The cardiac myxoma is a benign neoplasm arising within the heart. It is sometimes referred to as an atrial myxoma, since the vast majority arise within the atrial septum. For many years, there was a debate on the origin of this tumor. Some thought it represented an organizing thrombus. Today, it has clearly been shown that this is a true neoplasm.
Epidemiology Disease Associations Pathogenesis Laboratory/Radiologic/Other Diagnostic Testing Gross Appearance and Clinical Variants Histopathological Features and Variants Special Stains/
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EPIDEMIOLOGY CHARACTERIZATION SYNONYMS Atrial myxoma INCIDENCE Most common primary tumor of the heart
50% of all primary cardiac tumors
DISEASE ASSOCIATIONS CHARACTERIZATION CARNEY'S COMPLEX
Evidence for genetic heterogeneity of the Carney complex (familial atrial myxoma syndromes).
Milunsky J, Huang XL, Baldwin CT, Farah MG, Milunsky A.
Center for Human Genetics, Boston University School of Medicine, Massachusetts 02118, USA.
Cancer Genet Cytogenet 1998 Oct 15;106(2):173-6 Abstract quote
Myxoma is the most common type of primary cardiac tumor, accounting for 1/3 to 1/2 of all cases. Although a majority are sporadic, about 7% are familial, with autosomal dominant inheritance. The Carney complex refers to the association of atrial myxomas with extracardiac myxomas or Cushing syndrome or both, with or without multiple lentigines and pigmented nevi. The disorder is genetically heterogeneous, with multiple families being linked to 2p16 and a single report of one family not linked.
We investigated two multigenerational kindreds, with 10 members affected by the Carney complex. By using microsatellite markers that span the candidate region, we established haplotypes for affected and unaffected family members. Our two kindreds do not show linkage to the chromosome 2p16 region.
This study provides further evidence for genetic heterogeneity of the gene(s) involved in producing the Carney complex.
- Caspase-3-dependent apoptosis in cardiac myxoma: not associated with human papillomavirus or Epstein-Barr virus.
Chu PH, Jung SM, Lin HC, Yeh CH, Wu HH, Shiu TF, Huang SL, Tseng NM, Chu JJ, Lin PJ, Lai CH.
1The Division of Cardiology, Department of Internal Medicine, Chang Gung Memorial Hospital, Chang Gung University, Taipei, Taiwan.
Mod Pathol. 2005 Jun;18(6):822-7. Abstract quote
Cardiac myxoma is the most common tumor of the heart, has a variable clinical presentation and immunohistochemical profile. Viral infections, such as herpes simplex virus, human papillomavirus (HPV), and Epstein-Barr virus (EBV), may play an important role in the causes of cardiac myxoma. This investigation will demonstrate caspase-3-dependent apoptosis in cardiac myxoma without HPV or EBV infection.
This study included 15 patients with cardiac myxoma, who were treated with surgical excision of the lesion. Data were collected on detailed clinical parameters. Terminal deoxynucleotidyl transferase nick-end labeling assay, electrophoresis, and caspase-3 immunohistochemical studies were performed to characterize apoptosis. Genechip containing 39 subtypes was used to elucidate HPV; and polymerase chain reaction to detect LMP-1 gene of EBV. The patient population comprised of eight (53%) women and seven (47%) men. The mean age of patient participants was 45 years, with an age range of 30-70 years. All patient cases were sporadic myxomas rather than familial myxomas. The patient presentations included dyspnea (53%), asymptomatic (27%), stroke (7%), chest pain (7%), and fever (7%). All lesions were located in the left atrium. The individual patient cases of myxoma did not differ in location or clinical event in terms of pathological scores, such as vascular proliferation, inflammation, cellularity, hyaline, calcification, or thrombosis.
Cardiac myxoma is characterized by apoptosis through caspase-dependent pathway. HPV or EBV was not detected in any of the study patient samples. In conclusion, no viral genomes of HPV or EBV were detected in these 15 patients.
This study demonstrates that caspase-3-dependent apoptosis in cardiac myxoma is not dependent on concurrence of previous HPV and/or EBV infection.
LABORATORY AND RADIOLOGIC CHARACTERIZATION RADIOLOGIC
Cardiac myxomas: clinical and echocardiographic profile.
Goswami KC, Shrivastava S, Bahl VK, Saxena A, Manchanda SC, Wasir HS.
Dept. of Cardiology, Cardiothoracic Centre, All India Institute of Medical Sciences, Ansari Nagar, New Delhi.
