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A 68-year-old woman, developed subsequent recurrent hematomas of the left occipital lobe about 1 year after open-heart surgery for the left atrial myxoma. Radiological studies revealed multiple intracranial aneurysms with hemorrhage. Microscopic examination showed the presence of myxoma invasion of the vascular wall with aneurysmal formation and organized hematoma.

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We describe a woman who had a total resection of a cardiac myxoma followed 8 months later by a hemorrhage in the right frontal lobe secondary to extravascular metastasis of the myxoma. Six years later, after an asymptomatic follow-up, she developed a recurrence of left-sided seizures and an enhancing mass in the same location as the previous tumor. At operation, a malignant astrocytoma was demonstrated. Cardiac myxoma is a true neoplasm with benign histology, which may be associated with heart failure, systemic illness, or peripheral embolization. The neurological manifestations of embolization may include no symptoms, acute or delayed infarction, and intravascular proliferation with aneurysmal dilatation and potential for hemorrhage. The development of extravascular metastatic tumor deposits has been reported previously in only three histologically verified cases. Once the integrity of the blood vessel wall is destroyed by the tumor, a portal of entry is established for tumor cell proliferation in the brain parenchyma. There is no known association between a metastatic cardiac myxoma and a malignant glioma in the literature. Several possibilities for the occurrence of these two neoplasms are discussed.

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A rare case of bona fide cerebral metastasis of malignant cardiac myxoma is presented. The parenchymal brain tumor of a 32-year-old woman with a history of surgical operations on cardiac myxoma and jejunal leiomyosarcoma was surgically extirpated. The initial pathological diagnosis was metastatic sarcoma. Ten months later, an occlusion of bilateral carotid arteries occurred that was due to tumor emboli. The postmortem examination revealed an unusually rapid recurrence of the cardiac tumor containing sarcomatous components that were histologically identical to the tumors of the brain and the jejunum and the emboli of the cerebral vessels. Hence it was deduced that they all originated in the primary malignant cardiac myxoma.

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A 25-year-old male underwent right mastoid surgery for removal of a soft tissue mass. At age 29 a left cardiac atrial myxoma was excised. At age 34 an expanding right supra-auricular mass with extension into the middle and posterior fossa necessitated total temporal bone resection. Pathology proved to be myxoma. Retrospective review of tissue obtained from the initial mastoid procedure was also myxoma. The temporal bone lesion was considered to be persistent and metastatic atrial myxoma. A review of atrial myxomas and this most unusual metastatic presentation is discussed.

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Cardiologists rarely encounter tumoral pathology, the commonest being the myxoma which presents more often as cardiac rather than neoplastic disease. A few cases of "malignant or metastatic cardiac myxoma" have been reported in the literature. We have not observed this behaviour in the follow-up of 100 cases of myxoma between 1959 and 1989. However, during the same period, 14 intracardiac sarcomas (operated or not) were seen, which were all rapidly fatal. A critical review of the cases of metastatic cardiac myxomas reported in the literature enabled us to classify them into three groups: the first, 9 cases of false myxomas. They were cardiac sarcomas and therefore a histopathological diagnostic error had been made. The second group comprised false metastases. They concerned arterial embolism of fragments of myxoma causing ischemic infarction or infiltration of the arterial wall by the myxoma, damaging the artery and resulting in fusiform aneurysm. This cannot be accepted as a true metastasis: a number of examples are given as they are too many to be named individually. The cardiac and cutaneous myxomas observed in Carney's complex may be included in this category. The third group has to be discussed case by case: these "metastatic myxomas" can all be explained logically and malignancy can be excluded; they are few in number (5 cases!) but often published several times by different authors. In our opinion, a carcinologically "malignant" metastatic myxoma remains a questionable pathological entity. This does not mean that a myxoma cannot be lethal: its intracardiac position may cause fatal valvular obstruction, or it may give rise to fatal embolism.(ABSTRACT TRUNCATED AT 250 WORDS)

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We have presented a 23-year follow-up of a patient with left atrial myxoma with continuing slowly growing, skeletal metastases. The relatively indolent nature of the metastatic disease and the lack of mitotic figures suggest that the metastatic lesions may be treated locally. The patient underwent therapeutic tumor embolization and local resection of the pelvic lesion, with good results as she continues to do well with close follow-up.

