Cardiac myxomas are the commonest amongst benign tumors of the heart [1]. Although they have been reported in individuals of all ages, they are usually seen between the third and the sixth decade of life. Sporadic myxomas occur more commonly in women and the female to male ratio has been reported to be 2.7:1 [2]. A majority of the myxomas occur on the left side of the heart while 25% occur on the right side [3].

A small proportion of the cardiac myxomas are familial in origin [4] and can either be part of a syndrome (Carney's complex with its subsets LAMB syndrome or NAME syndrome) [5] [6] [7] [8] or can be non-syndromic [9]. The familial Carney's complex is inherited in an autosomal dominant manner and characterized by skin pigmentation, cardiac and soft tissue myxomas, accompanied by endocrine dysfunction. Compared to sporadic myxomas, familial cardiac myxomas have been reported to occur at an earlier age, have less female preponderance and a higher incidence of right heart involvement and recurrence [4].

Affected individuals usually present with symptoms secondary to embolism, mechanical obstruction within the heart or with constitutional symptoms (classic Goodwin's triad). A minority of the patients with myxomas are asymptomatic [10] and they are detected incidentally. The site of the embolization depends on whether the myxoma is located on the right or the left side of the heart and the presence or absence of an intracardiac shunt. Other presenting features include those due to mitral regurgitation and conduction abnormalities like atrial fibrillation or flutter.

• Pathologist

  The book is geared to pathology residents and practicing surgical pathologists and focuses on illustrated practical diagnosis, including differential diagnosis. [books.google.de]

• Heart Disease

  Provides a clinically based approach to echocardiography, with an in-depth discussion of the main cardiac events seen in practice, including adult congenital heart disease. [books.google.de]

  Braunwald's Heart Disease: A Textbook of Cardiovascular Medicine. 11th ed. Philadelphia, PA: Elsevier; 2019:chap 95. Mckenna WJ, Elliott P. Diseases of the myocardium and endocardium. In: Goldman L, Schafer AI, eds. Goldman-Cecil Medicine. 25th ed. [medlineplus.gov]

  In: Primary tumors of the heart, chapter 49. Heart Disease: A Textbook of Cardiovascular Medicine. 6th edition. Edited by Braunwald E, Zipes D, Libby P. Philadelphia, Pennsylvania, USA, W.B. Saunders Company 2001; 2: 1809-1819. [alliedacademies.org]

  In article View Article [16] McManus, B., “Primary tumors of the heart”, In Bonow, R.O., Mann, D.L., Zipes, D.P., Libby, P. (eds) Braunwald’s Heart Disease, 9th ed, Philadelphia, Elsevier Saunders, 2011, 1638-1650. [pubs.sciepub.com]

  Many children are already known to have heart disease before the recognition of cerebrovascular disease, but in other instances, a less obvious cardiac lesion is discovered only after a stroke. [pediatrics.aappublications.org]

• Diastolic Murmur

  Typical examination findings include abnormal heart sounds, such as a rumbling diastolic murmur over the apex or a characteristic “ tumor plop.” The diagnosis is not easily established clinically because of the nonspecific nature of symptoms. [amboss.com]

  A tumor plop may be followed by a low pitched diastolic murmur. If the mitral valve inflow obstruction is significant enough, physical exam findings of congestive heart failure will be present. Treatment Treatment is universally surgical excision. [healio.com]

  These abnormalities may include a holosystolic murmur of mitral insufficiency, a systolic ejection murmur, a diastolic murmur of mitral stenosis, a loud S1 or S2, an S4, or a tumor plop [ 1, 10, 12, 13 ]. [pubs.sciepub.com]

  Also detected were a pan-systolic murmur at the lower left sternal border, an early diastolic heart sound with a mid diastolic murmur at the apex, bibasilar crackles, ascites, and oedema up to the thighs. [bjmp.org]

  A pulmonary ejection murmur with a delayed and accentuated pulmonic second sound may be heard. There may also be an early, late or prolonged diastolic murmur heard. [atlasgeneticsoncology.org]

• Cardiac Signs and Symptoms

  Cardiac myxoma produces protean clinical manifestations that do not always include cardiac signs and symptoms. Neurologists may be called on for diagnostic consultation in patients who will prove to have cardiac myxoma. [neurology.org]

  Neither child had a history or other signs of cardiac disease. [pediatrics.aappublications.org]

• Prolapse

  If the mass is pedunculated, the motion within the heart can be demonstrated, including prolapse through the mitral valve. MRI MRI appearances are heterogeneous, reflecting the non-uniformity of these masses. [radiopaedia.org]

