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Ann Thorac Surg 1998;66:285-288
© 1998 The Society of Thoracic Surgeons

---

Collective Reviews

Recurrent cardiac myxoma: seeding or multifocal disease?

^a Department of Cardiac Surgery, Oxford Heart Centre, John Radcliffe Hospital, Oxford, England, UK

Address reprint requests to Dr Westaby, Department of Cardiac Surgery, Oxford Heart Centre, John Radcliffe Hospital, Headley Way, Headington, Oxford OX3 9DU, England

	Abstract

Top Abstract Introduction Case report Literature review Comment Acknowledgments References

 
We describe a patient with left atrial myxoma followed by a second lesion in the left ventricle. Previous reports of "recurrent" myxoma were studied, with the following conclusions: There is little evidence for the process of myxoma seeding, local recurrence can occur through inadequate resection or malignant change, and many alleged recurrences are at a different site and multifocal disease occurs frequently in the familial setting.

	Introduction

Top Abstract Introduction Case report Literature review Comment Acknowledgments References

 
Recurrence of benign tumors is unusual. Myxoma is the most common tumor of the heart, and though benign, recurrence has been observed in 3% of patients [1–5].

In this report we describe a case of left ventricular myxoma after resection of a left atrial myxoma. The debate regarding tumor recurrence or multifocal disease is addressed by literature review.

	Case report

Top Abstract Introduction Case report Literature review Comment Acknowledgments References

 
The 63-year-old woman first presented with a left femoral embolus. Femoral embolectomy was performed and myxomatous tissue identified. Two-dimensional echocardiography showed a left atrial mass attached to the interatrial septum. At operation, through a biatrial approach, a sessile 3-cm myxoma was excised from the interatrial septum. Full-thickness resection of the fossa ovalis was performed at the point of attachment. Inspection of the left atrium and left ventricle revealed no additional tumors. Postoperative recovery was uneventful and echocardiography revealed no residual tumor. Histologically, the benign character of the myxoma was confirmed.

Three years later, the patient suddenly became aware of right-side weakness, which suggested a further embolic event. On this occasion the transesophageal echocardiogram revealed a small sessile mass (1.5 cm diameter) at the base of the medial papillary muscle of the mitral valve. The left atrium was normal. At reoperation, left ventriculotomy was performed, and the myxoma was excised. The myocardium at the point of attachment was cauterized. Postoperative recovery was uncomplicated. Histologically, the ventricular tumor was a typical benign myxoma and indistinguishable from the previous lesion.

	Literature review

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The frequency of atrial myxoma is estimated at between 0.5 and 1 per million of population per year [6, 7], with a recurrence rate of 2% to 3%. We discovered only two previous reports of left ventricular myxoma after a left atrial myxoma [8, 9]. In the English-language literature we found 42 cases of first recurrence from a total of 584 patients, but very few cases (1.3%) of repeated recurrence . Table 1The age range at the time of the first surgical resection was 7 to 62 years, with a mean of 32.7 years. Reoperation was performed at between 3 months and 14 years afterwards, with an average of 3.9 years.

The recurrent myxoma can be divided into four groups: inadequate resection, totipotent multicentricity, familial type, and metastatic recurrence.

Several reports suggest the possibility of inadequate resection [10–13]. Gerbode and associates [14] first described recurrence of left atrial myxoma 4 years after initial excision. They postulated incomplete removal at the first operation and recommended wide resection of the atrial septum around the base or stalk of the tumor. Li and colleagues [9] reported a case of recurrent deep-seated left ventricular myxoma, removed through an aortotomy with video-assisted cardioscopy. The first operation had been 2 years previously, the tumor originating from a similar site in the interventricular septum. Read and colleagues [15] then reported recurrence after excision of the septum in the manner suggested. The second benign myxoma arose from the atrial free wall, not at the site of the first myxoma.

Some authors report recurrence and totipotent multicentricity. Jugdutt and associates [16] reported a patient with a left atrial recurrence at 30 months and stressed that five of nine recurrences recorded in the literature had occurred despite wide resection and patch replacement of the atrial septum. When this patient died 4 years later there were no fewer than six separate myxomas in the left atrium, one of which extended through the septum to the right atrium. There are three further reports of a myxoma recurring for a second time [10, 15, 17]. In one, O’Neil and colleagues [17] reported a patient in whom a second myxoma developed on the annulus of the tricuspid valve 4 years after a left atrial myxoma was removed; a third tumor appeared in the right atrium 8 years later. O’Neil and colleagues emphasized that each recurrence was "upstream" from the previous one and could not therefore be caused by seeding from an earlier lesion.

