Myxoma overview
| Myxoma | |
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| Left Atrial Myxoma. Case courtesy of Radiopaedia.org, rID: 11275 [1] | |
| ICD-10 | D21.9 |
| ICD-9 | 212.7 |
| DiseasesDB | 1075 |
| MedlinePlus | 000755 |
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Myxoma Microchapters |
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Diagnosis |
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Treatment |
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Case Studies |
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Myxoma overview On the Web |
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American Roentgen Ray Society Images of Myxoma overview |
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor-In-Chief: Maria Fernanda Villarreal, M.D. [2]Cafer Zorkun, M.D., Ph.D. [3]Ahmad Al Maradni, M.D. [4]
Overview
A myxoma (Myxo- = Latin for mucus) is the most common primary tumor of the heart. Myxomas are usually located in either the left or right atrium of the heart; about 86 percent occur in the left atrium.[2] Myxomas are typically pedunculated, with a stalk that is attached to the interatrial septum. The most common location for attachment of the stalk is the fossa ovalis region of the interatrial septum. The phrase "myxomatous degeneration" refers to the process in which connective tissue becomes filled with mucus. About 71% of myxomas occur in the heart, 41% on the skin, and 7% in the oral cavity (usually on the palate). Common physical examination findings of myxoma include murmur and abnormal heart sounds that change when the patient changes positions. Complications that can develop as a result of myxoma are: arrhythmias, pulmonary edema, peripheral emboli, metastasis, blockage of the mitral heart valve. Surgery is the mainstay of treatment for myxomas.
Historical Perspective
In 1845, the first description of a left atrial myxoma was reported by King.[3] In 1951, Prichard described a kind of microscopic endocardial structure of the atrial septum, which was suggested to be associated with cardiac myxoma.[4] Clarence Crafoord a Swedish cardiovascular surgeon, successfully removed a cardiac myxoma for the first time in 1954. This case also represented the first extirpation of a cardiac tumor on cardiopulmonary support. [5]. Before 1951, cardiac myxoma diagnosis was made only at autopsy. In 1959, the first M-mode echocardiogram of a left atrial myxoma was reported. [6]
Classification
Cardiac myxomas are classified by the WHO histological classification of tumors of the heart "Benign tumors and tumor-like lesions" and categorized into a type of pluripotent mesenchymal tumor. [7] [8]
Pathophysiology
Cardiac myxoma arises from remnants of subendocardial vasoformative reserve cells, which are primitive mesenchymal cells that are normally involved in the supportive structure of the endocardium. [9] [10] Myxomas are usually located in the fossa ovalis and endocardium of the atrial septum
Some symptoms of myxoma may be associated with the release of interleukin 6 (IL-6) [11][12] On gross pathology, a gelatinous, irregular surface that fills the left atrium is characteristic finding of myxoma. A common hystopathological finding is the Gamna-Gandy Bodies that consist of fibrosis and deposition of iron pigments.
Causes
About 10% of myxomas are passed down through families (inherited). Right atrial myxomas are sometimes associated with tricuspid stenosis and atrial fibrillation, however the underlying mechanism remains unknown. [13]
Differentiating Myxoma from other Diseases
Myxoma should be differentiated from other cardiac tumor including lipoma, rhabdomyoma, rhabdomyosarcoma, thrombosis and metastasis.
Epidemiology and Demographics
Cardiac myxomas are the most common primary cardiac tumor in adults, but are relatively infrequent in childhood. There is a wide range in the age of presentation (11-82 years), with most patients presenting in adulthood (mean ~50 years of age). There is a recognized female predilection.[14][15]The incidence of benign cardiac tumor including myxoma is about 250 per 100,000 persons.[16]
Risk Factors
Common risk factors in the development of myxoma are female gender and genetic predisposition.
Natural History, Complications and Prognosis
Complications that can develop as a result of myxoma are: arrhythmias, pulmonary edema, peripheral emboli, metastasis, blockage of the mitral heart valve.
Diagnosis
Staging
There is no established system for the staging of myxoma.
History and Symptoms
Atrial myxoma symptoms may occur at any time, but most often they tend to occur with changes in body position. Common symptoms include: chest pain, palpitation, dizziness, syncope and dyspnea on exertion.
Physical Examination
Common physical examination findings of myxoma include murmur and abnormal heart sounds that change when the patient changes positions.