  While a thrombus is smaller than a myxoma, the myxoma has specific morphological characteristics (shape, mobility and ability to prolapse) which help to identify it. [symptoma.com]

  Fig. 1b 3D full volume of the MV LAX displaying a large left atrial myxoma prolapsing through the mitral valve. Fig. 1c 3D full volume of a left atrial myxoma viewed from the left atrium. [pie.med.utoronto.ca]

  Intermittent Prolapse of Right Atrial Myxoma in a Four-Year-Old Girl, J Compr Ped. 2014 ; 5(4):e22931. doi: 10.17795/compreped-22931. Abstract Copyright © 2014, Iranian Society of Pediatrics. [comprped.com]

The workup of the patient depends on their manner of presentation. In all cases, a detailed history and physical examination are mandatory. If familial cardiac myxoma is suspected from family history then a detailed physical examination for other features of the syndrome should be performed.

Laboratory studies are not specific and findings can include hemolytic anemia (secondary to mechanical damage of erythrocytes), elevated levels of erythrocyte sedimentation rate (ESR), C-reactive protein (CRP) and serum gamma globulin. Levels of serum interleukin- 6 may be elevated and they are used as a marker for determining recurrence.

Electrocardiography (ECG) is performed in all patients and may show atrial fibrillation, flutter or other conduction abnormalities. Two-dimensional echocardiography (2D ECHO) is used to visualize all the cardiac chambers and to evaluate the location, size, and mobility of the myxoma. Transesophageal echocardiography is a more sensitive test than the 2D ECHO and detects myxomas as small as 1 mm in diameter.

Chest radiographs are used to identify pulmonary edema, tumor calcification, and cardiomegaly while magnetic resonance imaging (MRI) detects tumor size, mobility and its site of attachment. Computed tomography (CT scan) is performed to differentiate between a myxoma and an intracardiac thrombus. While a thrombus is smaller than a myxoma, the myxoma has specific morphological characteristics (shape, mobility and ability to prolapse) which help to identify it [11].

PRKAR1A molecular genetic testing is recommended in all patients suspected to have Carney's complex and if positive, then their family members are also evaluated for cardiac myxomas [12]

• Thrombocytosis

  Diagnostic Tests: Anemia, leukocytosis, increased ESR, thrombocytosis or thrombocytopenia, hypergammaglobulinemia, and hypocomplementemia are common. Imaging : Diagnosis is usually made by echocardiography, but CT or MRI can also be used. [rheumaknowledgy.com]

Treatment with intravenous recombinant tissue plasminogen activator (rtPA) and intra-arterial thrombolysis has been described previously but mechanical thrombectomy has not yet been reported, and treatment of myxoma-related ischemic stroke remains a clinical [ncbi.nlm.nih.gov]

Prognosis Excellent after excision. atrial myxoma Cardiology The most common 1º cardiac neoplasm, age of onset 25-55 Clinical Symptoms may be obstructive–right-sided congestion—most are right-sided, ± ascites, constitutional–fever, fatigue, arthralgias [medical-dictionary.thefreedictionary.com]

In general, the prognosis of infected cardiac myxoma is relatively benign and the long-term survival is always promising. [ncbi.nlm.nih.gov]

The prognosis of Cardiac Myxoma depends upon the location, size, and number of tumors It is also dependent upon the severity of underlying signs and symptoms associated with the syndrome (Carney complex) Sporadic tumors often have good prognosis upon [dovemed.com]

Each chapter includes eight to ten gross and microscopic photographs with legends highlighting the pathologic features of the disease, as well as succinct text covering etiology, clinical aspects, and differential diagnosis. [books.google.de]

Abstract Most cases of atrial myxoma are sporadic, and the exact etiology is unknown. We examined if herpes simplex virus (HSV)-1 and HSV-2 antigens and/or DNA could be detected in a cohort of Iranian patients with cardiac myxomas. [ncbi.nlm.nih.gov]

Etiology Clinical features General Clinical features caused directly by the tumor Symptoms caused by obstruction Dyspnea on exertion, paroxysmal nocturnal dyspnea, and/or orthopnea Dizziness or syncope Palpitations Auscultatory findings Low pitched, mid [amboss.com]

Etiology Sporadic – 90%. Familial – 10%. May form part of Carney's complex (Carney's, LAMB, NAME syndrome) or non-syndromic familial cardiac myxomas 10,11. Pathophysiology Originate from the endocardium. [pie.med.utoronto.ca]

(Etiology) The cause of development of Cardiac Myxoma is generally unknown. [dovemed.com]

