. The syndromic variety, unlike their isolated counterpart of cardiac myxomas, do not exhibit a clear-cut sex predilection and typically present in the third decade rather than the fifth decade.[5]

Previous
Next

Etiology

Although numerous theories have been posited regarding the etiology of cardiac myxomas, the precise histogenesis of these tumors remains to be determined. For a time, cardiac myxomas were believed to arise from mural thrombi.[6] However, the differences between myxomas and thrombi are substantial. Although mural thrombi tend to occur in individuals with underlying heart disease and in many locations within the heart (eg, atrial appendages, atria, and ventricles), myxomas arise with astonishing consistency in 1 location: primarily adjacent to the fossa ovalis.

Furthermore, histologically, myxomas do not organize into fibrous tissue or show stratification, a classic feature of mural thrombi. Cardiac myxomas also behave differently from thrombi in tissue culture studies.[7] This body of evidence in support of the neoplastic nature of myxomas has led to the consensus that cardiac myxomas are not of thrombotic origin and are, indeed, neoplastic.[8]

The impetus for this neoplastic transformation is equally unclear. Although genetic factors (see Molecular/Genetics) clearly play a role in myxoma syndromes, they do not appear to offer a consistent explanation in sporadic cases. A study by Li et al reported finding evidence for HSV-1 infection in 70% of a relatively small cohort (n=17) of surgically resected sporadic cardiac myxomas.[9]

Regardless of the precise etiology of cardiac myxomas, the morphologic, ultrastructural, and immunoperoxidase studies done to date suggest that the neoplastic cells are of primitive multipotential mesenchymal origin.[10, 8, 7]

Previous
Next

Location

Most myxomas (75%) are located in the left atrium. Most of the remainder (18%) are found in the right atrium,[11] a smaller number (6%) are equally split between the left and right ventricles (3% each),[12] and only a very small fraction (< 1%) involve the valves.[13, 14, 15, 16]

These tumors are typically found in the region of the fossa ovalis. For a time, myxomas were thought to arise from minute endocardial endothelial structures known as Prichard structures, located primarily in the fossa ovalis.[17] However, this theory has now been largely discredited by studies that show no relation between the seemingly age-related Prichard structures and myxomas.[18]

Cardiac myxomas arising as part of the Carney complex occur somewhat less commonly in the left atrium (62%) and are often multicentric, occasionally involving multiple chambers.[5]

Previous
Next

Clinical Features and Imaging

Cardiac myxomas elicit a wide variety of symptoms in patients, largely depending on the size and location of the tumor. Individuals with cardiac myxomas can present at any point along the clinical continuum, ranging from complete absence of symptoms (particularly with tumors less than 40 mm in size) to sudden death, usually owing to acute obstruction or embolization.[19, 20]

Although the clinical spectrum can be wide, most affected individuals present with 1 or more of a triad of symptoms (the so-called myxoma triad), which includes embolic phenomena, intracardiac flow obstruction, and constitutional symptoms.[21, 22, 23, 24, 25] These symptoms, however, are rather nonspecific, and myxomas (and cardiac tumors in general) are relatively rare in most clinicians' practices; consequently, cardiac myxomas are often misdiagnosed.[26]

Symptoms of intracardiac flow obstruction are the most commonly described manifestation, occurring in more than 50% of persons with cardiac myxomas.[1, 27] Typically, patients show symptoms of left-side heart failure (eg, dyspnea, orthopnea, or fatigue) or syncope. Right atrial myxomas can manifest with symptoms of right-side heart failure (eg, systemic edema or hepatomegaly). Occasionally, a systolic or diastolic murmur can be auscultated. In some cases, a characteristic “tumor plop” is audible in early diastole.[27]

About 30-40% of individuals with cardiac myxomas experience embolic phenomena. Sites of embolization include the central nervous system (CNS), kidneys, extremities, and coronary arteries. The clinical manifestations of embolization are broad and depend on the tissues downstream of the embolus. Note that paradoxical embolism may occur in individuals with an anatomically patent foramen ovale.[28] In rare cases, myxomas can also serve as a nidus for infection.[29] When this occurs, symptoms parallel those of infective endocarditis.

