Cerebral metastasis of malignant cardiac myxoma.

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.     

A case of cardiac myxoma with multiple brain hemorrhage]

A case of cardiac myxoma with multiple brain hemorrhage is reported. A 57-year-old male had complained of lower abdominal pain, diarrhea and fever for 3 days. On admission, he was in a condition of disseminated intravascular coagulation and sepsis. An abdominal CT scan showed infarction in the right kidney and spleen and an echocardiogram also showed myxoma in the left atrium. Although he presented no neurological symptoms, the brain CT showed multiple brain hemorrhage in the bilateral brain hemispheres. Total resection of the tumor was carried out for the improvement of the patient's general condition. Vimentin, S-100 protein and neuron specific enolase was positive in immunological staining and the pathological diagnosis was myxoma. Postoperative recovery of consciousness was poor and left hemiparesis developed. CT showed the increase of hematoma but angiography showed no cerebral aneurysm. The symptoms improved with conservative therapy. However the enhanced lesion remained in the right parietal lobe and an operation was performed 5 months later. The myxoma cell could not be found in the pathological examination, so tumor embolism, cerebral infarction, hemorrhagic infarction due to DIC, hematoma enlargement caused by heparinization during operation were suspected to have occurred in this order without tumor growth.     

Surgical treatment of intracardiac tumors in 25 patients

For most intracardiac tumors, operation is the only means of therapy. In our institute, we have aggressively performed operation for intracardiac tumors regardless of histological type because resection for tumor had a beneficial effect on the hemodynamics with congestive heart failure. Twenty-five cases of cardiac tumors were operated upon from 1980 through 1998. The follow-up period ranged from 2 months to 19 years. The histological diagnoses of the tumors were as follows: benign tumors 24 (myxoma 21, papillary fibroelastoma 1, fibroma 1, angiomyolipoma 1) and malignant tumor (angiosarcoma 1). There was one hospital death in this series. In the New York Heart Association classification, the cardiac performances of intracardiac benign tumors after operation were Class I or II. The results of surgical treatment of intracardiac benign tumors were satisfactory both in short-term and in long-term. On the other hand the long term result of malignant tumor was extremely poor. A patient with angiosarcoma died 8 months later due to bone metastasis.     

Surgical treatment of primary cardiac tumors]

Twenty patients with primary cardiac tumors were operated on during the past ten years. The age of 15 female and 5 male patients ranged from 17 to 73 years. Eighteen patients had myxomas, 16 of which located in the left atrium and 2 in the right atrium. Systemic embolism occurred in 8 patients, subsequently caused cerebral infarction in 4, ischemia of extremities in 2, myocardial infarction in 1 and pulmonary infarction in 1. Emergency operation was performed in 5 patients because of severe congestive heart failure. In all cases, removal of myxoma was performed together with the excision of the wall to which the pedicle attached with the use of cardiopulmonary bypass. One patient with pulmonary infarction underwent resection of the infarcted lung simultaneously. Only one patient with severe heart failure died of pulmonary insufficiency one month after the operation. Another patient with cerebral infarction underwent clipping of cerebral aneurysm which appeared later in the infarcted area. The 17 patients including the latter patient showed a good recovery and no local recurrence during the follow-up period of 1 to 120 months. Two patients had malignant tumors, which were malignant fibrous histiocytoma of the left atrium and leiomyosarcoma of the pulmonary artery, respectively. Both of these rare tumors were resected noncuratively and led to the death because of their local recurrence with distant metastasis, though they received adjuvant chemotherapy. The symptoms, complications, diagnoses, surgical treatment and outcome of the primary cardiac tumors are reviewed in this study.     

Giant cardiac metastasis of myxoid liposarcoma causing cardiac tamponade; report of a case

We report a very rare case of cardiac metastasis of myxoid liposarcoma. A 55-year-old man presented with dyspnea. Two and a half years ago, he underwent resection of myxoid liposarcoma in the left thigh. Magnetic resonance imaging (MRI) revealed a giant tumor occupying the pericardiac cavity and pressing the heart and consequently causing cardiac tamponade. The patient underwent surgery through a left thoracotomy approach. The pericardiac cavity was filled with a giant tumor with a stalk from the right ventricle and 2 small nodules on the main pulmonary artery. He was relieved from the symptom: however, he had a recurrence of the tumor at the same site 5 months after the operation. He underwent surgery for the removal of the second tumor; however, he died 49 days after the operation. Although cardiac metastasis is a very rare condition, its awareness is essential for careful long-term follow-up for the early detection of a metastatic cardiac liposarcoma after the resection of the primary tumor.     

Multiple intracranial aneurysms as delayed complications of an atrial myxoma: case report.

