Atrial myxoma …

Adrian Marchidann MD

Stroke & Vascular Disorders

Updated 09.12.2024
Released 06.11.2001
Expires For CME 09.12.2027

Atrial myxoma

Author: Adrian Marchidann MD

See Contributor Disclosures

Editor: Steven R Levine MD

Atrial myxoma

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Introduction

Overview

Atrial myxoma, the most common intracardiac tumor in adults, may cause cardiac dysfunction, embolic stroke, and systemic manifestations that mimic infective, immunologic, and malignant diseases. Several associated cutaneous syndromes may facilitate timely recognition. Its various clinical manifestations make clinical diagnosis difficult. Approximately 60% of all tumors are diagnosed by echocardiogram. The definitive treatment is surgical excision. Potentially life-threatening, atrial myxomas require prompt diagnosis and urgent treatment.

Key points

	• Atrial myxoma is the most common intracardiac tumor. Although benign, its central location often results in significant morbidity and mortality.
	• Myxomas have a wide variety of cardiac, embolic, and systemic manifestations, including paraneoplastic syndromes.
	• No guidelines for acute ischemic stroke caused by atrial myxoma embolism treatment have been published.
	• Intravenous thrombolysis within a 3-hour window was successful in patients with acute stroke caused by myxoma.
	• Hemorrhagic complications after thrombolysis may be due to recent, undiagnosed infarcts or aneurysms.
	• Intra-arterial recanalization is effective in patients with large artery occlusion due to myxoma embolization.

Historical note and terminology

Left atrial myxoma was first described in the 19th century (76) and was diagnosed by angiocardiography in 1952 (79). The first surgical excision occurred three years later (30).

The first non-embolic dermatologic manifestation of atrial myxoma was described in 1973 as the nevus, atrial myxoma, myxoid neurofibromata, and ephelides or endocrine hyperactivity (NAME syndrome). Later, it was described as the lentigines, atrial myxoma, and blue nevi (LAMB syndrome); Carney complex (atrial myxoma associated with multiple neoplasia lentigines) was also reported (51). Sneddon syndrome, which commonly presents with a livedo-type rash and diffuse cerebrovascular disease, has also been associated with atrial myxoma (152).

Clinical manifestations

Presentation and course

The following are the three ways in which atrial myxomas manifest:

	• embolization
	• cardiac and obstructive symptoms
	• syndromes, including paraneoplastic

Due to its various manifestations, the diagnosis of atrial myxoma may take up to 126 months according to one series (126). Presenting symptoms include fatigue, shortness of breath, fever of unknown origin, and various embolic symptoms (23).

Embolization. Embolism of myxoma fragments or thrombi occurs in 30% to 40% of patients affecting any organ (137; 128). Simultaneous embolization of the brain and heart may lead to stroke and cardiac arrest (65). Skin embolism manifests as erythematous papules, petechiae, digital cyanosis, splinter hemorrhages, livedo reticularis, and Raynaud phenomenon (111; 51). Constitutional symptoms associated with a purpuric rash of unknown origin should prompt evaluation for atrial myxoma (95). Small bowel ischemia is suggested by abdominal pain aggravated by oral intake and is associated with lactic acidosis (62). Right atrial myxoma may cause Budd-Chiari syndrome (73). Limb ischemia requires immediate revascularization (193).

In a single-center study of 93 patients with atrial myxoma, embolism predictors were tumor diameter larger than 4.6 cm, irregular surface, enlarged left atrium, and atrial fibrillation (69). In a systematic review and metanalysis including 12 studies that enrolled 1814 patients, New York Heart Association (NYHA) heart failure class I and II, hypertension, atypical tumor location, narrow base of tumor, and increased fibrinogen were associated with embolism (93). Electroconvulsive therapy triggered embolism in one patient (105).

Cerebrovascular disease. Approximately 45% of patients with atrial myxoma experience neurologic deficits (79).

The transient ischemic attack is the most common initial neurologic manifestation of atrial myxoma (04). Global transient amnesia may also be caused by atrial myxoma (157).