Nonspecific constitutional symptoms have been reported in 20-60% of individuals with cardiac myxomas.[1, 11, 27] Such symptoms may include fever, arthralgia, myalgia, and weight loss.[23] Laboratory findings may include elevated erythrocyte sedimentation rate (33%), normochromic anemia (15%), and thrombocytopenia (5%).

These findings are probably due to the tumor’s constitutive ability to elaborate interleukin (IL)-6, a cytokine that induces the acute-phase response. Individuals who experience these constitutional symptoms have been found to have higher serum levels of IL-6, with these levels dropping precipitously after removal of the tumor.[30]

Transthoracic echocardiography (TTE) is the diagnostic imaging modality of choice for cardiac myxomas, but transesophageal echocardiography (TEE) is better at defining the tumor's location and characteristics.[31] Both TTE and 2-dimensional (2D) TEE are highly sensitive for identifying cardiac myxomas; however, anatomic and morphologic information is often incomplete. Real-time 3-dimensional (3D) TEE may help surgeons gain a better understanding of the nature and location of the tumor.[32]

The differential diagnosis of cardiac myxoma is briefly summarized in Table 1 below.

Table 1. Differential Diagnosis of Cardiac Myxoma (Open Table in a new window)

Differential Diagnosis Differentiating Features
Thrombus Zonation pattern with fibrin



Absence of myxomas cells



Myxoid sarcoma Pleomorphic spindle cells



Infiltrative growth pattern



Papillary fibroelastoma Endothelial-lined avascular papillary fronds



Absence of myxoma cells



Calcified amorphous tumor (CAT) of the



heart (cardiac CAT)



Prominent calcification



Absence of myxoma cells



Mesothelial incidental cardiac



excrescence (MICE)



Lack of myxoid stroma



Previous history of cardiac procedure



Fibroma Abundant collagenous stroma



Typically ventricular



Intracavitary hemangioma Lobular arrangement of vessels



Typical lack of myxoid stroma



Vessels surrounded by actin-positive pericytes



Metastatic carcinoma Myxomas rarely contain glandular elements



Patients typically have history of carcinoma



Previous
Next

Gross Findings

The vast majority of cardiac myxomas manifest as cavitary gelatinous masses that arise adjacent to the fossa ovalis in the left atrium.[21] They are typically pedunculated but can also arise in a sessile fashion, ranging in size from as small as a few millimeters to upward of 15 cm (see the image below).[33, 27] Cardiac myxomas have been reported to weigh as much as 250 g.[1]

Sessile myxoma arising within left atrium. Note ho Sessile myxoma arising within left atrium. Note how myxoma has very broad attachment to underlying myocardium.

Cardiac myxomas may have a smooth or papillary surface and may have thrombus adherent. Both the papillary excrescences and the surface thrombi can be friable in nature and undergo embolization. On cut section, they often have a variegated appearance, with areas of pearly white, pale-gray, and yellow tissue punctuated by patchy dark-red hemorrhage, gritty necrosis, or calcification (see the image below).

Photograph shows sizable myxoma arising in left at Photograph shows sizable myxoma arising in left atrium adjacent to mitral valve. Tumor has been bivalved to demonstrate its variegated appearance. Clearly seen are pearly white, pale-gray, and yellow areas of tissue punctuated by patchy dark-red hemorrhage.
Previous
Next

Microscopic Findings

The diagnosis of cardiac myxoma is primarily dependent on the presence of the myxoma or lepidic cells described in the World Health Organization (WHO) definition cited earlier (see Definition). Myxoma cells are frequently stellate with eosinophilic cytoplasm and indistinct cell borders (see the image below). Their ovoid nuclei are typically pale with open chromatin. Nucleoli may be prominent.

Microscopic image shows so-called myxoma cell. Myx Microscopic image shows so-called myxoma cell. Myxoma cells are frequently stellate with eosinophilic cytoplasm and indistinct cell borders. Their ovoid nuclei are typically pale with open chromatin.