This pattern suggests multifocal disease rather than inadequate resection or the unlikely scenario of seeding in the presence of high-velocity blood flow. Other recurrences that suggest multifocal disease were reported by Gray and Williams [10], Nakata and colleagues [11], Kabbani and associates [12], and Castells and coworkers [13].

Recently, Bjessmo and Ivert [18], in a series of 63 patients, described a 28-year-old female patient in whom suspected myxomatous tissue was cauterized close to the pulmonary veins during resection of a septal myxoma. Echocardiography showed recurrence at this site 2 years after the primary operation, and she underwent an uneventful second operation.

Myxoma occurs with a family history in about 5% of cases [19]. Myxomas with a normal DNA ploidy are unusual [10, 14, 19]. According to McCarthy and associates [20], the risk of tumor recurrence is much higher in the familial type (10%), in the presence of multiple myxomas (33%), and in the syndrome of complex myxoma (21%). Up to 40% of patients experience recurrence when their tumor exhibits an abnormal DNA ploidy pattern. In 1996 Singh and Lansing [21] reported a family in which three members had cardiac myxoma, and two had other characteristics of the Carney complex, the syndrome in which tumors are multicentric in affected organs and bilateral in paired organs. The disorder is transmitted as an autosomal dominant trait with mesenchymal tumors (myxomas), pigmented skin lesions, functioning endocrine tumors, and peripheral nerve tumors [22, 23]. Myxomas develop in the skin of the breast and in the heart. In a review of 26 familial myxoma families (including 68 individuals), Singh and Lansing [21] found 17 episodes of recurrence (25%), at the same or different intracardiac sites. Farah [24] provided similar information for patients with familial multiple myxomas followed up for a mean of 8.1 years after resection of the first lesion. The earliest recurrence presented 4 years after the first tumor resection. Patients with familial cardiac myxoma are likely to be female and younger than in sporadic cases, with multicentric origins [19, 23–26].

The potential for malignant change in myxoma is debatable. We [2] have previously reported same-site recurrence with more aggressive histology, then second recurrence with multiple smaller myxomas elsewhere in the left atrium. Although the first lesion was typically benign, the second recurrence was frankly malignant, and the patient died with extensive left atrial sarcoma. Reynen [27], in an extensive review, also suggests transformation from a benign to a malignant lesion as an explanation for recurrence, although histologic proof supports this suggestion only rarely. Nevertheless 3 cases have been described in which a cardiac myxoma presented with an intracranial mass [28–30]. There remains the potential for misdiagnosis on the basis of histopathology.

Goldstein and colleagues [31] described cardiac transplantation for recurrent myxoma in a 47-year-old woman. At the first operation, two left atrial tumors were excised and reported as benign myxoma. Three months later, echocardiography showed a recurrent tumor coating the wall of the left atrium and mitral annulus, extending to the chordal apparatus. After cardiac transplantation, the pathologic findings were summarized as fibroma of uncertain malignant potential. There is a further case in which a mesenchymal sarcoma was misinterpreted histologically [6]. Burke and Viramani [32] have recently commented that most, if not all, "malignant myxomas" were misdiagnosed cardiac sarcomas, and most pathologists lack experience with cardiac tumors.

	Comment

Top Abstract Introduction Case report Literature review Comment Acknowledgments References

 
The concept of intracardiac tumor seeding of myxoma through deposition of detached fragments carried by a high-velocity bloodstream is untenable, and there is little evidence to support this concept. Myxomas seldom seed in peripheral vascular beds and even highly aggressive malignant tumor cells fail to deposit in cardiac cavities.

Whereas same-site recurrence may occur as a result of inadequate resection, this is probably rare through awareness of the problem and reliable operative methods. Although we [2] have previously provided evidence for a progressive change in the histologic nature of a tumor from myxoma to sarcoma, this appears to be a very rare occurrence. Many second myxomas arise at a different site as part of multifocal disease that may have a genetic predisposition. A family history should be sought for all patients with myxoma and the risk of further problems considered, particularly in younger women.

	Acknowledgments

Top Abstract Introduction Case report Literature review Comment Acknowledgments References

 
We thank Miss Katherine L. Ely for editorial assistance.

	References

Top Abstract Introduction Case report Literature review Comment Acknowledgments References

 

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