Atrial myxoma …

Adrian Marchidann MD

Stroke & Vascular Disorders

Updated 08.23.2023
Released 06.11.2001
Expires For CME 08.23.2026

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 cutaneous syndromes associated may facilitate timely recognition. Its protean clinical manifestations make the clinical diagnosis difficult. Approximately 60% of all tumors are found 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, mediated by numerous humoral factors.
	• 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 (32).

The first nonembolic 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 (52). Sneddon syndrome, which commonly presents with a livedo-type rash and diffuse cerebrovascular disease, has also been associated with atrial myxoma (150).

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 protean manifestations, diagnosis of atrial myxoma may take a long time to recognize, up to 126 months in one series (125). Patients may present with fatigue, shortness of breath, fever of unknown origin, and various embolic symptoms (25).

Embolization. Embolic tumor fragments or thrombi may occur in 30% to 40% of patients with atrial myxomas. Embolization can occur to any organ leading to failure, depending on the myxoma location (135; 127). In a single center study encompassing 93 patients with atrial myxoma, predictors of embolization were atrial fibrillation, myxoma diameter larger than 4.6 cm, enlarged left atrium, and irregular tumor surface (69). Additionally, 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 significant risk factors of embolism (93).

Cerebrovascular disease. Approximately 45% of patients with atrial myxoma experience neurologic deficits (79). Myxomas cause 0.4% of strokes, either ischemic or hemorrhagic. Most strokes occur by the age of 50 (125).

Transient ischemic attack is the most common initial neurologic manifestation of atrial myxoma (04). Global transient amnesia may also be triggered by atrial myxoma (155).

Strokes are often recurrent. Brain infarcts can also accrue asymptomatically resulting in multi-infarct dementia (74; 61). Alternatively, they may present with rapid multi-organ failure and death due to embolic showers or even a single massive embolic, potentially fatal stroke (20; 16). Monocular blindness may be caused by ocular embolism (166). Paraplegia may result from embolism to the anterior spinal cord artery or abdominal aorta (58; 174).

Aneurysms result from the mural invasion of the myxomatous emboli, which typically lodge in the distal branches of middle cerebral arteries. The aneurysms may be multiple and bilateral (22; 129; 33; 103). Approximately 90% of aneurysms are fusiform; the remaining are saccular (142).

Cardiovascular disease. Left atrial myxoma may embolize into the coronary, causing myocardial infarction (119). Right atrial myxoma embolization results in pulmonary embolism, right heart failure, and ascites (121; 67; 96).

Pulmonary embolism is usually caused by right atrial myxoma and may be the initial presentation of myxoma (94; 31). Even left atrial myxoma can present as pulmonary embolism. Because there was no shunt, the most likely cause was a hypercoagulable state promoted by myxoma (133).

Cutaneous embolization cause acral erythematous papules, petechiae, digital cyanosis, splinter hemorrhages, livedo reticularis, and Raynaud phenomenon (110; 52). Constitutional symptoms associated with a purpuric rash of unknown origin should prompt evaluation for atrial myxoma (95).

Gastrointestinal manifestations depend on the embolism location. Abdominal pain aggravated by eating or drinking and associated with lactic acidosis suggest small bowel ischemia (63). Small bowel obstruction may also occur (181). Right atrial myxoma may cause Budd-Chiari syndrome (73).

Limb ischemia may occur due to systemic embolization and requires immediate revascularization (188).

Cardiac and obstructive symptoms. The cardiac symptoms correlate with the tumor location, size, and mobility (50). Very rarely, myxoma is found in both atria.

Cardiac structural complications include mitral valve regurgitation (75), mitral valve obstruction with congestive heart failure (125), left ventricular failure (38; 96), and hypertrophic cardiomyopathy (71). A steal phenomenon in the large anterior descending artery was also described (159).

Cardiac arrhythmias like atrial fibrillation or paroxysmal supraventricular tachycardia may be triggered by atrial myxoma (21; 152).

Endocarditis is suggested by cardiac vegetations with positive cultures for histoplasma and Streptococcus sanguinis (45; 87; 137). Hypercoagulability in patients with myxomas was associated with elevated IL-6 and IL-8 (14; 15).

Compression by a giant myxoma may lead to dysphagia, Horner syndrome (anhidrosis, partial ptosis, and miosis), dyspnea, and cough-syncope syndrome (51; 105; 40).

Sudden death may occur due to obstruction of the intracardiac blood flow (78). Simultaneous embolization of the heart and brain circulation may lead to fatal cardiac arrest and stroke (66).

Auscultatory findings are associated with mitral valve dysfunction caused by larger lesions (50). A murmur suggesting mitral stenosis is heard in 54% (125). 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 (135; 50).

Syndromes

NAME syndrome. The acronym NAME stood initially for nevi, atrial myxoma, myxoid neurofibromata, and ephelides (07). Later, ephelides was replaced by endocrine overactivity due to increased insulin-like growth factors level (185). Transmission is usually autosomal dominant, but X-linked dominant transmission may also occur (82).