Architecturally, myxoma cells form rings, cords, and nests that are often closely associated with capillaries (see the images below). The cells can also exist singly as stellate cells in a myxoid stroma that is composed of variable amounts of proteoglycans, collagen, and elastin and that often contains lymphocytes, plasma cells, and histiocytes.

Photomicrograph demonstrates myxoma cells forming Photomicrograph demonstrates myxoma cells forming rings that are in close association with capillaries.
Photomicrograph demonstrates myxoma cells forming Photomicrograph demonstrates myxoma cells forming cords within myxoid background.
Photomicrograph shows myxoma cells forming rings, Photomicrograph shows myxoma cells forming rings, cords, and nests throughout tumor.

Extravasated erythrocytes and hemosiderin deposition are invariably present (see the first and second images below). Thin-walled blood vessels (occasionally large and cavernous) can be seen throughout the lesion (see the third image below), with thick-walled blood vessels typically present near the pedicle or attachment (see the fourth image below). Myxomas have been shown to elaborate vascular endothelial growth factor (VEGF), which may contribute to the high vascularity frequently encountered in these tumors.[34]

Prominent intratumoral hemorrhage can be seen in t Prominent intratumoral hemorrhage can be seen in this photomicrograph, correlating with dark-red hemorrhagic areas seen grossly.
Photomicrograph demonstrates erythrocyte extravasa Photomicrograph demonstrates erythrocyte extravasation with accompanying hemosiderin deposition, indicating that hemorrhage occurred in vivo and is not artifact of processing.
Photomicrograph demonstrates numerous cavernous, t Photomicrograph demonstrates numerous cavernous, thin-walled blood vessels that can be seen throughout tumor.
Larger, thick-walled blood vessels are frequently Larger, thick-walled blood vessels are frequently located near pedicle or attachment of myxoma to underlying myocardium, as can be seen in this photomicrograph.

Secondarily, fibrosis, necrosis, thrombosis, calcification, or Gamna-Gandy body formation (calcific/siderotic elastic fiber degeneration) can be identified (see the images below). These secondary, degenerative changes are often attributed to a long clinical course and are more frequently encountered in right atrial myxomas. To prevent misdiagnosis, it is important for the surgical pathologist to keep in mind that myxomas frequently have adherent thrombus on their surface.

Photomicrograph shows appearance of Gamna-Gandy bo Photomicrograph shows appearance of Gamna-Gandy bodies, which are calcific/siderotic elastic fibers often found within cardiac myxomas.
Photomicrograph demonstrates rather prominent calc Photomicrograph demonstrates rather prominent calcific/siderotic elastic fiber degeneration: so-called Gamna-Gandy body formation.

In addition to the more common histopathologic features of cardiac myxomas described above, heterologous elements, such as columnar epithelium (sometimes forming glands),[11, 35] bone formation (see the images below),[36] extramedullary hematopoiesis,[11] and thymic rests[11] have been described. However, these elements are quite uncommon and have been found in fewer than 2% of cardiac myxomas.[1]

Photomicrograph demonstrates heterologous bone for Photomicrograph demonstrates heterologous bone formation within tumor.
Photomicrograph demonstrates heterologous bone for Photomicrograph demonstrates heterologous bone formation (upper left) within tumor. Bottom right of image shows classic myxoma cells in myxoid background.

To date, no study has been able to document consistently reproducible differences between sporadic and familial myxomas at the gross or the microscopic level.

Previous
Next

Immunohistochemistry

Most studies that set forth to define myxomas on the basis of immunohistochemistry (IHC) have yielded somewhat conflicting results; accordingly, IHC is not particularly helpful in the diagnosis of cardiac myxomas. Myxoma cells exhibit immunoreactivity to calretinin (75-100%), vimentin (> 50%), and alpha-1 antichymotrypsin.[37, 21, 38] Calretinin, in particular, has been proved quite useful in discriminating cardiac myxomas from mural thrombi and papillary fibroelastomas, both of which lack immunoreactivity to this substance.