Myxomas cause 0.4% of strokes, mostly before the age of 50 (126). Recurrent strokes, often asymptomatic, can lead to multi-infarct dementia (60; 74). Alternatively, embolic showers or a single massive embolic event may cause rapid multi-organ failure and death (18; 14). Other stroke symptoms include monocular blindness, paraplegia, or convulsive seizures (168; 57; 177; 189).

Arterial wall invasion by the myxomatous emboli often results in multiple bilateral aneurysms in the distal branches of the middle cerebral arteries (20; 130; 31; 103). Approximately 90% of aneurysms are fusiform; the remaining are saccular (144).

Cardiovascular disease. Embolism to the left coronary artery may cause myocardial infarction (120). Right atrial myxoma complications include right heart failure, ascites, and pulmonary embolism (122; 66; 96). Pulmonary embolism may be the initial presentation of myxoma (29; 94). In the absence of a shunt, pulmonary embolism from a left atrial myxoma is most likely caused by the hypercoagulable state associated with the myxoma (135).

Cardiac and obstructive symptoms. The cardiac symptoms correlate with the tumor’s location, size, and mobility (49). Very rarely, myxoma grows in both atria.

Cardiac complications include mitral valve obstruction with congestive heart failure, steal phenomenon in the large anterior descending artery, left ventricular failure, hypertrophic cardiomyopathy, and mitral valve regurgitation (126; 161; 36; 71; 75).

Atrial fibrillation and paroxysmal supraventricular tachycardia may be triggered by atrial myxoma (19; 154).

Endocarditis is suggested by cardiac vegetations associated with positive cultures for Histoplasma and Streptococcus sanguinis (43; 87; 139).

Compression by a giant myxoma may lead to dysphagia, Horner syndrome (anhidrosis, partial ptosis, and miosis), dyspnea, and cough-syncope syndrome (50; 106; 38). Small bowel obstruction may also occur (184). Sudden death may occur due to obstruction of the intracardiac blood flow (78).

Auscultatory findings are associated with mitral valve dysfunction caused by larger lesions (49). A murmur suggesting mitral stenosis is heard in 54% (126). The classic "tumor plop," an extra heart sound heard 80 to 150 milliseconds after the second heart sound, occurs in approximately one third of the patients (137; 49).

Syndromes

NAME syndrome. The acronym NAME stood initially for nevi, atrial myxoma, myxoid neurofibromata, and ephelides (07). Later, the term “ephelides” was replaced by endocrine overactivity due to increased insulin-like growth factor concentration (188). Transmission is usually autosomal dominant, but X-linked dominant transmission may also occur (82).

LAMB syndrome. LAMB syndrome is characterized by mucocutaneous lentigines, cardio-mucocutaneous myxomas, and multiple blue nevi (138).

Carney complex is an autosomal dominant condition associated with neoplasia, myxomas, mottled cutaneous pigmentation, endocrine dysfunction, and non-myxomatous extracardiac tumors (eg, breast fibroadenomas in women and testis Sertoli-cell tumors in men) (165; 187). The Carney complex, familial and sporadic, is associated with a mutation of the PRKAR1A gene (164; 15; 162). Cutaneous embolism may result in livedoid macules (111). Cushing syndrome and acromegaly are also associated with the Carney complex (179).

Paraneoplastic syndromes. Paraneoplastic syndromes are associated with increased levels of humoral mediators and resolve after myxoma resection. Several paraneoplastic syndromes were described: constitutional symptoms, vasculitis/vasculopathy, hematological, constitutional, and other, uncommon manifestations (160).

Constitutional symptoms like fever, fatigue, myalgia, arthralgia, and weight loss occur in up to 90% of atrial myxoma cases (85; 126). These symptoms mimic rheumatological disease and may be masked by nonsteroidal anti-inflammatories (16).