LAMB syndrome. LAMB syndrome is characterized by mucocutaneous lentigines, cardiomucocutaneous myxomas, and multiple blue nevi (136).

Carney complex is an autosomal dominant multiple neoplasia associated with myxomas, mottled cutaneous pigmentation, endocrine dysfunction, and nonmyxomatous extracardiac tumors (eg, breast fibroadenomas in women and testis Sertoli-cell tumors in men) (163; 184). The Carney complex, both familial and sporadic, is associated with mutation of the PRKAR1A gene (162; 17; 160). Cutaneous embolism may result in livedoid macules (110). Cushing syndrome and acromegaly are also associated with Carney complex (176).

Paraneoplastic syndromes. Paraneoplastic syndromes associated with myxomas may occur due to increased levels of humoral mediators. Several categories of paraneoplastic syndromes were described: constitutional symptoms, vasculitis/vasculopathy, hematological, constitutional, and other, uncommon manifestations (158). These manifestations resolve after tumor resection.

Constitutional symptoms like fever, fatigue, myalgia, arthralgia, and weight loss occur in up to 90% of atrial myxoma cases (85; 125). These symptoms mimic rheumatological disease and may be masked by nonsteroidal anti-inflammatories (18).

Vasculitis or vasculopathy syndromes include Raynaud phenomenon (101), livedo reticularis (89), malar erythematous violaceous eruption with peripheral cyanosis, splinter hemorrhages, serpiginous, annular, violaceous, and nonblanching lesions of fingertips (60), medium vessel vasculitis, erythema and petechiae of hands and feet, acral erythematous papular eruption (122), and biphasic changes in color of fingertips on cold exposure (23).

Hematologic manifestations include anemia, leukocytosis, increased acute phase reactants (85), thrombocytopenia (178), antiphospholipid antibodies (146), depletion of factor VII (42), systemic AL amyloidosis (106), and generalized AA amyloidosis (111).

Uncommon manifestations include iridocyclitis (Plewan et el 1996), worsening of coexistent systemic lupus (186), acute pancreatitis (183), acute renal failure (55), proteinuria (70), painful peripheral sensory neuropathy (147; 104), mimicking of polyarteritis nodosa (148), and mediastinal lymphadenopathy (168).

Prognosis and complications

Intracerebral or subarachnoid hemorrhage may complicate the development of cerebral aneurysms. The aneurysms may regress and resolve after myxoma resection (33) but can also enlarge or even appear many years later (141; 179; 68).

Metastases to the vasculature walls may cause occlusion and infarction (35). They may penetrate through the vessel wall to form intra-axial metastases (11). Cerebral metastases may be diagnosed several years after resection of myxoma (30).

Recurrent embolization may occur despite anticoagulation (79).

Endocarditis due to infection of atrial myxoma may occur rarely (170).

The recurrence rate of myxoma is 1% to 3% in the sporadic cases, is often due to incomplete resection (100; 180). Recurrence may be up to 25% in the familial cases.

Vanished myxoma by systemic embolization was described (34).

Malignant transformation can rarely occur (145; 92).

Clinical vignette

A 56-year-old Caucasian woman with history of mild static encephalopathy developed acute right hemiparesis and dysarthria. She was afebrile, had a blood pressure of 123/71, and pulse of 79. General exam was remarkable for the lack of cervical bruits and a 2/6 diastolic rumble consistent with mitral stenosis. Peripheral pulses were intact. Neurologic exam showed 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 a 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. 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 (135) and tend to present earlier than the sporadic ones. Most cardiac myxomas appear in the left atrium in the region of the fossa ovalis of the interatrial septum (135). Macroscopically, myxoma is a solid mass, pedunculated, shiny, and irregular (153); however, myxoma may also be cystic (120).

Myxomas originate from mesenchymal cells capable of endothelial differentiation surrounded by a myxoid extracellular matrix rich in proteoglycans (135; 144). Deriving from adult developmental remnants (116), 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). Overexpression of matrix metalloproteinase contributes to degradation of the extracellular matrix, increasing the risk of embolism (117).

The constitutional symptoms are mediated by interleukin-6 (IL-6) (151) in up to 74% of cases (01). IL-6 is secreted by the immature mesenchyme cells of myxomas (167; 165).

Resection often leads to normalization of serum levels of IL-6 and resolution of the symptoms (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 (142; 158; 41).

Mutation of the tumor suppressor PRKAR1-alpha gene on chromosome 17q22-24 has been identified in the Carney complex (77; 184). 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 (175). Screening for this mutation identifies family members at high risk of developing myxomas (10; 06). The most commonly involved regions in sporadic cases are 12p1 and 17p1. Dicentric chromosomes and telomeric associations are the most frequent genetic abnormalities (37).