Cardiac myxomas have shown to be variably immunoreactive to S-100, smooth muscle actin, desmin, alpha-1 antitrypsin, synaptophysin, neuron-specific enolase (NSE), factor VII, CD34, and CD31.[37, 1, 21, 39] Although myxoma (lepidic) cells are not themselves immunoreactive to cytokeratins, myxomas can exhibit reactivity within heterologous glandular elements, when present.[35]

Hemminger et al studied the usefulness of NY-ESO-1 as a marker for myxoid and round cell liposarcoma among mesenchymal myxoid neoplasms. They concluded that this can be a less expensive, faster, and more accessible test for confirming the diagnosis.[40]

Previous
Next

Molecular/Genetics

Between 5% and 10% of cardiac myxomas are associated with a heritable disorder that also includes spotty pigmentation of the skin and endocrinopathy, a set of conditions collectively referred to as the Carney complex.[11] Before Carney’s description of this form of multiple endocrine neoplasia, individuals with familial myxoma were described as having either the nevi, atrial myxoma, myxoid neurofibroma, and ephelides (NAME) syndrome[41] or the lentigines, atrial myxoma, and blue nevi (LAMB) syndrome.[42]

Two genetic loci have been ascribed to the Carney complex through linkage studies: 2p16 and 17q22-24.[43] The cellular mechanism of cardiac myxoma development has yet to be elucidated. The gene PRKAR1A does appear to play a role in the development of cardiac myxomas in the context of Carney complex, but its role in nonsyndromic myxoma genesis is much less clear.

The clinical manifestations of the Carney complex are as follows:

  • Spotty skin pigmentation with a typical distribution (lips, conjunctiva, and inner or outer canthi, vaginal, and penile mucosa)
  • Myxoma (cutaneous and mucosal)
  • Cardiac myxoma
  • Breast myxomatosis or fat-suppressed magnetic resonance imaging (MRI) findings suggestive of this diagnosis
  • Primary pigmented nodular adrenocortical disease (PPNAD) or paradoxical positive response of urinary glucocorticosteroids to dexamethasone
  • Administration during Liddle test
  • Acromegaly due to growth hormone (GH)-producing adenoma
  • Large-cell calcifying Sertoli cell tumor
  • Thyroid carcinoma or multiple, hypoechoic nodules on thyroid ultrasonography in a young patient
  • Psammomatous melanotic schwannoma
  • Blue nevus, epithelioid blue nevus (multiple)
  • Breast ductal adenoma (multiple)
  • Osteochondromyxoma

Supplemental criteria are as follows:

  • Affected first-degree relative
  • Inactivating mutation of the PRKAR1A gene

Diagnostic criteria for the Carney complex include having 2 recognized clinical manifestations or 1 clinical manifestation plus evidence of genetic transmission, which is usually autosomal dominant.[44]

Previous
Next

Tumor Spread and Staging

Although the clinical course of cardiac myxomas is considered by most to be entirely benign, isolated cases of cardiac myxomas undergoing so-called malignant change have been reported.[45, 46, 47] Reports typically cite areas of hypercellularity, necrosis, and atypia as a rationale for this designation; however, in many instances, follow-up has been incomplete, leaving little objective evidence of a true malignant process. In addition, it is likely that many of these examples represent misdiagnosed myxoid sarcomas, a distinct and separate entity.

Myxomas can recur locally (usually with incomplete resection) and spread to distant sites through embolization. Embolization appears to be much more likely in myxomas that are friable with a broad-based attachment than they are in tumors that are fibrotic or calcified.[11] One study suggests that the expression of the mucin gene MUC5AC within sporadic cardiac myxomas may correlate with a lower risk of embolization.[43]

After embolization, myxomas are capable of infiltrating into the arterial wall and producing histologically identical tumors at the embolization site. Such lesions have been described in the skin, the central nervous system (CNS), the lungs, and bone.[48, 49, 50, 51]

Because of the benign nature of cardiac myxomas, no official staging system has been developed for them.