OBJECTIVE AND IMPORTANCE: Atrial myxomas are rare cardiac tumors that may cause neurological complications; however, delayed neurological events after total tumor resection are rare. In this report, we present a patient who developed transient cerebral ischemic attacks and was found to have multiple intracranial aneurysms 5 years after successful resection of her atrial myxoma. At the time of myxoma resection, there were no neurological symptoms; at the time of presentation with transient ischemic attacks, there was no evidence of atrial recurrence. CLINICAL PRESENTATION: A 32-year-old woman presented with five episodes of right arm and face paresthesia, each lasting 15 to 20 minutes, 5 years after successful resection of her atrial myxoma. Clopidogrel bisulfate therapy was initiated, with resolution of her symptoms. Angiography revealed multiple, peripherally located, fusiform cerebral aneurysms. INTERVENTION: A left frontal craniotomy for resection and biopsy of one of the aneurysms was performed, to establish the diagnosis. Pathological analysis of the biopsied aneurysm provided evidence of direct atrial myxoma invasion and occlusion of the cerebral blood vessel. CONCLUSION: Neurological symptoms may accompany or lead to the diagnosis of atrial myxoma. Rarely, as in this case, myxomatous aneurysms may develop years after definitive treatment of the primary tumor. Patients who have undergone successful resection of a left atrial myxoma may be at risk for delayed cerebral ischemia associated with aneurysm development, and this phenomenon must be considered for patients with neurological symptoms who present even years after myxoma removal. The natural history, pathophysiological features, and treatment dilemma of these aneurysms are discussed.     

28例心脏肿瘤患者的临床特点和外科治疗

目的 总结心脏肿瘤的临床特点和外科治疗结果.方法 回顾性分析2013～ 2019年我中心收治的28例诊断为心脏肿瘤患者的临床资料,其中男12例、女16例,年龄1个月至69岁,中位年龄11.5岁,体重39.0 (4.8～ 100.0)kg.患者均在全身麻醉体外循环下行肿瘤切除术,术中切除肿瘤标本行病理学检查.结果 原发性...     

Diagnosis and treatment of 52 cases of cardiac myxoma

43 patients with cardiac myxoma had undergone surgical removal of the tumors under CPB 38 tumors were located in the left atrium, 5 in right atrium, and 1 in right ventricle respectively. The sites of attachment of the tumor stalk observed during operation were as follows: 34 left atrial myxomas were attached to the interatrial septum and 4 left atrial myxomas to the atrial wall. 5 right atrial myxomas were attached to the interatrial septum and 1 right ventricular myxoma to the ventricular wall. 1 patient with a right ventricular myxoma which had been removed one year ago appeared new one in the left atrium and the tumor was cut too. The diagnosis of cardiac myxoma was confirmed by 2D-echo-cardiography in all of our cases. Mitral valve replacement was done in 1 patient in this series. There was one death postoperatively. Besides these 43 cases, there were 9 patients who had not been operated upon, because of refusal of operation in 5, sudden death from cardiac arrest before operation in 2, coma from cerebral thrombosis in 1, and serious pulmonary tuberculosis in another one respectively.     

Second recurrence of cardiac myxoma 7 years after the initial operation

We report a rare case of a 23-year-old woman in who recurrent multiple cardiac myxomas were resected first time 4 years after the initial operation and second time three years after the second operation. In February 2000, she was diagnosed with right ventricular myxoma. The initial cardiac tumor was successfully resected through a right atrium approach and the tricuspid valve was changed with a biological prosthesis; the patient has been followed-up by ultrasound echocardiography every 6-month after discharge. Four years after, the echocardiography revealed one masse in the left atrium, which was resected using a trans-septal approach. Due to malfunction of the tricuspid prosthesis, this was changed with a biological one. After another three years, the echocardiography revealed once again two masses, this time in the right atrium and left ventricle. The third surgery was performed and all 2 myxomas were successfully resected through a combined right atrial and left ventricle approach. One year after the third operation, she has been doing well without any sign of recurrence of myxoma. A long-term follow-up is mandatory in patients after the resection of a cardiac myxoma.     

A case of biatrial multiple myxomas with glandular structure.

A 45-year-old male, who had been indicated by brain magnetic resonance imaging to have cerebral infarctions, was found by echocardiography to have a tumor in the left atrium. He had experienced several of the constitutional disturbances associated with myxoma. At the ages of 19 and 35 he had had two episodes associated with embolisms, and at the later one he was diagnosed as having multiple cerebral aneurysms. He received an urgent operation in which three left atrial tumors and one right atrial tumor were resected. Histologically, the tumors were myxomas, and the left atrial main tumor had glandular structure. In view of his clinical history, this patient seems to have had cardiac myxomas for a long period. The multiple growths that occurred in this case may be a good argument for allowing this condition to last for so long. To our knowledge, the present case was the first report of cardiac myxoma with glandular structure in Japan.     

