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.     

Undifferentiated pleomorphic sarcoma in the left atrium; report of a case

Primary cardiac sarcomas are rare. A 41-year-old woman complaining of dyspnea was admitted to our hospital. Echocardiography and computed tomography (CT) showed a primary cardiac tumor in the left atrium. During surgery, the tumor was noted to be arising from the anterior wall of the left atrium, under the aortic sinuses. Histological and immunohistochemical studies revealed an undifferentiated pleomorphic sarcoma. Eleven months later, echocardiography and CT showed recurrence of the cardiac sarcoma in the left atrium. The patient underwent wide resection of the left atrium and mitral valve replacement because the tumor extended to the mitral valve leaflet. The patient died 3 months after the 2nd surgery because of the 2nd recurrence of the cardiac sarcoma. Although most tumors that develop in the left atrium are benign myxomas, preoperative differential diagnosis is important. It is especially necessary to suspect a sarcoma in the case of a non-septal orgin of the mass.     

A very rare cardiac hibernoma in the right atrium: a case report

Cardiac lipomas are rare tumors often detected incidentally during routine examinations. They usually remain asymptomatic for a long time and cause arrhythmia, heart valve dysfunction, or embolization in the later stages. In this article, we report a case of a 64-year-old patient with a very rare cardiac hibernoma located in the right atrium. Transesophageal echocardiography and computed tomography have been shown to be useful for differentiating between benign and malignant tumors in order to plan surgery. The treatment of choice for these tumors is resection. The tumor was excised with the use of cardiopulmonary bypass surgery. Histology confirmed diagnosis of a benign cardiac hibernoma.     

Surgical excision of a hemangioendothelioma of the left ventricle

Primary intracardiac neoplasms are most commonly histologically benign with only 30% exhibiting signs of malignancy. Metastatic tumors of the heart are 30 times more common than primary cardiac tumors. Patients with intracardiac masses may present with dyspnea on exertion, thromboembolic events, pericarditis, arrhythmias, or congestive heart failure. In many, however, the diagnosis is made upon discovering a murmur in an otherwise asymptomatic patient. Hemangioendotheliomas are very rare tumors of the heart characterized by capillary-sized vessels lined by rounded and often multilayered endothelial cells. Cardiac muscle cells are found compressed between proliferating vascular channels. We present a patient with this unusual cardiac tumor with no previous cardiac history and a new systolic murmur.     

Extracavitary lipoma of the heart. Operative resection

A rare benign primary tumor of the heart found in a 40-year-old woman is reported. Although extracardiac in nature, some of these tumors may involve a significant portion of the heart itself, and thus careful preoperative cardiac catheterization and angiographic study are indicated. While difficult to recognize preoperatively, extracavitary lipoma may be suspected in patients with unexplained cardiomegaly and otherwise essentially normal cardiac physical findings.     

Rare presentation of cardiac hemangiomas

Cardiac hemangiomas are exceptionally rare tumors with an incidence of 1% to 2% of all detected benign heart neoplasms. The clinical appearance of the tumor varies considerably and may mimic other pathological findings of definite heart structures. We report two cases of cardiac hemangiomas presenting with an unusual location and clinical course.     

Mitral valve fibroelastoma

Papillary fibroelastomas are rare benign tumors of the heart with predisposition for cardiac valvular involvement that were traditionally incidental findings at autopsy. Echocardiography now is allowing clinicians to diagnose these tumors in living patients. We reviewed the literature and, to our knowledge, are reporting the fifth documented case of a mitral valve papillary fibroelastoma in a living patient. Our patient was seen with bilateral transient ischemic attacks and was found to have a mitral valve tumor by two-dimensional echocardiography and cardiac catheterization. The tumor involved the entire mitral valve and subvalvular structures. The patient's valve was replaced with a bioprosthesis, and she remains free from symptoms.     

Clinical classification of cardiovascular tumors and tumor-like lesions, and its incidences

Tumors of the heart and the great vessels are very rare disease, and there are many disorders such as tumors originated from the heart and great vessels, metastatic tumors, and tumor-like lesions which do not fit into the usual concept of tumor or neoplasm; thus, it is very difficult to classify these tumors. We proposed a new classification of cardiovascular tumors for clinical use based on the accumulated biological analyses and clinical data of the reported literatures and our own study as benign tumors, malignant tumors, ectopic hyperplasia/ectopic tumors/others, and tumors of great vessels, with reference to the series of Atlas of tumor pathology of the Armed Forces Institute of Pathology and the recent World Health Organization classification of cardiac tumors issued in 2004. More than 50 disorders have been reported as tumors originated from the cardiovascular system, and various metastatic tumors from nearby organs, distant lesions, and intravascular extension tumors to the heart were reported. Based on the new classification, we reviewed epidemiology and incidence of cardiovascular tumors. Metastatic tumors are more frequent than tumors originated from the heart and great vessels, and cardiac myxoma is the most frequent tumors in all cardiac tumors.     