Previous
Next

Prognosis and Predictive Factors

The factor that appears most predictive of a recurrence of myxoma is whether the tumor arose in the setting of a familial myxoma syndrome. The recurrence rate is 1-3% in patients with sporadic myxomas but 10-20% in patients with familial myxomas.[52, 23] The likelihood of developing a recurrence has been associated with abnormal DNA ploidy patterns, mostly in these familial myxomas.[53]

In patients with sporadic myxomas, incomplete excision is thought to be the most likely reason for tumor recurrence. Cellularity and mitotic index do not seem to correlate consistently with recurrence. Close clinical follow-up is recommended for individuals diagnosed with familial cardiac myxomas.

Previous
 
Contributor Information and Disclosures
Author

Joseph J Maleszewski, MD Assistant Professor, Division of Anatomic Pathology, Department of Laboratory Medicine and Pathology, Mayo Medical School

Joseph J Maleszewski, MD is a member of the following medical societies: Alpha Omega Alpha, College of American Pathologists, United States and Canadian Academy of Pathology, Society for Cardiovascular Pathology

Disclosure: Nothing to disclose.

Chief Editor

Allen Patrick Burke, MD Associate Professor, Department of Pathology, University of Maryland School of Medicine; Chairman, Department of Cardiovascular Pathology, Armed Forces Institute of Pathology

Allen Patrick Burke, MD is a member of the following medical societies: American College of Cardiology, American College of Gastroenterology, American Medical Association, United States and Canadian Academy of Pathology, American Academy of Forensic Sciences, Society for Cardiovascular Pathology

Disclosure: Nothing to disclose.

References
  1. Burke AP TH, Gomez-Roman JJ, Loire R, et al. World Health Organization: Tumours of the Lung, Pleura, Thymus and Heart. IARC Press; 2004.

  2. Goodwin JF. Diagnosis of left atrial myxoma. Lancet. 1963 Mar 2. 1(7279):464-8. [Medline].

  3. Kusumi T, Minakawa M, Fukui K, et al. Cardiac tumor comprising two components including typical myxoma and atypical hypercellularity suggesting a malignant change. Cardiovasc Pathol. 2008 Jul 10. [Medline].

  4. Wold LE, Lie JT. Cardiac myxomas: a clinicopathologic profile. Am J Pathol. 1980 Oct. 101(1):219-40. [Medline].

  5. Carney JA. Differences between nonfamilial and familial cardiac myxoma. Am J Surg Pathol. 1985 Jan. 9(1):53-5. [Medline].

  6. 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. 2008 Aug. 109(2):318-20. [Medline].

  7. Stratakis CA, Kirschner LS, Carney JA. Clinical and molecular features of the Carney complex: diagnostic criteria and recommendations for patient evaluation. J Clin Endocrinol Metab. 2001 Sep. 86(9):4041-6. [Medline].

  8. Leone S, dell'Aquila G, Giglio S, Magliocca M, Maio P, Nigro FS, et al. Infected atrial myxoma: case report and literature review. Infez Med. 2007 Dec. 15(4):256-61. [Medline].

  9. Li Y, Pan Z, Ji Y, Sheppard M, Jeffries DJ, Archard LC, et al. Herpes simplex virus type 1 infection associated with atrial myxoma. Am J Pathol. 2003 Dec. 163(6):2407-12. [Medline]. [Full Text].

  10. Grebenc ML, Rosado de Christenson ML, Burke AP, Green CE, Galvin JR. Primary cardiac and pericardial neoplasms: radiologic-pathologic correlation. Radiographics. 2000 Jul-Aug. 20(4):1073-103; quiz 1110-1, 1112. [Medline].

  11. Burke AP, Virmani R. Cardiac myxoma. A clinicopathologic study. Am J Clin Pathol. 1993 Dec. 100(6):671-80. [Medline].

  12. Yoshikai M, Kamohara K, Fumoto H, Kawasaki H. Left ventricular myxoma originating from the papillary muscle. J Heart Valve Dis. 2003 Mar. 12(2):177-9. [Medline].

  13. Cina SJ, Smialek JE, Burke AP, Virmani R, Hutchins GM. Primary cardiac tumors causing sudden death: a review of the literature. Am J Forensic Med Pathol. 1996 Dec. 17(4):271-81. [Medline].