Malignant astrocytoma six years after the resection of a cerebral metastatic cardiac myxoma: case report.

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.     

Primary cardiac tumors.

Cardiac tumors are a rare, but potentially curably form of heart disease. A high index of clinical suspicion is necessary for diagnosis as these tumors have protean manifestations that mimic a variety of other cardiac and noncardiac diseases. Presently, M-mode and two-dimensional echocardiography are utilized as safe, reliable, and noninvasive imaging modalities. Seventy-five per cent of these tumors are benign, with myxoma accounting for 50% and rhabodomyoma comprising 20% of lesions. Various histologic types of sarcoma are the predominant malignant cardiac neoplasms. With strict attention to avoiding perioperative tumor embolization, surgical resection of these lesions can be accomplished with minimal morbidity and mortality. Sixteen consecutive primary tumors of the heart have been surgically treated at Duke University Medical Center since 1966 with no perioperative deaths and no late recurrences.     

原发性心脏心包肿瘤及瘤样病变的外科治疗

为探讨原发性心脏心包肿瘤的发病率及其特征，并评估其治疗方法。病例均经胸部Ｘ线、超声心动图、ＣＴ、ＭＲＩ和心导管检查，并经病理学检查证实诊断。２４例行手术治疗病人中，良性肿瘤２０例术后存活至今，其中１５例术后随访５～１５年无复发；４例恶性肿瘤中３例行部分切除，１例仅做病理学检查，均于术后４个月内复发或转移死亡。作者认为，原发性心脏心包良性肿瘤采用手术治疗常可获得良好结果，恶性肿瘤早期手术结合放疗可延长病人生命。     

Familial cardiac myxoma with multiple and contralateral recurrence

This report described a familial recurrent cardiac myxoma involving mother and daughter. The mother, at 27 year of age, had developed recurrent multiple myxomas in both left and right atrium and right ventricle 4 years after surgical excision of left atrial myxoma. Excision was successful and remains well without signs of recurrence 9 years postoperatively. In an asymptomatic 13-year-old daughter, a recurrent left atrial myxoma was found 3 years after the excision of right atrial myxoma by echocardiographic follow-up at 6 month intervals. Excision of left atrial myxoma was performed and histology showed the essentially the same findings as primary myxoma without signs of malignancy. From an experience of this familial recurrent myxoma and a review of 38 cases of 17 familial cardiac myxoma, it is recommended that wide excision of tumor including surrounding tissues, thorough search for multiple heterotopic tumors at surgery, close postoperative echocardiographic follow-up for at least 5 years, and examination of skin and breast tumor, and endocrine disorder for "complex" myxoma.     

Left atrial myxoma with cerebral oncotic aneurysms with special reference to the importance of serial angiography

A case of atrial myxoma with cerebral oncotic aneurysms was presented. A 37 year-old woman was admitted complaining of right hemiparesis, and episodes of syncope. Neurological examination on admission revealed a right arm monoparesis, a right hemisensory disturbance, and a motor aphasia. Computed tomography (CT) demonstrated low density areas in the left fronto-parietal region, and multiple discrete enhancing high density spots scattered bilaterally in the parietal lobes. Cerebral angiography showed multiple fusiform peripheral aneurysms especially in the distribution of the bilateral middle cerebral arteries. Cerebral emboli from the cardiac source were suspected, and an echocardiography was performed, which disclosed a huge villous mass in the left atrium. The cardiac mass was resected uneventfully under cardiopulmonary bypass. It proved to be a left atrial myxoma. Postoperatively, her neurological deficit persisted. After the resection of the cardiac tumor, follow-up angiographies were undertaken twice. The second angiography performed 6 months later revealed spontaneous resolution or stabilization of most of the aneurysms detected before. On the other hand, newly-developed aneurysms were seen at the sites which had seemed normal previously on the first angiography. No remarkable changes were seen on the third one except the slight enlargement of one of the aneurysms in the middle cerebral territory. At 3 years she was neurologically stable. Because of the paucity of knowledge concerning the natural history of cerebral oncotic aneurysms and lack of definite treatment for them, long-term follow-up including serial angiography is mandatory after the resection of left atrial myxoma.     

原发性心脏恶性肿瘤的诊断与治疗

目的 探讨原发性心脏恶性肿瘤的诊断和治疗方法.方法 回顾性分析1980年6月至2008年5月收治的21例原发性心脏恶性肿瘤者的临床资料,并结合文献复习.结果 所有病例均行手术治疗,并经病理学检查确定诊断.6例因肿瘤无法切除仅行活检,10例行肿瘤根治切除术,5例行肿瘤局部切除.术后失访3例.除根治性切除术后2～5个月目前仍生存的3例外,其余病例均于术后4年内因病情加重、肿瘤复发和(或)转移死亡.结论 UCG、CT、MRI、三维CT、冠状动脉CT、心血管造影等检查对诊断和手术方法选择有帮助,确诊仍需依靠病理学检查.早期诊断、早期手术治疗、尽量切除肿瘤,术后结合放化疗能够延长病人生命.     