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The first case of 'a metastatic intraluminal myxoma of the aorta' is reported. The patient was a 48-year-old man who had already developed metastases to his skin and brain from a left atrial myxoma. Then, his myxoma grew in his leg and intraluminally in the aorta and caused renovascular hypertension. Surgical removal of the tumor could relieve his hypertension, but he finally died 6 months after surgery. The findings at autopsy were described and previous reports on metastasizing left atrial myxomas were also reviewed.

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A 55-year-old man, who had previously had a left atrial myxoma excised, developed recurrent hematomas of the left occipital lobe. Microscopic examination revealed the presence of metastatic myxoma.

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A 25 year old woman developed 2 subcutaneous myxomas one month after removal of an atrial myxoma. The pathogenesis of this event is discussed. Special attention was given to distinction between true myxomas and myxoid sarcomas as well as to myxomatous diathesis, caused by a widespread, genetically determined specific abnormality of certain mesenchymal cells (Swiss-syndrome). With all aspects considered, this appears to be the best interpretation of the case under review.

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We have reported a case of pseudomyxoma peritonei manifested as a splenic mass in a 38-year-old woman. Upon reviewing previously reported cases of pseudomyxoma peritonei with visceral involvement or extension above the diaphragm, we conclude that such spread of the disease does not significantly alter the prognosis. Furthermore, our findings support the concept that pseudomyxoma peritonei represents the implantation of malignant cells rather than metaplastic transformation of mesothelial cells.

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This paper presents a case of a cardiac myxoma with cutaneous emboli. The diagnosis of a cardiac lesion was anticipated after the histologic examination of a skin lesion. The first clue to the existence of a cardiac myxoma was a distinctive intravascular lesion of a dermal vessel. The clinical and pathologic features of cardiac myxoma are discussed. In addition, new evidence regarding the nature of a previously reported case of metastasizing cardiac myxoma is also presented.

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Two metastases from an atrial myxoma occurred in the right common carotid artery distribution of a 34-year-old man. One metastasis caused the phenomenon of intracranial pseudoaneurysm in a distal branch of the middle cerebral artery. The other resulted in the unusual radiographic appearance of a tremendous, "bubbly" expansion of the temporal bone.

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A 73-year-old man had myocardial infarct and coronary emboli from a left atrial myxoma diagnosed at necropsy. The tumor was attached to the atrial roof and showed no local myocardial infiltration. Transmission electron microscopic (TEM), light microscopic, and immunoperoxidase (IPX) studies confirmed the neoplastic character of this lesion and pointed to undifferentiated mesenchymal cells as the origin of the atrial myxoma cells. This case emphasizes two aspects: (1) glandlike structures were also found in the myxoma, and their epithelial-like nature was supported by TEM and IPX studies, which showed positivity for carcinoembryonic antigen and H blood substance; (2) many systemic tumor masses were found, and their metastatic nature was evidenced by the markedly infiltrative and destructive character; the only cytologic marker that could discriminate this case from other usual, noninfiltrative cardiac myxomas was the epithelial-like cells.

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The clinical experience with cardiac myxomas and "myxoid imitators" at the Humana Heart Institute International is reviewed and contrasted with a comprehensive review of the world literature. Twenty-two patients with primary cardiac tumors have been treated at our institution. Three (13.6%) of them had myxoid imitators, and 19 had classic myxomas. One of the 19 patients with a classic myxoma had recurrence on two occasions (recurrence rate, 5.3%). The cases of 57 patients from the world literature are reviewed. Recurrence and/or metastases (including formation of mycotic aneurysm) occurred in approximately 50% of these patients. The overall mortality in these patients was 47%. This review provides documentation of the malignant clinical behavior of cardiac myxomas and myxoid imitators. Neither local recurrence nor regrowth of the tumor in a new location, whether intracardiac or extracardiac, can be predicted from the microscopic appearance of the primary tumor. Resection of the myxoma with a wide margin is the recommended surgical procedure but does not ensure a cure. Therefore, long-term follow-up is mandatory.

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A 56 year old woman developed multiple metastases in the cerebrum and cerebellum, four years after cardiac intervention on a left atrial myxoma. The absence of stroke is noteworthy. Multiple high density lesions with contrast enhancement were seen by CT scan, suggesting metastatic neoplasms. Histological examination confirmed the diagnosis of metastases of cardiac myxoma. Only four cases were recorded in the literature.

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