Epidemiology Most common primary cardiac neoplasm (usually benign) Most common location: left atrium ( ∼ 75% of all cases ) Sex: ♀ > ♂ (3:1) Peak incidence : 40–60 years References: [1] [2] [3] Epidemiological data refers to the US, unless otherwise specified [amboss.com]

This timely volume covers history, epidemiology, demographics, clinical diagnosis, pathology, imaging by echo, CT and MRI of both benign and malignant cardiac tumors, either primary or secondary. [books.google.de]

Epidemiology Cardiac myxomas can occur across all age groups, however, they occur with the greatest frequency amongst between the third and sixth decades of life. [atlasgeneticsoncology.org]

Epidemiological and pathological characteristics of cardiac tumors: A clinical study of 242 cases. Interact CardiovascThorac Surg 2007; 6: 636-639. [alliedacademies.org]

Unusual components (i.e. glands, lymphoid proliferation, thymic rests) may be noted if present ICD coding ICD-9: 212.7 - benign neoplasm of heart ICD-10: D21.9 - benign neoplasm of connective and other soft tissue ICD-10: D15.1 - benign neoplasm of heart Epidemiology [pathologyoutlines.com]

(J Thorac Oncol 2016;11:441) 90% occur in atria, 75% on left side and localized to the interatrial septum, close to fossa ovalis 90% are solitary and pedunculated tumors Can be found in any other cardiac chamber (right atrium, ventricles and valves) Pathophysiology [pathologyoutlines.com]

Pathophysiology Originate from the endocardium. Composed of embryonic multipotential mesenchymal cells that persist during cardiac septation which can differentiate into angioblasts, fibroblasts, cartilage and myoblasts. [pie.med.utoronto.ca]

Molecular analysis of the cyclic AMP-dependent protein kinase A (PKA) regulatory subunit 1A (PRKAR1A) gene in patients with Carney complex and primary pigmented nodular adrenocortical disease (PPNAD) revels novel mutations and clues for pathophysiology [revespcardiol.org]

Pathophysiology Myxomas account for 40-50% of primary cardiac tumors. Approximately 90% are solitary and pedunculated, and 75-85% occur in the left atrial cavity. Up to 25% of cases are found in the right atrium. Most cases are sporadic. [emedicine.medscape.com]

We describe a multidisciplinary approach applying cerebrovascular stent retriever devices in tumor embolectomy of the renal artery of a young patient with systemic morbidity, preventing safe laparotomy for open surgical tumor embolectomy. [ncbi.nlm.nih.gov]

Presently, the sporadic cases of Cardiac Myxoma cannot be prevented. Also, there are no specific methods or guidelines to prevent tumor formation, if it occurs in association with Carney complex, which is a genetic condition. [dovemed.com]

However I am not quite sure, whether the sole and meticulous resection of the endocardium is sufficient to prevent recurrency in a primary mesenchymal tumor. [ctsnet.org]

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2. Zheng JJ, Geng XG, Wang HC. et al. Clinical and histopathological analysis of 66 cases with cardiac myxoma. Asian Pac J Cancer Prev. 2013;14(3):1743-6.
3. Nath M P, Singh B, Chakrabarty A. Left atrial myxoma presenting as stroke: Case report and review of literature. The Indian Anaesthetists’ Forum 2011;1:1-6.
4. Farah MG. Familial cardiac myxoma. A study of relatives of patients with myxoma. Chest 1994;105:65–8.
5. Carney JA, Gordon H, Carpenter PC, Shenoy BV, Go VL. The complex of myxomas, spotty pigmentation, and endocrine overactivity. Medicine (Baltimore) 1985;64:270–83.
6. Carney JA. Psammomatous melanotic schwannoma. A distinctive, heritable tumor with special associations, including cardiac myxoma and the Cushing syndrome. Am J Surg Pathol 1990;14:206–22.
7. Yen RS, Allen B, Ott R, Brodsky M. The syndrome of right atrial myxoma, spotty skin pigmentation, and acromegaly. Am Heart J 1992;123:243–4.
8. Krause S, Adler LN, Reddy PS, Magovern GJ. Intracardiac myxoma in siblings. Chest 1971;60:404–6.
9. Dandolu BR, Iyer KS, Das B, Venugopal P. Non-syndrome familial atrial myxoma in two generations. J Thorac Cardiovasc Surg 1995;110:872–4.
10. Ture P, Kurdi M, Hiregoudar S, Hegde G. Anesthetic management of a patient with asymptomatic atrial myxoma for hernia repair. Anaesth, Pain and intensive Care. 2016 Apr-Jun; 20(2): 246 -248
11. 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. 2009 Mar;192(3):639-45.
12. 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. 2011 Apr;12(4):624.