Vasculitis or vasculopathy syndromes include Raynaud phenomenon, livedo reticularis, malar erythematous violaceous eruption with peripheral cyanosis, splinter hemorrhages, serpiginous, annular, violaceous, non-blanching lesions of fingertips, biphasic changes in the color of fingertips on cold exposure, medium-vessel vasculitis, erythema and petechiae of the hands and feet, and acral erythematous papular eruption (101; 89; 59; 21; 123).

The hematologic manifestations of atrial myxomas include thrombocytopenia, anemia, leukocytosis, increased acute phase reactants, amyloidosis, antiphospholipid antibodies, and depletion of factor VII (181; 85; 107; 148; 112; 40).

Uncommon manifestations include proteinuria, iridocyclitis, mediastinal lymphadenopathy, worsening of coexistent systemic lupus, acute pancreatitis, acute renal failure, painful peripheral sensory neuropathy, and mimicking of polyarteritis nodosa (70; 127; 170; 190; 186; 54; 149; 104; 150).

Prognosis and complications

The recurrence rate of sporadic myxoma is 1% to 3% and may be due to incomplete resection (100; 183). Recurrence occurs in up to 25% of familial cases (159). Vanished myxoma by systemic embolization was described (32).

Recurrent embolization may occur despite anticoagulation (79). Endocarditis due to infection of atrial myxoma is rare (172). A case of Takotsubo cardiomyopathy was described in a patient with large left atrial myxoma (176).

Metastases to the vasculature walls can lead to occlusion and infarction (33). Intra-axial metastases can develop by penetrating the blood vessel walls (11). Cerebral metastases were diagnosed several years after myxoma resection (28).

Intracerebral or subarachnoid hemorrhage may complicate the development of cerebral aneurysms. The aneurysms may regress and resolve after myxoma resection but can also enlarge or appear many years later (31; 143; 182; 67).

Malignant transformation of atrial myxoma is rare (147; 92; 68).

Clinical vignette

A 56-year-old Caucasian woman with a history of mild static encephalopathy developed acute right hemiparesis and dysarthria. She was afebrile, had a blood pressure of 123/71, and a pulse of 79. The general examination was remarkable for the lack of cervical bruits and a 2/6 diastolic rumble consistent with mitral stenosis. Peripheral pulses were intact. The neurologic examination was remarkable for dysarthria and mild right hemiparesis, worse in the arm than the leg. Routine laboratory evaluation, including complete blood count, chemistry panel, and coagulation studies, was significant for microcytic anemia and elevated platelet count of 499,000/µL. EKG showed normal sinus rhythm, with chest x-ray showing minimal prominence of pulmonary vascular markings and mild cardiomegaly. Noncontrast head CT was normal. MRI of the brain showed gyral contrast enhancement on T1-weighted imaging in the left parieto-occipital region. The carotid artery ultrasound was normal. Transthoracic echocardiography showed a left atrial mass measuring 3.5 cm by 8.7 cm. The left atrial diameter was 4.6 cm (1.9 to 4.0 cm). The mass caused mitral valve obstruction. The attachment was not visualized. Mild pulmonary hypertension was also noted. She underwent open resection of the mass. A large gelatinous mass was found to be attached at the level of the superior portion of the septum, with a second point of attachment inferiorly at the level of the inferior vena cava. The mass was excised along with the underlying septum, creating an atrial septal defect, which was repaired. Transesophageal echocardiography was used to check mitral flow. Final pathology demonstrated an atrial myxoma. The patient did well postoperatively and was discharged with some improvement in her neurologic symptoms.

Biological basis

	• Myxomas originate from mesenchymal cells.
	• Most cases of atrial myxoma are sporadic.
	• Familial cases have autosomal dominant transmission.
	• Neuromodulators like IL-6 are elevated in patients with constitutional symptoms and usually decrease after resection.

Etiology and pathogenesis

Most cases of atrial myxoma are sporadic. The familial cases are autosomal dominant with variability in the phenotype and tend to present earlier than the sporadic ones. Most myxomas grow on the left side of the interatrial septum close to the fossa ovalis (137). Macroscopically, myxoma is a solid mass, occasionally cystic, pedunculated, shiny, and irregular (155; 121).

