Familial Myxomas Research Articles

Surgical experience with intracardiac myxomas: long-term follow-up

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.

Multiple Local Recurrent Myxoma.

Cardiac myxomas, usually considered benign tumors, occasionally pursue an aggressive clinical course. The recurrence of sporadic myxomas is much less frequent than that of complex or familial myxomas (1-3% vs. 22% vs. 12%, respectively). We report a case of sporadic myxoma with multiple local recurrence in the left atrium diagnosed by transesophageal two-dimensional echocardiography 4 years after the first excision.

Cardiac myxomas: Clinical and echocardiographic profile

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.

Transesophageal echocardiography in the evaluation of cardiac myxoma: A case of familial myxoma

The vast majority of atrial myxomas are readily apparent on standard transthoracic echocardiography. We present the case of a young woman with known history of familial myxoma, in whom a large left atrial mass was identified on transthoracic echocardiography. Transesophageal study more clearly delineated the extent and origin of the tumor. In addition, an unsuspected tumor in the right ventricle was also identified. These findings were confirmed at surgery, and both masses were excised. The advantages of transesophageal echocardiography in evaluation and management of this unusual subset of patients with cardiac tumor are discussed.

Cardiac Myxoma:A Clinicopathologic Study

The clinicopathologic features of 107 cardiac myxomas are presented. Eighty-one were in the left atrium, 18 in the right atrium, and 8 were biatrial or located in the ventricles. The mean age at presentation was 50 years and did not vary significantly by sex, race, or site in the left or right atrium. Histologically, 41% of tumors had surface thrombus; 41%, fibrosis; 23%, mitotic activity; 20%, calcification; 17%, gamma bodies; 8%, ossification; 7%, extramedullary hematopoiesis; 3%, mucin-forming glands; 3%, atypical cells simulating malignancy; and 1% had thymic rests. A majority of myxomas was immunohistochemically positive for QBEnd throughout the lesion, but in most tumors, factor-VIII related antigen was limited to the surface cells. A minority of myxomas stained for antismooth muscle actin and Ulex europaeus; KP-1 and cytokeratin were always negative. Right atrial tumors were more likely calcified than those in the left atrium. Embolic tumors were less often fibrotic than nonembolic myxomas and were more likely thrombosed and extensively myxoid with an irregular frond-like surface. Fibrotic and non-thrombosed tumors had a longer mean duration of clinical symptoms and were found in older persons. Recurrent, multiple, and familial myxomas were more often found in younger women and, more likely irregular surfaced and histologically myxoid; however, they were not likely to be histologically atypical, cellular, or incompletely excised. Five tumors caused the death of the patient by emboli to the brain or heart, but none metastasized. The histology of myxomas varied by site, clinical presentation, patient age, and immunohistochemical findings.

Atrial myxoma: a tumour in search of its origins.

To determine whether atrial myxomas express antigens suggesting a neural origin. A retrospective analysis based on immunohistochemical examination of myxoma tissue. Atrial myxomas excised by two tertiary referral cardiothoracic surgical units. 24 excised atrial myxomas. Three were from known cases of familial myxoma syndrome. Immunohistochemical identifications of three neuroendocrine markers (protein gene product (PGP) 9.5, neurone specific enolase (NSE), synaptophysin) and S100 antigen; CD34 and von Willebrand factor; and chi smooth muscle actin to identify possible Schwann cell differentiation, endothelial cells, and smooth muscle cells respectively. The myxoma cells were PGP 9.5 positive in 18, S100 positive in 16, and NSE positive in 12. Of the 12 NSE positive myxomas seven were synaptophysin positive. All tumours that were NSE positive were also S100 and PGP 9.5 positive. The tumour surface was partially covered by myxoma cells, partly by endothelial cells. The histological appearances of myxomas with stellate cells embedded within a loose connective tissue stroma, abundant basophil cell infiltration, and the presence of pericellular type IV collagen are similar to nerve sheath tumours (neurofibromas) at other sites. A significant proportion of myxomas also express Schwann cell and neuroendocrine differentiation markers. These features cannot prove the origin of myxomas because tumours may develop aberrant phenotype expression but they do accord with the view that myxomas originate from endocardial sensory nerve tissue.

Familial myxomas in four siblings

Familial myxomas of the heart are very rare, with only two previous reports noted in the literature. This report documents four siblings with myxomas, and three of these patients had two or more myxomas. One sibling had four myxomas excised. The clinical findings, history, examination, and surgical treatment are described.