Unusual primary tumors of the heart

Primary tumors of the heart, with the exception of atrial myxomas, occur rarely; tumors metastatic to or directly invasive of the heart are far more common. About 75% of primary tumors are benign, and 75% of these are atrial myxomas. The benign tumors include rhabdomyomas, fibromas, papillary fibroelastomas, hemangiomas, pericardial cysts, lipomas, hamartomas, teratomas, mesotheliomas, and paragangliomas or pheochromocytomas. The last 3 may also be malignant. The malignant tumors consist of various sarcomas: myxosarcoma, liposarcoma, angiosarcoma, fibrosarcoma, leiomyosarcoma, osteosarcoma, synovial sarcoma, rhabdomyosarcoma, undifferentiated sarcoma, reticulum cell sarcoma, neurofibrosarcoma, and malignant fibrous histiocytoma. Cardiac tumors produce a large variety of symptoms through any of 4 mechanisms. Their mass can obstruct intracardiac blood flow or interfere with valve function. Local invasion can lead to arrhythmias or pericardial effusions with tamponade. Bits of tumor can embolize, causing systemic deficits when the tumors are on the left side of the heart. Finally, the tumors may cause systemic or constitutional symptoms. Some tumors, of course, produce no symptoms and become evident as incidental findings. The most useful diagnostic tool is the echocardiogram, which in almost all cases precisely locates the tumor and defines its extent. The echocardiographic appearance may also allow quite accurate prediction of the tumor type and whether it is malignant or benign. Magnetic resonance imaging serves as the next most important test where the density of T1 and T2 images may allow tumor cell type identification. With few exceptions, these tumors require operative excision. Most benign tumors can be resected completely; a few, because of their large size, cannot be, and only tumor debulking may be possible. Heart transplantation should be considered for these patients. Many of the malignant tumors cannot be resected completely, either because of the extent of local spread and invasion or because of the frequent distant metastases. Transplantation may also be an option for those with extensive local disease. The long-term results for resected benign tumors are excellent; the long-term results for sarcomas are very poor, and there are few survivors. For patients with unresectable sarcomas, radiation and chemotherapy may be used, but without great expectation of successful results.     

Mitral annular tumors: report of two cases in childhood

Primary tumors of the mitral valve are extremely rare especially in the pediatric age group; only a few cases have been reported previously. The clinical appearance of the tumor may mimic other pathological findings of heart structures or remain asymptomatic. We describe two different benign primary cardiac tumors, which were hemangioma and myxoma originating from the anterior annulus of the mitral valve and presenting with an unusual clinical course in two children. Both tumors were resected successfully with mitral valve conservation and there was no recurrence at 6-month and 1-year follow-ups.     

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.     

Left atrial sarcoma with the initial diagnosis of myxoma

Primary tumors of the heart are rare. The majority of these tumors are benign, with myxomas located in the left atrium being the most common form. Almost all malignant tumors are sarcomas and occur preferentially in the right side of the heart. The case of a 73-year-old woman who presented with symptoms of mitral valve stenosis is reported. Echocardiography revealed a left atrial mass that was interpreted as atrial myxoma. At the operation, the tumor which was lobulated and had a smooth surface was arising from posterior wall of left atrium extending into the mitral annulus. Wide surgical excision was possible with left atrial reconstruction and mitral valve replacement. Histological and immunohistochemical studies revealed malignant undifferentiated sarcoma extending to the surface of mitral valve. Postoperative metastatic work-up showed no evidence of dissemination. The patient survived the operation without adjuvant radiation and chemotherapy and has been symptom-free over 1 year. At present, 16 months following the operation, the patient is alive with no evidence of disease.     

Comprehensive review of the epidemiology and treatments for malignant adult cardiac tumors

Carcinomas rarely occur in the heart, and cardiac surgeons are generally not familiar with cardiac tumors. Some characteristics of cardiac and intrapericardial tumors are reviewed to understand the features of cardiac tumors. Cardiac tumors are discussed separately from intrapericardial tumors. Primary cardiac tumors are predominantly benign whereas primary intrapericardial tumors are usually malignant. The prevalence of each tumor types is presented in this review. In both cardiac and intrapericardial tumors, the incidences of metastatic tumors from cancers outside of the heart are high with carcinomas occupying more than half of the cases. Generally, the prognosis of primary malignant cardiac tumors is very poor. Cardiac tumors metastasize to other organs as hematogenous metastases. Surgery must be performed based on all the above-mentioned features of both cardiac and intrapericardial tumors.     