Myxomas originate from mesenchymal cells capable of endothelial differentiation surrounded by a myxoid extracellular matrix rich in proteoglycans (137; 146). Deriving from adult developmental remnants, myxoma consists of a highly mucinous myxoid stroma containing plasma cells, mast cells, and stromal stellate cells. Types I, III, and IV collagen are also found within the tumor. The surface is covered by myxoma cells and endothelium (84; 117). Overexpression of matrix metalloproteinase contributes to the degradation of the extracellular matrix, increasing the risk of embolism (118).

The constitutional symptoms are mediated by interleukin-6 (IL-6) in up to 74% of cases (153; 01). IL-6 is secreted by the immature mesenchyme cells of myxomas (169; 167). Myxoma resection often leads to the normalization of IL-6 and clinical improvement (102). Other modulators of the constitutional symptoms include IL-4, IL-12, p70, interferon gamma, and tumor necrosis factor alpha (90).

Tumor size is influenced by basic fibroblast growth factor (FGF), IL-6, monocyte chemotactic protein (MCP-1), thymidine phosphorylase TP-2, chemokine receptor 2 (CCR-2), and vascular endothelial growth factor (VEGF). IL-8 was associated with stroke and myocardial infarction and IL-6 with cerebral aneurysms (144; 160; 39).

Mutation of the tumor suppressor PRKAR1-alpha gene on chromosome 17q22-24 has been identified in the Carney complex (77; 187). A variant of the Carney complex, the trismus–pseudocamptodactyly syndrome with cardiac myxoma, has a missense mutation (Arg674Gln) in the perinatal myosin heavy-chain gene (MYH8) on chromosome 17p12–p13.1 (178). The most involved regions in sporadic cases are 12p1 and 17p1. Dicentric chromosomes and telomeric associations are the most frequent genetic abnormalities (35).

Myxomas associated with the Carney complex may reemerge after removal and cause heart dysfunction. The oncogene c-MYC, cancer-associated metabolic transcriptional factor HIF-1α, and the epithelial-mesenchyma transition protein vimentin could detect malignancy in myxoma (119).

Diagnostic workup

The following are key tests and procedures for diagnosis of atrial myxoma and its complications:

• Blood tests: CBC, ESR, CRP, antinuclear antibodies, rheumatoid factor, and blood cultures
• Chest x-ray
• ECG and cardiac monitoring
• Transthoracic and transesophageal echocardiogram
• CT chest with contrast
• Cardiac CT with contrast
• MRI of the brain with and without contrast and MR angiography of the head
• Cardiac MR with contrast
• FDG-PET/CT
• Coronary angiography

Because of its multisystem involvement, the diagnostic workup is guided by the suspicion of atrial myxoma and its complications.

Laboratory. The routine studies are helpful, but not diagnostic. Anemia is present in 40% and elevated erythrocyte sedimentation in 55% of patients (163). Elevated C-reactive protein, thrombocytosis or thrombocytopenia, serum globulins, antinuclear antibodies, and rheumatoid factor may suggest an infectious or autoimmune condition (137; 58). Blood cultures are needed to exclude infection (136). Streptococci and staphylococci are the most frequent organisms that grow in blood cultures (52; 175; 64). However, Histoplasma capsulatum has also been reported (08).

MRI of the brain. The deep white matter lesions seen on MRI resemble multiple sclerosis or metastatic lesions (113; 132; 185). Gadolinium brain MRI may detect fusiform aneurysms (114). The resolution of MR angiography is approximately 3 mm and is inferior to that of digital subtraction angiography (02).

ECG. ECG changes include left atrial enlargement, nonspecific T-wave and ST-segment abnormalities, and chronic and paroxysmal atrial fibrillation (19).

Chest x-ray. Chest x-ray may reveal left atrial enlargement, blood diversion to upper lobes, left ventricular enlargement, and calcification of the myxoma (163).

