Primary cardiac Rhabdomyosarcoma

BACKGROUND: Primary tumors of the heart are rare even in major cardiac surgery centers. Because of the low case numbers, there is an insufficient evidence base to determine the optimal treatment, particularly for malignant tumors. METHOD: The authors review the pertinent literature retrieved by a selective PubMed search on the terms "cardiac tumor," "heart tumor," "cardiac myxoma," and "cardiac sarcoma." They also present operative techniques and their own long-term results in 181 patients with cardiac tumors...

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Primary cardiac tumors are rare. Nearly 25% of primary cardiac tumors are malignant, with rhabdomyosarcoma being the second most common primary sarcoma. Symptoms are variable, and the clinical presentation depends on the location and propagation of the tumor. Transthoracic and transesophageal echocardiography are preliminary tests in diagnosing the disease. Echocardiographic findings should be supported by other imaging methods. In appropriate cases, surgery combined with chemotherapy and radiotherapy is suggested...

Heart autotransplantation is an exceptional surgical technique used in the treatment of uncontrolled cardiac arrhythmias and primary unresectable cardiac tumors. We report a 28-year-old male with a rhabdomyosarcoma of the left ventricle, localized in the lateral and posterior wall, which involved the mitral valve and circumflex artery. After a complete study ruling out dissemination of the tumor, the patient was operated. Surgical exploration determined the unresectability of the tumor with the heart in situ...

The most common childhood genitourinary cancers are Wilms tumour, rhabdomyosarcoma and germ cell tumour (GCT). Long-term survival rates for patients with these tumours are generally excellent, ranging from 80% to 100%. However, the high cure rates have highlighted the need to minimize the long-term complications of treatments (referred to as 'late effects'), which can be caused by the three treatment modalities used to treat genitourinary tumours: surgery, chemotherapy and radiation therapy. Serious late effects, such as death, second cancers and tumour recurrence, are uncommon but do occur occasionally...

Primary cardiac tumours are extremely rare with the most commonest being left atrial myxomas. In general, surgical resection is indicated, whenever the tumour formation is mobile and embolization can be suspected. Within 17280 patients receiving heart surgery at the Innsbruck Medical University, 78 patients (0.45%) underwent tumourectomy of primary cardiac tumours. The majority of patients (63) suffered from a left or right atrial myxoma, 12 showed a papillary fibroelastoma of the valves at echocardiographical or histological examination, 1 suffered from a hemangioma, 1 from a chemodectoma, and another one from a rhabdomyosarcoma...

The aim of this study was to determine the histological spectrum of operated cardiac tumors, excluding myxoma, at a tertiary center in India. Between 1995 and 2010, we encountered 188 cases of operated cardiac tumors that had been subjected to histopathological examination. Morphological characterization was done by light microscopy along with histochemical stains. Immunohistochemistry using a panel of antibodies, i.e., vimentin, desmin, myogenin, smooth muscle actin (SMA), epithelial membrane antigen (EMA), cytokeratins, factor VIII-related antigen, S100-protein, synaptophysin, chromogranin, Bcl2, MIB-1, leukocyte common antigen (LCA), CD 3, CD20, CD34, and CD 99 (MIC-2) was performed wherever applicable...

Cardiovascular magnetic resonance imaging (CMRI) is the reference noninvasive imaging technique for assessment and characterization of a suspected cardiac or juxta-cardiac mass. The multiplanar assessment of anatomy, tissue composition, and functional impact afforded by CMRI allows for early differentiation between a nonneoplastic mass and a tumor mass, be it benign or malignant. Malignant cardiac tumors have a poor prognosis; however, early detection and characterization confer some survival advantage, enabling early instigation of chemotherapy and/or consideration of a surgical debulking procedure...

Primary heart tumors are uncommon in children. The majority of them are benign, with only 10% malignant. Among malignant cardiac tumors, sarcoma (rhabdomyosarcoma, angiosarcoma, synovial sarcoma) and lymphoma (Burkitt's lymphoma, large B-cell lymphoma, lymphoblastic lymphoma) predominate. There are few published pediatric series on malignant primary cardiac tumors. We report here 3 observations of primary malignant cardiac tumors, 2 cases of sarcoma (angiosarcoma and synovial sarcoma) and 1 case of Burkitt's lymphoma...

Cardiac magnetic resonance imaging (MRI) is an important tool for the diagnosis of cardiac masses. Various cardiac tumors are predisposed to occurring in atrial structures. The aim of this review article is the description of atrial tumors and their morphological features in MRI. In general, cardiac tumors are rare: approximately 0.001 - 0.03 % in autopsy studies. About 75 % of them are benign. The most common cardiac tumor is the myxoma. They are predisposed to occur in the atria and show a characteristically strong hyperintense signal on T 2-wieghted images in MRI...