Rare case of aggressive angiomyxoma presenting as a retrovesical tumor

Aggressive angiomyxoma (AAM) is a rare mesenchymal benign tumor that preferentially involves the pelvic and perineal regions in relatively young females. We report here a rare case of AAM presenting as a retrovesical tumor in a male patient. A 59-year-old man undergoing abdominal ultrasound examination because of benign prostatic hyperplasia was found to have a retrovesical mass. Computed tomography and magnetic resonance imaging of the pelvis showed the retrovesical tumor to be 7.4 x 6.7 cm. The tumor was resected, and diagnosed histopathologically as AAM. The patient showed no recurrence 26 months after resection. Although the majority of retrovesical tumors are considered to be sarcoma or neurogenic tumor, AAM should also be recognized as a differential diagnosis.     

Recurrent cardiac intimal sarcoma misdiagnosed as a myxoma or malignant transformation of a cardiac myxoma?

Cardiac intimal sarcoma is extremely rare and aggressive primary malignant cardiac tumors. Here, we reported the case of a young man initially operated for a tumor of the left atrium, causing a dynamic obstruction of the mitral valve and (mis‐)diagnosed as a myxoma at the histopathological analysis. Patient presented a local recurrence at 3 months and was reoperated. Pathology revealed this time the presence of an intimal sarcoma. Patient received adjuvant chemotherapy. Despite a good local control, the 1‐year follow‐up positron emission tomography scan revealed the presence of a metastasis in the left adrenal gland that was surgically resected. This article aims to highlight the risk of misdiagnosis in case of cardiac tumors, the hypothetical concept of malignant transformation of a cardiac myxoma, the aggressive course of the extremely rare cardiac intimal sarcoma, and the therapeutic modalities available to treat this pathology.     

Papillary fibroelastoma of the left atrial wall: a case report

Cardiac papillary fibroelastoma is a rare, benign cardiac tumor. It often arises from valvular endocardium, and non-valvular endocardial location is rare. Although transthoracic echocardiography is usually sufficient for the diagnosis of most cardiac tumors, small tumors such as papillary fibroelastoma may be missed. Transesophageal echocardiography is superior to transthoracic echocardiography in diagnosing these tumors. Despite their benign histology, and independent of their size, they should be resected surgically because of their high potential for embolization.     

Recurrent primary cardiac osteosarcoma: a case report and literature review

Primary malignant cardiac tumors are very rare. Among malignant tumors, sarcomas occupy first place. In particular, primary cardiac osteosarcoma is extremely rare. To the best of our knowledge, only 42 cases have been reported worldwide. Cardiac malignant tumors usually require complex operations due to the difficulty in completely removing the tumor with acceptable free surgical margins and because of the proximity to vital structures. The current multimodality treatment strategies for cardiac sarcoma are still suboptimal, and surgery in particular frequently has unsatisfactory results. We report a case of recurrent primary cardiac osteosarcoma in a young male who underwent trans-sternal right pneumonectomy and a wide resection of the left and right atrium followed by reconstruction with heterologous pericardium under extracorporeal circulation. The patient died 6 months after the operation due to local and systemic disease recurrence.     

Papillary fibroelastoma of the interatrial septum: a case report

Primary cardiac tumors are rare, with benign lesions outnumbering malignant ones by a margin of over four to one. Amongst the benign cardiac neoplasms, papillary fibroelastomas are reported second only to myxomas. Papillary fibroelastomas commonly rise from heart valves and their size ranges from a few millimeters to well over 2.0 cm in diameter. While myxomas commonly arise from the interatrial septum, we present the rare example of a large papillary fibroelastoma arising from the left side of the interatrial septum.     

Cardiac rhabdomyomas with atrial septal defect and tricuspid insufficiency: A case report

Primary cardiac tumors are very rare and generally benign. The most common type, cardiac rhabdomyoma, comprises 45% to 75% of primary cardiac tumors. Cardiac rhabdomyoma is a rare benign tumor that commonly presents with tuberous sclerosis. We present a case of an infant with multifocal cardiac rhabdomyomas with an atrial septal defect and tricuspid insufficiency and no sign of tuberous sclerosis. She was successfully treated with an operation, the treatment plan included mass resection, tricuspid annuloplasty, and closure of the patent foramen ovale. The right atrial lesion was resected entirely, while the lobulated lesion in the right ventricle was resected as two pieces. There was no evidence of recurrence 1 year after the surgery.     

Myxoinflammatory Fibroblastic Sarcoma: A Case Report and Review of the Literature

The majority of soft tissue masses seen in the lower extremity are benign tumors caused by degenerative, reactive, or inflammatory processes. Sarcomas are relatively uncommon entities but need to be diagnosed and treated appropriately. We present a case of a myxoinflammatory fibroblastic sarcoma of the leg and review the literature on this topic. This is a rare tumor that predominately involves the distal extremities. It often presents as a painless mass within the subcutaneous tissue and can easily be confused with benign lesions. A high rate of local recurrence means patients must be followed up closely after resection of the tumor.