Echocardiography. Echocardiography is usually the first diagnostic modality. A 3D echography helps visualize the stalk and characterize the heterogeneity of the myxoma (192). Transthoracic echography has a false-negative rate of 21% for detecting atrial masses, whereas transesophageal echocardiography (TEE) has a sensitivity of 100% for atrial myxoma (180). TEE better localizes the area of attachment—information useful for surgical planning (171). Contrast-enhanced cardiac ultrasound may differentiate a myxoma from a thrombus (25).

Myxoma has a smooth globular surface, sometimes irregular and friable with a narrow stalk. Echolucent areas represent hemorrhage or necrosis (49). Echolucency helps distinguish myxomas from thrombi or vegetations. A large myxoma correlates with constitutional, hemodynamic, and obstructive symptoms, whereas a small one correlates with embolism (44; 49). Polypoid appearance also correlates with embolic phenomena (53).

Cardiac MRI with gadolinium can delineate the tumor size, attachment, and mobility, and help elucidate some histological characteristics (99; 03). An atypical appearance is that of either a hypervascular or nonenhancing mass (05; 134).

Noncontrast chest CT helps detection of atrial myxomas (156) and preoperative evaluation (41). Cardiac contrast-enhanced CT may differentiate atrial myxoma from other intracardiac lesions like thrombi, fibroelastomas, or metastatic tumors and is helpful for preoperative assessment (22; 55).

FDG-PET/CT showing a mildly hypermetabolic hypodense area in the atrium is useful for detecting myxomas in patients with systemic symptoms (46) or pulmonary embolism (91).

Coronary angiography should be performed in most, if not all, patients with atrial myxoma; a tumor blush suggests coronary artery supply (141). CT coronary angiography also helps to distinguish myxomas from thrombi (151). Coexistent coronary artery disease may require both coronary artery bypass grafting and myxoma resection (37).

Management

	• There is no medical therapy for myxoma.
	• Anticoagulation may not be protective.
	• Surgical resection is the definitive treatment.
	• After resection, screening with an echocardiogram is needed.
	• Intravenous tPA may be effective against embolic stroke.
	• Large vessel occlusion may benefit from endovascular neurointervention.

No medical therapy exists for atrial myxomas. Anticoagulation may not be protective and may be hazardous in the event of aneurysm formation. Early surgical resection is the definitive treatment as it prevents recurrent embolization (125; 63). Cardiac arrhythmia, occurring in 26% of patients, is the most common postoperative complication. Persistent bradycardia requires pacemaker placement in less than 2% of cases (126). Postoperative mortality ranges between 2.2% to 3.5% (86; 126).

Total robotic resection is relatively safe and causes minimal cosmetic complications (45; 115). Multiple aneurysms may be approached with endovascular and surgical treatment (124). Minimally invasive procedures can remove increasingly large lesions (27).

The addition of cryoablation to resection for right atrial myxoma helps preserve the cardiac conduction system (97). Percutaneous ablation with retrieval is another option (81).

Transthoracic echocardiography is used after resection to screen for recurrence (129). Most recurrences occur within the first 2 years, but some may take up to 12 years (49).

Treatment of acute ischemic stroke caused by atrial myxoma is guided by a few case reports. In most instances, ischemic stroke is the first manifestation of atrial myxoma. Intravenous recombinant tissue plasminogen activator (IV rt-PA), the only FDA-approved medication for acute stroke treatment, was used with variable success (61; 109; 116; 166; 34). Improvement after endovascular thrombectomy occurs even without intravenous tPA (174). However, the outcome is improved with earlier recanalization (98).

Because of the short therapeutic window, hemorrhagic transformation and subarachnoid hemorrhages are the most feared complications (24; 26). Bleeding may be associated with occult tumor emboli, microaneurysms, or asymptomatic ischemic infarcts with onset beyond the therapeutic window. Despite hemorrhagic conversion, some patients may still improve after thrombolysis.