Laboratory Findings
Laboratory findings consistent with Myxoma show anemia, leukocytosis and elevated erythrocyte sedimentation rate.
Chest X-ray
On chest x-ray, Myxomas are characterised by calcification overlying the posterior aspect of heart.
CT Scan
On myxoma CT scan is characterised by low attenuation and areas of dystrophic calcification in cardiac chambers.
MRI
Findings on MRI of cardiac myxoma include heterogenous appearence, non-uniformity of the masses, spherical or ovoid masses (sessile or pedunculated)[17]
Echocardiography
On cardiac ultrasound, myxoma is characterised by the presence of a heterogeneous pedunculated mass that is commonly located in the left atrium.
Other Diagnostic Studies
Cardiac ventriculography is also used in the diagnosis of myxoma
Treatment
Medical Therapy
There is no known medical therapy for Myxomas. The only treatment available is for the complications derivated from the myxoma.
Surgery
Surgery is the mainstay of treatment for myxomas.
References
- ↑ Cardiac Myxoma: Cases. Radiopedia.URL http://radiopaedia.org/cases/cardiac-myxoma Accessed on November, 18 2015
- ↑ Knepper LE, Biller J, Adams HP Jr, Bruno A. Neurologic manifestations of atrial myxoma. A 12-year experience and review. Stroke. 1988 Nov;19(11):1435-40. (Medline abstract)
- ↑ King TW. On simple vascular growths in the left auricle of the heart. Lancet 1845; 2:428-9
- ↑ Amano J, Kono T, Wada Y, Zhang T, Koide N, Fujimori M, Ito K (2003). "Cardiac myxoma: its origin and tumor characteristics". Ann Thorac Cardiovasc Surg. 9 (4): 215–21. PMID 13129418.
- ↑ Chitwood WR (1992). "Clarence Crafoord and the first successful resection of a cardiac myxoma". Ann. Thorac. Surg. 54 (5): 997–8. PMID 1417305.
- ↑ Pinede L, Duhaut P, Loire R (2001). "Clinical presentation of left atrial cardiac myxoma. A series of 112 consecutive cases". Medicine (Baltimore). 80 (3): 159–72. PMID 11388092.
- ↑ Amano J, Nakayama J, Yoshimura Y, Ikeda U (2013). "Clinical classification of cardiovascular tumors and tumor-like lesions, and its incidences". Gen Thorac Cardiovasc Surg. 61 (8): 435–47. doi:10.1007/s11748-013-0214-8. PMC 3732772. PMID 23460447.
- ↑ Wold LE, Lie JT (1980). "Cardiac myxomas: a clinicopathologic profile". Am. J. Pathol. 101 (1): 219–40. PMC 1903582. PMID 7446701.
- ↑ Roscher AA, Kato NS, Quan H, Padmanabhan M (1996). "Intra-atrial myxomas, clinical-pathologic correlation based on two case studies including historical review". J Cardiovasc Surg (Torino). 37 (6 Suppl 1): 131–7. PMID 10064365.
- ↑ Acebo E, Val-Bernal JF, Gómez-Román JJ (2001). "Prichard's structures of the fossa ovalis are not histogenetically related to cardiac myxoma". Histopathology. 39 (5): 529–35. PMID 11737312.
- ↑ Seino Y, Ikeda U, Shimada K. (1993). "Increased expression of interleukin 6 mRNA in cardiac myxomas". Br Heart J. 69 (6): 565–7. PMID 8343326.
- ↑ Jourdan M, Bataille R, Seguin J, Zhang XG, Chaptal PA, Klein B (1990). "Constitutive production of interleukin-6 and immunologic features in cardiac myxomas". Arthritis Rheum. 33 (3): 398–402. PMID 1690543.
- ↑ Atrial Myxoma.Wikipedia URL https://en.wikipedia.org/wiki/Atrial_myxoma Accessed November 13, 2015
- ↑ Cardiac Myxoma. Radiopedia URL http://radiopaedia.org/articles/cardiac-myxoma Accessed on November 13,2015
- ↑ Yoon DH, Roberts W (2002). "Sex distribution in cardiac myxomas". Am. J. Cardiol. 90 (5): 563–5. PMID 12208428.
- ↑ National Cancer Institute. Physician Data Query Database 2015. http://www.cancer.gov/publications/pdp
- ↑ Myxoma.Radiopedia.http://radiopaedia.org/articles/cardiac-myxoma Accessed on November 13, 2015