Myxomas associated with 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 potentially detect malignancy in myxoma (118).

No association of myxoma with either human papillomavirus (HPV) or Epstein-Barr virus was found (27).

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 brain with and without contrast and MR angiography of 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 (161). Elevated C-reactive protein, thrombocytosis or thrombocytopenia, serum globulins, antinuclear antibodies, and rheumatoid factor may suggest an infectious or autoimmune condition (135; 59). Blood cultures are needed to exclude infection (134). Streptococci and staphylococci are the most frequent organisms found in blood culture (53; 173; 65). However, histoplasma capsulatum has also been reported (08).

MRI of the brain. The deep white matter lesions seen on MRI mimic multiple sclerosis or metastatic lesions (112; 131; 182). Gadolinium brain MRI may detect fusiform aneurysms (113). 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 as well as paroxysmal atrial fibrillation (21).

Chest x-ray. Chest x-ray may reveal left atrial enlargement, blood diversion to upper lobes, left ventricular enlargement, and calcification of the myxoma (161).

Transthoracic echocardiography is the diagnostic modality of first choice. 3-D echography helps determine the origin of the stalk and characterize the heterogeneity of the myxoma (187). Echography has a false-negative rate of 21% for detecting atrial masses, whereas transesophageal echocardiography (TEE) has a sensitivity of 100% for atrial myxoma (177). TEE better localizes the area of attachment, information needed for surgical planning (169).

Myxoma appears as a smooth globular surface, sometimes irregular and friable with a narrow stalk. Echolucent areas represent hemorrhage or necrosis (50). Echolucency helps distinguish myxomas from thrombi or vegetations. Larger size of myxoma correlates with constitutional, hemodynamic, and obstructive symptoms, whereas smaller size correlates with embolic phenomena (46; 50). Polypoid appearance also correlates with embolic phenomena (54).

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 a hypervascular mass (05). However, cardiac MRI is less sensitive than TEE (140).

Noncontrast chest CT helps detection of atrial myxomas (154) and preoperative evaluation (43). Cardiac contrast-enhanced CT may differentiate atrial myxoma from other intracardiac lesions like thrombi, fibroelastomas, or metastatic tumors and is helpful for preoperative assessment (24; 56).

FDG-PET/CT showing mildly hypermetabolic hypodense area in the atrium is useful for detecting myxomas in patients with systemic symptoms (48) 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 (139). CT coronary angiography also helps to distinguish myxomas from thrombi (149). Coexistent coronary artery disease may require both coronary artery bypass grafting and myxoma resection (39).

Management

	• There is no medical therapy for myxoma.
	• Anticoagulation may not be protective.
	• Surgical resection is the definitive treatment.
	• After resection, screening for recurrence with 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 (124; 64). Cardiac arrhythmia, occurring in 26% of patients, is the most common postoperative complication. Persistent bradycardia requires pacemaker placement in less than 2% of cases (125). Postoperative mortality ranges between 2.2% to 3.5% (86; 125).

Total robotic resection is relatively safe and causes minimal cosmetic complications (47; 114). Multiple aneurysms may be approached with a combination of endovascular and open surgical treatment (123). Minimally- invasive procedures make possible removal of increasingly large lesions (29).

Transthoracic echocardiography is used after resection to screen for recurrence (128). Most recurrences occur within the first two years, but there may be a delay up to 12 years (50). Addition of cryoablation to resection for right atrial myxoma helps preserve the cardiac conduction system (97). Percutaneous ablation with retrieval is another option (81).

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 (62; 108; 115; 164; 36). Significant improvement after endovascular thrombectomy was seen even in absence of intravenous tPA (172). However, the outcome is better outcome with earlier recanalization (98).

Because of the short therapeutic window, hemorrhagic transformation and subarachnoid hemorrhages are the most feared complications (26; 28). 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 case reports showed benefit from endovascular thrombectomy (09; 171; 13). Additionally, cerebral angiography helps exclude pseudoaneurysm formation and allows injection of intra-arterial thrombolytic (12).

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Former Authors

Devin Brown MD, Clarke Haley MD (original authors), Ashfaq Shuaib MD, Fintan O’Rourke MD, Naeem Dean MBBS, Sombat Muengtaweepongsa MD, and 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-9: Benign neoplasm of the heart, myxoma: 212.7
ICD-10: Benign neoplasm of the heart, myxoma: D15.1
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

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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Also in This Category

Vein of Galen malformations

Hemorrhagic transformation of ischemic stroke

Thalamic hemorrhage

Infective endocarditis

Stroke associated with drug abuse

Carotid-cavernous fistulas

Amaurosis fugax

Epidural hematoma

	• Myxoma is the most common heart 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

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

Hemorrhagic transformation of ischemic stroke

Thalamic hemorrhage

Infective endocarditis

Stroke associated with drug abuse

Carotid-cavernous fistulas

Amaurosis fugax

Epidural hematoma

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