Int J Cardiol 1998 Feb 28;63(3):251-9 Abstract quote
We reviewed our clinical and echocardiographic experience in 70 consecutive patients with 73 cardiac myxomas, diagnosed over an 11 year period. There were 21 males and 49 females, ages ranged from 18 to 80 years.
Only in 5.7% cases was the diagnosis of myxomas made clinically. 88.6% cases were initially diagnosed as having: mitral valve disease (70%), tricuspid valve disease (10%), ischemic heart disease (5.7%), cardiomyopathy (2.9%), and the remaining 5.7% were detected during family screening and follow-up. The mean duration of symptoms was 10.6 months. The commonest symptom was dyspnoea (80%), followed by constitutional symptoms (45.7%), embolization (30%), palpitation (25.7%), syncope (15.7%), pedal oedema (15.7%) and pain chest (12.9%). The sites of myxomas were as follows: left atrium, 58; right atrium, 9; and, biatrium, 3. All myxomas except 3 were attached to the interatrial septum. The site, size, shape, attachment, mobility, prolapse into ventricle, and surface characteristic of myxomas were accurately assessed by 2D-echocardiography and confirmed in all (65 of 70) who underwent surgery.
When the morphological characteristic of myxomas were studied and correlated with clinical features large left atrial myxoma size was closely related with constitutional symptoms, congestive heart failure, with syncope and auscultatory findings suggestive of mitral valve disease, whereas smaller myxoma size and irregular surface were associated with embolization. Constitutional symptoms were only present in left atrial myxoma. Post-operative mean echocardiographic follow-up of 60 months showed no recurrence except in 2 with familial myxoma.
We conclude that the majority of myxomas mimic many cardiovascular diseases and were detected in symptomatic patients, so a high index of clinical suspicion is important for its early and correct diagnosis. The size and appearance of the myxomas correlated with the presenting symptoms.
GROSS APPEARANCE/CLINICAL VARIANTS CHARACTERIZATION General
75% located in the left atrium
About 25% located in right atrium
Small percentage are multicentric or intraventricular
Clinical presentation of left atrial cardiac myxoma. A series of 112 consecutive cases.
Pinede L, Duhaut P, Loire R.
Department of Internal Medicine, Edouard Herriot Hospital, 69437 Lyons, France.
Medicine (Baltimore) 2001 May;80(3):159-72 Abstract quote
We analyzed a series of 112 consecutive cases of left atrial myxoma diagnosed in a single French hospital (72 women and 40 men; age range, 5-84 yr) over 40 years, from 1959 to 1998.
Symptoms of mitral valve obstruction, the first arm of the classic triad of myxoma presentation, were present in 75 patients (67%), with mostly cardiac failure or malaise. Symptoms of embolism, the second frequent presentation in the classic triad, were observed in 33 cases (29%) with 1 or several locations, essentially cerebral emboli with stroke.
Males are statistically at greater risk than females of developing embolic complications. The third arm of the classic triad consists of constitutional symptoms (34%) with fever, weight loss, or symptoms resembling connective tissue disease, due to cytokine (interleukin-6) secretion.
Younger and male patients have more neurologic symptoms, and female patients have more systemic symptoms. Seventy-two patients (64%) had cardiac auscultation abnormalities, essentially pseudo-mitral valve disease (53.5%) and more rarely the suggestive tumor plop (15%). The most frequent electrocardiographic sign was left atrial hypertrophy (35%), whereas arrhythmias were uncommon. The greater number of myxoma patients (98) diagnosed preoperatively after 1977 reflects the introduction of echocardiography as a noninvasive diagnostic procedure. However, there was no significant reduction in the average time from onset of symptoms to operation between patients seen in the periods before and after 1977.
The tumor diameter ranged from 1 to 15 cm with a weight of between 15 and 180 g (mean, 37 g). The myxoma surface was friable or villous in 35% of the cases, and smooth in the other 65% cases. Myxomas in patients presenting with embolism have a friable surface; those in patients with cardiac symptoms, pseudo-mitral auscultation signs, tumor plop, and electrocardiogram or radiologic signs of left atrium hypertrophy and dilatation are significantly the larger tumors.
The long-term prognosis is excellent, and only 4 deaths occurred among our 112 cases over a median follow-up of 3 years. The recurrence rate is low (5%), but long-term follow-up and serial echocardiography are advisable especially for young patients.