  14. Karlof E, Salzberg SP, Anyanwu AC, Steinbock B, Filsoufi F. How fast does an atrial myxoma grow?. Ann Thorac Surg. 2006 Oct. 82(4):1510-2. [Medline].

  15. McCarthy PM, Schaff HV, Winkler HZ, Lieber MM, Carney JA. Deoxyribonucleic acid ploidy pattern of cardiac myxomas. Another predictor of biologically unusual myxomas. J Thorac Cardiovasc Surg. 1989 Dec. 98(6):1083-6. [Medline].

  16. Yoon DH, Roberts W. Sex distribution in cardiac myxomas. Am J Cardiol. 2002 Sep 1. 90(5):563-5. [Medline].

  17. Powers JC, Falkoff M, Heinle RA, et al. Familial cardiac myxoma: emphasis on unusual clinical manifestations. J Thorac Cardiovasc Surg. 1979 May. 77(5):782-8. [Medline].

  18. Acebo E, Val-Bernal JF, Gomez-Roman JJ. Prichard's structures of the fossa ovalis are not histogenetically related to cardiac myxoma. Histopathology. 2001 Nov. 39(5):529-35. [Medline].

  19. Chu PH, Jung SM, Yeh TS, Lin HC, Chu JJ. MUC1, MUC2 and MUC5AC expressions in cardiac myxoma. Virchows Arch. 2005 Jan. 446(1):52-5. [Medline].

  20. Gopalakrishnaiah Guptak, Reddy DJ, Sakuntala Devi P, Suryaprakasa Rao T, Venkaiah KR, Venkataswamy Naidu N. Congenital myxoma of the heart. Indian J Pediatr. 1956 Jun. 23(100):210-2. [Medline].

  21. Burke AP, Virmani R. Tumors of the Heart and Great Vessels. Armed Forces Institute of Pathology; 1995. 16: 231.

  22. Galanti G, Rostagno C, Stefani L, et al. Right ventricular myxoma detected incidentally in a young athlete. Clin J Sport Med. 2008 May. 18(3):295-7. [Medline].

  23. Reed RJ, Utz MP, Terezakis N. Embolic and metastatic cardiac myxoma. Am J Dermatopathol. 1989 Apr. 11(2):157-65. [Medline].

  24. Todo T, Usui M, Nagashima K. Cerebral metastasis of malignant cardiac myxoma. Surg Neurol. 1992 May. 37(5):374-9. [Medline].

  25. Wang JG, Li YJ, Liu H, Li NN, Zhao J, Xing XM. Clinicopathologic analysis of cardiac myxomas: Seven years' experience with 61 patients. J Thorac Dis. 2012 Jun 1. 4(3):272-83. [Medline]. [Full Text].

  26. Attum AA, Johnson GS, Masri Z, Girardet R, Lansing AM. Malignant clinical behavior of cardiac myxomas and "myxoid imitators". Ann Thorac Surg. 1987 Aug. 44(2):217-22. [Medline].

  27. Parissis JT, Mentzikof D, Georgopoulou M, et al. Correlation of interleukin-6 gene expression to immunologic features in patients with cardiac myxomas. J Interferon Cytokine Res. 1996 Aug. 16(8):589-93. [Medline].

  28. Pinede L, Duhaut P, Loire R. Clinical presentation of left atrial cardiac myxoma. A series of 112 consecutive cases. Medicine (Baltimore). 2001 May. 80(3):159-72. [Medline].

  29. Larsson S, Lepore V, Kennergren C. Atrial myxomas: results of 25 years' experience and review of the literature. Surgery. 1989 Jun. 105(6):695-8. [Medline].

  30. Ozcan AV, Evrengul H, Bir F, Tanriverdi H, Goksin I, Kaftan A. Multiple myxomas originating from anterior and posterior mitral leaflets in the left ventricle leading to LV outflow tract obstruction. Circ J. 2008 Oct. 72(10):1709-11. [Medline].