Stroke of a cardiac myxoma origin

Objective

The clinical features of cardiac myxoma stroke have not been sufficiently described. Debates remain concerning the options and timing of treatment and the clinical outcomes are unknown. This article aims to highlight the pertinent aspects of this rare condition.

Methods

Data source of the present study came from a comprehensive literature collection of cardiac myxoma stroke in PubMed, Google search engine and Highwire Press for the year range 2000-2014.

Results

Young adults, female predominance, single cerebral vessel (mostly the middle cerebral artery), multiple territory involvements and solitary left atrial myxoma constituted the outstanding characteristics of this patient setting. The most common affected cerebral vessel (the middle cerebral artery) and areas (the basal ganglion, cerebellum and parietal and temporal regions) corresponded well to the common manifestations of this patient setting, such as conscious alteration, ataxia, hemiparesis and hemiplegia, aphasia and dysarthria. Initial computed tomography scan carried a higher false negative rate for the diagnosis of cerebral infarction than magnetic resonance imaging did. A delayed surgical resection of cardiac myxoma was associated with an increased risk of potential consequences in particular otherwise arterial embolism. The mortality rate of this patient population was 15.3%.

Conclusion

Cardiac myxoma stroke is rare. Often does it affect young females. For an improved diagnostic accuracy, magnetic resonance imaging of the brain and echocardiography are imperative for young stroke patients in identifying the cerebral infarct and determining the stroke of a cardiac origin. Immediate thrombolytic therapy may completely resolve the cerebral stroke and improve the neurologic function of the patients. An early surgical resection of cardiac myxoma is recommended in patients with not large territory cerebral infarct.     

Multiple recurrence of cardiac myxoma in a Carney complex patient 4 years after the first operation

Carney complex is a rare syndrome which includes cardiac myxoma, hyperactive endocrine neoplasm, spotty pigmented skin, and extracardiac myxomatous tumors. We report a case of a 26-year-old woman with Carney complex in whom recurrent multiple cardiac myxomas were resected 4 years after the first operation for left atrial (LA) myxoma. She had a history of left adrenalectomy in 1997 for Cushing syndrome due to primary pigmented nodular adrenocortical disease (PPNAD). In February 2001, she was diagnosed with Carney complex because of evidence of LA myxoma, her spotty pigmented skin lesions, her past history and family history of cardiac myxoma in her mother. Then, LA myxoma was successfully resected through the superior trans-septal approach and has been followed-up by ultrasound cardiography (UCG) every 6-month after discharge.In January 2005, UCG revealed 2 masses in the LA and the right ventricle outflow tract. The 2nd surgery was performed in February 2005. We found the 3rd myxoma during surgery, resembling a flat polyp in the LA just at the inflow of the right upper pulmonary vein. All 3 myxomas were successfully resected. Sixteen months after the 2nd operation, she has been doing well without any sign of recurrence of myxoma.     

Concomitant surgery of a left atrial myxoma and a pulmonary carcinoma

We report the first successful outcome of a patient undergoing concomitant surgery for a left atrial myxoma and a squamous cell carcinoma of the lung. Preoperative evaluations revealed lung cancer and a left atrial tumor of undetermined origin. An operation to excise both cardiac and pulmonary tumors was performed. The cardiac tumor was first resected with the aid of cardiopulmonary bypass. The histopathological diagnosis was myxoma. Resection of the left upper pulmonary lobe and mediastinal lymph nodes followed. The postoperative course was uneventful, and the patient is well without evidence of recurrence 7 years after the operation.     

Urgent indications for surgery in primary or secondary cardiac neoplasm

Ten patients underwent resection of primary or secondary cardiac tumor. Two-dimensional transthoracic echocardiography per se accurately located the endoluminal cardiac mass in nine patients, and transesophageal echocardiography demonstrated a right atrial tumor in the tenth case. The indications for urgent surgery included prior embolic events (3 cases), syncopal attacks (2) or echocardiographic evidence of a multilobulated mass (2 cases). The operative strategy was standardized for atrial tumors, but for malignant myocardial neoplasm both the anatomic site and the extent of tumor growth determined the surgical procedure. Histologic examination showed myxoma in seven cases, fibroma in one and metastases of malignant melanoma in two cases. The course after resection of endoluminal benign tumor was uneventful apart from transient atrial fibrillation in four cases. Follow-up echocardiography (after 4-28 months) showed no recurrent growth. In both cases of intracardiac metastases there was recurrence within 6 to 8 months after resection of the growth.