HISTOLOGICAL TYPES CHARACTERIZATION General Myxoid stellate cells forming multilayered rings around blood vessels
Short cordes and syncytia within a myxoid background
VARIANTS Secondary changes of fibrosis, calcifications, and thrombosis
CHARACTERIZATION Immunoperoxidase Calretinin Am J Clin Pathol 2000;114:754-759
Strong and diffuse cytoplasmic and nuclear staining
PROGNOSIS AND TREATMENT CHARACTERIZATION PROGNOSIS GENERAL
Cardiac myxoma: diagnostic approach, surgical treatment and follow-up. A twenty years experience.
Jelic J, Milicic D, Alfirevic I, Anic D, Baudoin Z, Bulat C, Coric V, Dadic D, Husar J, Ivancan V, Korda Z, Letica D, Predrijevac M, Ugljen R, Vucemilo I.
University Hospital Rebro, Department of Cardiac Surgery, Zagreb, Republic of Croatia.
J Cardiovasc Surg (Torino) 1996 Dec;37(6 Suppl 1):113-7 Abstract quote
At the University Department of Cardiovascular Surgery in Zagreb, Croatia, we treated 81 patients with primary intracardiac myxoma, in a period from January 1975 to December 1994.
There were 55 female and 26 male pts, in age from 1 month to 80 years, mean 46+/-15 years. Clinical manifestations varied from no symptoms and very poor or no clinical signs to various manifestations of chronic or acute congestive heart failure, syncope and arrhythmias with or without systemic findings such as high erythrocyte sedimentation rate, anaemia, leucocytosis, elevated gamma globulin, thrombocytopenia or low grade fever, as well as cerebrovascular accidents due to tumour embolization. Cardiac symptoms were predominant in 54 pts (66.6%) and cerebrovascular in 20 pts (24.7%). Seven pts (8.6%) were symptomless and discovered accidentally, mostly regarding on an unexplained heart murmur. In almost all the patients preoperative diagnosis of intracardiac myxoma was sufficiently established by echocardiography. The tumour was located in the left atrium in 62 pts (76.5%) and in the right atrium in 19 pts (23.5%). Delay from the onset of symptoms to the diagnosis was 6 months in average (range 10 days to 25 months). The average waiting for the operation was 9 days (range from 1 to 60 days).
The echocardiographic diagnosis was confirmed during intraoperative examination followed by histological analysis. All pts underwent excision of myxoma using cardiopulmonary bypass with core and topical hypothermia and cold crystaloid cardioplegia. According to the additional preoperative and intraoperative findings, in 6 pts sinchronous mitral valve reconstruction, in 3 pts artificial mitral valve implantation and in 2 pts atrial wall reconstruction was performed. There was no perioperative mortality. After the operation, we could not evaluate all the patients long enough, mostly because of some paramedical circumstancies, such as war, migrations, etc. Twenty two pts undevent evaluation for at least 5 years after the operation. Among them there was no evidence of the tumour recurrence, 15 pts were asymptomatic and 7 had NYHA II class symptoms. For 17 pts with a left atrial myxoma preoperative and postoperative echocardiographic data were available for comparison, showing a significant reduction of the left atrial diameter (p<0.001) during the postoperative follow-up.
Our data, presenting one of the biggest reports concerning cardiac myxomas, showed a broad spectrum of their clinical presentation, importance of echocardiography in diagnosing and postoperative follow-up and efficacy of a proper surgical intervention as a definite, curative therapy since there were no deaths and no significant cardiac dysfunction neither tumour reccurrence as well.
The long term results of treatment of heart myxomas with special attention to very rare myxoma of the right ventricle.
Gawdzinski MP, Sypula S.
Department of Cardiothoracic Surgery, Central Clinical Hospital, Warsaw, Poland.
J Cardiovasc Surg (Torino) 1996 Dec;37(6 Suppl 1):121-9 Abstract quote
Fifteen patients with cardiac myxomas, 13 in the left atrium, one in the right atrium and one in the right ventricle were treated surgically during a 15 years period with no hospital and no late deaths.
Left atrial myxomas revealed symptoms of obstruction to blood flow in 100%, symptoms of constitutional effects in 55% and thromboembolic events in 23%. Diagnosis of left atrial myxomas was made before operation in eleven patients by echocardiography or angiography or by both methods. In two patients diagnosis of left atrial myxoma was made incidentally during cardiac surgery for mitral stenosis. 8 left atrial myxomas originated from septum and 5 from the wall. Removal of the myxoma with the portion of the septum or the wall was performed in 11 patients and direct suture was satisfactory in 9 cases. In 2 patients septum was repaired by pericardial patch. Septum was left intact in 2 patients.