  31. Oliveira R, Branco L, Galrinho A, Abreu A, Abreu J, Fiarresga A, et al. Cardiac myxoma: a 13-year experience in echocardiographic diagnosis. Rev Port Cardiol. 2010 Jul-Aug. 29(7-8):1087-100. [Medline].

  32. Tolstrup K, Shiota T, Gurudevan S, Luthringer D, Luo H, Siegel RJ. Left atrial myxomas: correlation of two-dimensional and live three-dimensional transesophageal echocardiography with the clinical and pathologic findings. J Am Soc Echocardiogr. 2011 Jun. 24(6):618-24. [Medline].

  33. Johansson L. Histogenesis of cardiac myxomas. An immunohistochemical study of 19 cases, including one with glandular structures, and review of the literature. Arch Pathol Lab Med. 1989 Jul. 113(7):735-41. [Medline].

  34. Kennedy P, Parry AJ, Parums D, Pillai R. Myxoma of the aortic valve. Ann Thorac Surg. 1995 May. 59(5):1221-3. [Medline].

  35. Prichard RW. Tumors of the heart; review of the subject and report of 150 cases. AMA Arch Pathol. 1951 Jan. 51(1):98-128. [Medline].

  36. 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. 2000 Jan. 119(1):101-7. [Medline].

  37. Acebo E, Val-Bernal JF, Gomez-Roman JJ. Thrombomodulin, calretinin and c-kit (CD117) expression in cardiac myxoma. Histol Histopathol. 2001 Oct. 16(4):1031-6. [Medline].

  38. Tanimura A, Kitazono M, Nagayama K, Tanaka S, Kosuga K. Cardiac myxoma: morphologic, histochemical, and tissue culture studies. Hum Pathol. 1988 Mar. 19(3):316-22. [Medline].

  39. Pucci A, Bartoloni G, Tessitore E, Carney JA, Papotti M. Cytokeratin profile and neuroendocrine cells in the glandular component of cardiac myxoma. Virchows Arch. 2003 Nov. 443(5):618-24. [Medline].

  40. Hemminger JA, Iwenofu OH. NY-ESO-1 is a sensitive and specific immunohistochemical marker for myxoid and round cell liposarcomas among related mesenchymal myxoid neoplasms. Mod Pathol. 2013 Apr 19. [Medline].

  41. Atherton DJ, Pitcher DW, Wells RS, MacDonald DM. A syndrome of various cutaneous pigmented lesions, myxoid neurofibromata and atrial myxoma: the NAME syndrome. Br J Dermatol. 1980 Oct. 103(4):421-9. [Medline].

  42. Reynen K. Cardiac myxomas. N Engl J Med. 1995 Dec 14. 333(24):1610-7. [Medline].

  43. Casey M, Mah C, Merliss AD, et al. Identification of a novel genetic locus for familial cardiac myxomas and Carney complex. Circulation. 1998 Dec 8. 98(23):2560-6. [Medline].

  44. Scarpelli M, Montironi R, Ricciuti R, Vecchioni S, Pauri F. Cardiac myxoma with glandular elements metastatic to the brain 12 years after the removal of the original tumor. Clin Neuropathol. 1997 Jul-Aug. 16(4):190-4. [Medline].

  45. Attum AA, Johnson GS, Masri Z, Girardet R, Lansing AM. Malignant clinical behavior of cardiac myxomas and "myxoid imitators". Ann Thorac Surg. 1987 Aug. 44(2):217-22. [Medline].

  46. Kugai T, Chibana M. [Left atrial myxoma with extramedullary hematopoiesis and ossification]. Kyobu Geka. 2002 May. 55(5):376-8. [Medline].

  47. Salyer WR, Page DL, Hutchins GM. The development of cardiac myxomas and papillary endocardial lesions from mural thrombus. Am Heart J. 1975 Jan. 89(1):4-17. [Medline].

  48. Pucci A, Gagliardotto P, Zanini C, Pansini S, di Summa M, Mollo F. Histopathologic and clinical characterization of cardiac myxoma: review of 53 cases from a single institution. Am Heart J. 2000 Jul. 140(1):134-8. [Medline].