The authors report a case of a 16-year-old man who presented progressive dyspnea. At that time the diagnosis of rheumatic fever with mitral valve involvement was performed. The bidimensional echocardiogram showed presence of mobile mass inside the left atrium. The tumor presented lobules, projecting into the left ventricle during the diastole and provoking turbulence. The patient underwent surgical resection with postoperative course needing re-operation for mitral valve replacement. Histopathology has proven that such tumor was a primary cardiac rhabdomyosarcoma and the early clinical diagnosis of rheumatic mitral valve disease was very difficult...

Rhabdomyosarcoma (RMS) is the most common soft-tissue tumor in childhood, but is extremely rare in elderly. We present a rare case of cardiac RMS, which developed 1 year after the diagnosis and management of acute lymphoblastic leukemia in a 68-year-old female. The occurrence of such phenomena is intriguing, especially in an individual without prior history of malignancy at a younger age. Through the review of the existing literature, we attempt to approach the pathogenesis and clinical manifestations of this rare clinical entity...

Primary cardiac sarcomas are rare instances and only occasionally documented in the cytologic literature. Usually, the diagnosis of these rare lesions can be made at echocardiography, aspiration biopsy cytology, cardiac biopsy, and open cardiac surgery (intraoperative diagnosis). In this study, cytologic configurations and immunohistochemistry for 3 primary cardiac sarcomas (rhabdomyosarcoma, angiosarcoma, and malignant fibrous histiocytoma) were revealed. In rhabdomyosarcoma (right ventricle), the tumor cells exhibited an anisocytotic spindle-shaped nuclei with hyperchromasia and an obscure cytoplasmic margin...

We present a case report of a 48-year-old man with a huge left atrial rhabdomyosarcoma who presented as severe mitral stenosis and accepted emergency surgery. Two years later, a pathologic fracture revealed osseous metastasis, and intracardiac recurrence was detected by echocardiography. The patient died of multiple organ failure in the end. This case is an unusual presentation of complete course of cardiac rhabdomyosarcoma (CR) from diagnosis, operation, recurrence, and metastasis to death. Although primary CR is highly lethal, operation should be indicated to clarify the diagnosis, relieve symptoms, and improve short-term survival...

Primary cardiac tumors are rare. Of all primary heart neoplasms, sarcomas account for around 10%, and of these, rhabdomyosarcomas (RMS) constitute a minority. A case of primary left atrial RMS, reminiscent of leiomyosarcoma, is reported herein. Histologically, the tumor was composed of tightly-packed spindle-shaped cells arranged in long fascicles. At immunohistochemistry, the tumor cells stained strongly and diffusely with vimentin, muscle-specific actin, desmin and myogenin, and focally with fast-myosin and sarcomeric actin...

Rhabdomyosarcoma accounts for 20% of primary malignant cardiac tumours. We describe a woman with cardiac rhabdomyosarcoma. She had loss of appetite, loss of weight, and symptoms of heart failure. A mobile tumour in the left atrium, entering the left ventricle during diastole, was detected by transthoracic echocardiography. The tumour was removed surgically in total; histological diagnosis was rhabdomyosarcoma; however, the patient died after 6 months. In conclusion, primary malignant cardiac tumours can simulate heart failure or systemic disorders...

Rhabdomyosarcoma is a rare malignant tumor of the heart that accounts for 20% of all primary malignant neoplasms of the heart. Symptoms vary in accordance with the location of the mass; unfortunately, by the time the patient becomes symptomatic, the tumor has already metastasized to other organs. Diagnosis is frequently obtained via transthoracic or transesophageal echocardiography and nuclear magnetic resonance imaging. Surgery is indicated for malignant cardiac neoplasms to relieve cardiac symptoms and to prolong patient survival...

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Primary cardiac neoplasms are rare and occur less commonly than metastatic disease of the heart. In this overview, current published studies concerning malignant neoplasms of the heart are reviewed, together with some insights into their aetiology, diagnosis and management. We searched medline using the subject 'cardiac neoplasms'. We selected about 110 articles from between 1973 and 2006, of which 76 sources were used to complete the review. Sarcomas are the most common cardiac tumours and include myxosarcoma, liposarcoma, angiosarcoma, fibrosarcoma, leiomyosarcoma, osteosarcoma, synovial sarcoma, rhabdomyosarcoma, neurofibrosarcoma, malignant fibrous histiocytoma and undifferentiated sarcoma...

We describe a case of a 37-year-old Hispanic woman who presented with a right atrial botryoid rhabdomyosarcoma 2 years after doxorubicin therapy for a recurrent pleomorphic rhabdomyosarcoma of the chest wall. Although this may be a second primary, it most likely represents a cardiac metastasis with maturation of chemotherapy-resistant tumor cells.