Follow-up period varied from 1/2 to 15 years, mean-above 8 years. Estimation of late results was achieved by 2-D echocardiography in every patients. Results remain very good, patients are in the NYHA class 1, examinations revealed no recurrences. Special attention was paid to very rare case of huge right ventricular myxoma demonstrating wide infiltration of the endocardium and involvement of the tricuspid valve, which was completely destroyed. The technique of endocardial decortication was used for removal of the myxoma and tricuspid valve had to be replaced. Three months after operation extreme obstruction of the artificial valve was recognized. During second operation valve was cleaned from the thrombus but also fragments of the myxoma probably left during first operation were removed by wider technique of endocardial decortication.
Results after 15 years remains very good. Right ventricular myxoma being on the border of operability needs more aggressive technique than simple removing.
Surgical experience with intracardiac myxomas: long-term follow-up.
Bhan A, Mehrotra R, Choudhary SK, Sharma R, Prabhakar D, Airan B, Kumar AS, Venugopal P.
Cardiothoracic Centre, All India Institute of Medical Sciences, New Delhi.
Ann Thorac Surg 1998 Sep;66(3):810-3 Abstract quote
BACKGROUND: Myxomas are the most common benign intracardiac tumors. This report summarizes our 20-year experience with these tumors.
METHODS: Sixty-six patients (25 male) with a median age of 39 years (range, 6 to 70 years) underwent surgical excision of primary or recurrent intracardiac myxomas during the years 1976 to 1996. Symptom duration ranged from 2 to 8 months. There were 55 left atrial myxomas, 10 right atrial myxomas, and 1 biatrial myxoma. Three of the patients were in one family. The surgical approach comprised complete wide excision.
RESULTS: There were two early deaths. Late follow-up is 89% (57/64) complete. There was one late death, which was not due to a cardiac cause. Echocardiography at a mean follow-up of 66.9 months (range, 7 to 241 months) showed no recurrence of sporadic myxomas. However, 2 of the 3 patients with familial myxomas had recurrence.
\CONCLUSIONS: Surgical excision of atrial myxoma gives excellent short-term and long-term results leading to eventual cure of nonfamilial myxomas. However, familial myxomas retain a strong tendency to recur even 20 years after excision.
ACUTE MYOCARDIAL INFARCTION
Left atrial myxoma associated with acute myocardial infarction.
Isomatsu Y, Nishiya Y, Hoshino S, Hara M, Tsukui H.
Department of Cardiovascular Surgery, Toyama Prefectural Central Hospital, Japan.
Jpn J Thorac Cardiovasc Surg 1999 Sep;47(9):452-4 Abstract quote
We describe a patient with left atrial myxoma associated with acute myocardial infarction. Since hemodynamics were impaired even with the support of an intra-aortic balloon pump, the patient underwent removal of the tumor concomitant with coronary artery bypass grafting to the right coronary artery on the fifth day from infarction onset.
In circumstances of life-threatening of myxoma associated with acute myocardial infarction, removal of myxoma with coronary artery bypass should be performed in an acute phase of myocardial infarction.
Fusiform aneurysms detected 5 years after removal of an atrial myxoma.
Oguz KK, Firat MM, Cila A.
Radiology Department, Faculty of Medicine, Hacettepe University, Ankara, Turkey.
Neuroradiology 2001 Nov;43(11):990-2 Abstract quote
Neurological signs frequently accompany atrial myxomas. Association of multiple cerebral aneurysms in patients with atrial myxomas are less common than embolic infarctions. The development of aneurysms years after the cardiac tumor removal is rare, and few MR images have been reported to date.
A case of multiple peripheral fusiform aneurysms detected 5 years after tumor resection is presented in this report, together with MR images and CT and angiographic images.
Myxoma of the small bowel in a 47-year-old woman with a left atrial myxoma.
Wang Y, Sharkey FE.
Department of Pathology, The University of Texas Health Science Center, San Antonio 78229-3900, USA.
Arch Pathol Lab Med 2003 Apr;127(4):481-4 Abstract quote
Although atrial myxoma is the most common primary tumor of the heart, the synchronous occurrence of myxomas of the intestine and the heart has not been reported in the English literature.
We report a case of a 47-year-old woman who presented with small bowel obstruction by a pedunculated mass that was found to be a myxoma after resection. A left atrial mass was found incidentally by a computed tomographic scan, and a diagnosis of atrial myxoma was confirmed after a second surgery. The cardiac myxoma showed classic histologic features, with tumor cells layered around vascular channels in an abundant myxoid matrix, while the small bowel lesion was less cellular. Immunohistochemical stains yielded identical results in both. No vascular involvement was noted at either site.