  49. Rhodes AR, Silverman RA, Harrist TJ, Perez-Atayde AR. Mucocutaneous lentigines, cardiomucocutaneous myxomas, and multiple blue nevi: the "LAMB" syndrome. J Am Acad Dermatol. 1984 Jan. 10(1):72-82. [Medline].

  50. Rupp GM, Heyman RA, Martinez AJ, Sekhar LN, Jungreis CA. The pathology of metastatic cardiac myxoma. Am J Clin Pathol. 1989 Feb. 91(2):221-7. [Medline].

  51. Terracciano LM, Mhawech P, Suess K, et al. Calretinin as a marker for cardiac myxoma. Diagnostic and histogenetic considerations. Am J Clin Pathol. 2000 Nov. 114(5):754-9. [Medline].

  52. Lie JT. The identity and histogenesis of cardiac myxomas. A controversy put to rest. Arch Pathol Lab Med. 1989 Jul. 113(7):724-6. [Medline].

  53. McCarthy PM, Piehler JM, Schaff HV, et al. The significance of multiple, recurrent, and "complex" cardiac myxomas. J Thorac Cardiovasc Surg. 1986 Mar. 91(3):389-96. [Medline].

 
Previous
Next
 
Sessile myxoma arising within left atrium. Note how myxoma has very broad attachment to underlying myocardium.
Photograph shows sizable myxoma arising in left atrium adjacent to mitral valve. Tumor has been bivalved to demonstrate its variegated appearance. Clearly seen are pearly white, pale-gray, and yellow areas of tissue punctuated by patchy dark-red hemorrhage.
Microscopic image shows so-called myxoma cell. Myxoma cells are frequently stellate with eosinophilic cytoplasm and indistinct cell borders. Their ovoid nuclei are typically pale with open chromatin.
Photomicrograph demonstrates myxoma cells forming rings that are in close association with capillaries.
Photomicrograph demonstrates myxoma cells forming cords within myxoid background.
Photomicrograph shows myxoma cells forming rings, cords, and nests throughout tumor.
Prominent intratumoral hemorrhage can be seen in this photomicrograph, correlating with dark-red hemorrhagic areas seen grossly.
Photomicrograph demonstrates erythrocyte extravasation with accompanying hemosiderin deposition, indicating that hemorrhage occurred in vivo and is not artifact of processing.
Photomicrograph demonstrates numerous cavernous, thin-walled blood vessels that can be seen throughout tumor.
Larger, thick-walled blood vessels are frequently located near pedicle or attachment of myxoma to underlying myocardium, as can be seen in this photomicrograph.
Photomicrograph shows appearance of Gamna-Gandy bodies, which are calcific/siderotic elastic fibers often found within cardiac myxomas.
Photomicrograph demonstrates rather prominent calcific/siderotic elastic fiber degeneration: so-called Gamna-Gandy body formation.
Photomicrograph demonstrates heterologous bone formation within tumor.
Photomicrograph demonstrates heterologous bone formation (upper left) within tumor. Bottom right of image shows classic myxoma cells in myxoid background.
Table 1. Differential Diagnosis of Cardiac Myxoma
Differential Diagnosis Differentiating Features
Thrombus Zonation pattern with fibrin



Absence of myxomas cells



Myxoid sarcoma Pleomorphic spindle cells



Infiltrative growth pattern



Papillary fibroelastoma Endothelial-lined avascular papillary fronds



Absence of myxoma cells



Calcified amorphous tumor (CAT) of the



heart (cardiac CAT)



Prominent calcification



Absence of myxoma cells



Mesothelial incidental cardiac



excrescence (MICE)



Lack of myxoid stroma



Previous history of cardiac procedure



Fibroma Abundant collagenous stroma



Typically ventricular



Intracavitary hemangioma Lobular arrangement of vessels



Typical lack of myxoid stroma



Vessels surrounded by actin-positive pericytes



Metastatic carcinoma Myxomas rarely contain glandular elements



Patients typically have history of carcinoma



Previous
Next
 
 
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2016 by WebMD LLC. This website also contains material copyrighted by 3rd parties.