Large vessel occlusions (LVO) may occasionally be opened with intravenous thrombolysis (72). However, most large vessel occlusions fail to open with intravenous therapy alone (80). Several patients benefited from endovascular thrombectomy (09; 173; 13). Additionally, cerebral angiography helps exclude pseudoaneurysm formation and allows injection of intra-arterial thrombolytic (12).

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Contributors

All contributors' financial relationships have been reviewed and mitigated to ensure that this and every other article is free from commercial bias.

Author

Adrian Marchidann MD

Dr. Marchidann of Kings County Hospital has no relevant financial relationships to disclose.
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Editor

Steven R Levine MD

Dr. Levine of the SUNY Health Science Center at Brooklyn has no relevant financial relationships to disclose.
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Former Authors

Devin Brown MD
Clarke Haley MD
Ashfaq Shuaib MD
Fintan O'Rourke MD
Naeem Dean FRCP MBBS
Sombat Muengtaweepongsa MD
Salvador Cruz-Flores MD

Patient Profile

Age range of presentation: 0 month to 65+ years
Sex preponderance: female>male, >2:1
Heredity: heredity may be a factor

autosomal dominant
Population groups selectively affected: none
Occupation groups selectively affected: none

ICD & OMIM codes

ICD-10: Benign neoplasm of the heart, myxoma: D15.1
ICD-11: Benign neoplasm of intrathoracic organs: 2F01
OMIM: Atrial myxoma (familial): #255960

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Atrial myxoma

Associated Disorders

Carney complex
Congestive heart failure
Lentigines, atrial myxoma, blue nevi syndrome
Nevus, atrial myxoma, neurofibroma, ephelides syndrome
Stroke

Differential Diagnosis

mitral stenosis
endocarditis
rheumatic disease
collagen vascular diseases
intracardiac thrombi
- intracardiac tumors
- cardiac lymphoma
- left atrial metastasis
- leiomyosarcoma
- vasculitis

Atrial myxoma …

Atrial myxoma

Introduction

Overview

Key points

Historical note and terminology

Clinical manifestations

Presentation and course

Syndromes

Prognosis and complications

Clinical vignette

Biological basis

Etiology and pathogenesis

Epidemiology

Prevention

Differential diagnosis

Confusing conditions

Diagnostic workup

Management

Special considerations

Pregnancy

Anesthesia

References

Contributors

Author

Editor

Former Authors

Patient Profile

ICD & OMIM codes

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Questions or Comment?

Also in This Category

Vein of Galen malformations

Patent foramen ovale

Neuroimaging in acute stroke

Anterior cerebral artery stroke syndromes

Hemorrhagic transformation of ischemic stroke

Intracerebral hemorrhage due to thrombolytic therapy

White matter abnormalities in the brain

Thalamic hemorrhage

	• Myxoma is the most common cardiac tumor in adults.
	• Atrial myxoma may occur in all ages.
	• Familial cases may occur as part of a syndrome.

	• Screening plays an important role in early detection of atrial myxomas.
	• Semiannual screening echocardiography is used to detect recurrence after resection.
	• Recurrence after surgery is usually due to incomplete resection.

Atrial myxoma

Introduction

Overview

Key points

Historical note and terminology

Clinical manifestations

Presentation and course

Syndromes

Prognosis and complications

Clinical vignette

Biological basis

Etiology and pathogenesis

Epidemiology

Prevention

Differential diagnosis

Confusing conditions

Diagnostic workup

Management

Special considerations

Pregnancy

Anesthesia

References

Contributors

Author

Editor

Former Authors

Patient Profile

ICD & OMIM codes

This is an article preview. Start a Free Account to access the full version.

Questions or Comment?

Also in This Category

Vein of Galen malformations

Patent foramen ovale

Neuroimaging in acute stroke

Anterior cerebral artery stroke syndromes

Hemorrhagic transformation of ischemic stroke

Intracerebral hemorrhage due to thrombolytic therapy

White matter abnormalities in the brain

Thalamic hemorrhage

This is an article preview.
Start a Free Account
to access the full version.