This case supports the recommendation that a search for a cardiac lesion should be performed when a myxoma is identified at an unusual location.
Recurrent pulmonary embolism originating from right atrial myxoma.
Alsafwah S, Lababidi Z.
Department of Medicine, Mercy Hospital and Medical Center, Chicago, Ill., USA.
J Am Soc Echocardiogr 2001 Apr;14(4):305-7 Abstract quote
The use of transesophageal echocardiography is a useful adjunct to transthoracic echocardiography in the diagnosis and management of right atrial tumors in patients who are thought to have idiopathic recurrent pulmonary embolism, especially with suboptimal transthoracic echocardiography studies.
We describe a 30-year-old woman with a history of recurrent pulmonary embolism who was admitted for investigation of pleuritic chest pain in whom transesophageal echocardiography played a critical role in the diagnosis and management.
Right atrial myxoma complicated with pulmonary embolism.
Oshiumi M, Hashimoto K, Sasaki T, Takakura H, Hachiya T, Onoguchi K.
Department of Cardiovascular Surgery, Saitama Cardiovascular and Respiratory Center, 1696 Itai, Konan-machi, Osatogun, Saitama 360-0105, Japan
Jpn J Thorac Cardiovasc Surg 2001 Jul;49(7):449-52 Abstract quote
A 25-year-old woman was admitted to our hospital with chest pain and dyspnea, and was diagnosed as having a right atrial myxoma complicated with pulmonary embolism.
An emergency operation was performed with cardiopulmonary bypass. A papillary pedunculated tumor was found having a narrow-based attachment to the free atrial wall. After the tumor was carefully removed together with the atrial wall around the attachment, pulmonary embolectomy was performed. Several fragments of the tumor were removed, and sufficient back-flow from the pulmonary artery was established. The postoperative course was uneventful. However, a non-perfused area was observed in the left lower lung on pulmonary hemodynamic scintigraphy at 3 months after the operation.
Long-term observation is required due to the high risk for metastasis and recurrence, and further surgical treatment remains the most appropriate treatment option. A second operation may be needed to prevent progression in complications.
Recurrence After surgical excision has been reported to occur in 2% of cases Metastasis SKIN
Metastatic atrial myxoma to the skin at 15 years after surgical resection.
Terada Y, Wanibuchi Y, Noguchi M, Mitsui T.
Department of Cardiovascular Surgery, Mitsui Memorial Hospital, Tokyo, Japan.
Ann Thorac Surg 2000 Jan;69(1):283-4 Abstract quote
Metastasis from atrial myxoma is uncommon. We report on a patient who developed metastasis of the left atrial myxoma to the skin at 15 years after surgical resection. The primary lesion had not recurred.
The myxoma has the potential for metastatic growth.
TREATMENT Surgical removal SURGERY
Surgical management of right atrial myxomas. A 15 year experience with review of the literature.
Selvaraj A, Kumar R, Ravikumar E.
Department of Cardiothoracic Surgery, Christian Medical College & Hospital, Vellore, South India.
J Cardiovasc Surg (Torino) 1999 Feb;40(1):101-5 Abstract quote
BACKGROUND: A retrospective study to evaluate the clinical symptoms, signs, investigations, surgical technique and follow-up of an uncommon cardiac tumour.
METHODS: Experimental design and setting: this retrospective study was carried out in a tertiary care hospital in South India on patients treated from 1981 to 1996. Duration of follow-up has been for a maximum period of 9.5 years. Participants: all patients who were diagnosed to have right atrial myxoma were included in this study. There were seven patients (4 males, 3 females) with ages ranging from 16 to 52 years. Interventions: six of these patients underwent complete excision. One patient who presented with SVC obstruction had locally invasive tumour and only a biopsy was done. In order to reduce the risk of intraoperative pulmonary embolism in the last two cases, we crossclamped the pulmonary artery as soon as cardiopulmonary bypass was initiated.
RESULTS: There was one postoperative death due to refractory right ventricular failure (14.3%). Four patients have been followed-up for a mean of 40.7 months. All of them are free of recurrence and most of them are asymptomatic.
CONCLUSIONS: Right atrial myxoma is a rare usually benign cardiac tumor whose definitive diagnosis is made on the basis of 2D echocardiography. The crucial aspects of surgery are measures for prevention of intraoperative embolism, en-bloc excision of the tumour with a wide cuff of normal tissue and inspection of all four chambers in order to avoid missing tumour emboli or an occasional multicentric lesion. Patients with myxomatous emboli into the pulmonary artery require simultaneous pulmonary embolectomy.
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