1/12. Primary cardiac sarcoma in pregnancy: a case report and review of the literature.Primary cardiac sarcoma (PCS) is a rare disease with a poor prognosis, because of diagnostic delay, therapeutic difficulties, and high metastatic potential. Surgery is the standard treatment. A case of PCS in pregnancy is reported, with a review of published surgical series of PCSs, focusing on the role of surgery and adjuvant therapy. Prompt surgery improved cardiac function and patients' outcome in comparison with untreated cases. The role of adjuvant treatment was analyzed only in a few series, mainly without distinction between postoperative chemotherapy and radiotherapy; adjuvant therapy improved survival in the larger series of resected PCSs. Only three other cases of PCS in pregnancy were reported. In the present case, resection was performed with no major complication for the mother and the infant. Even if the patient's survival was short, cardiac surgery allowed prolonging of pregnancy until an acceptable possibility of fetal survival was reached. Although resection is not curative in most cases, surgery remains the treatment of choice for PCS and has a definite palliative significance. The role of postoperative chemotherapy and radiotherapy is difficult to ascertain; however, adjuvant chemotherapy seems advisable in high-grade tumors.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
2/12. GnRH agonist for intravenous leiomyomatosis with cardiac extension. A case report.BACKGROUND: Intravenous leiomyomatosis with cardiac extension is an extremely rare disease. CASE: We recently treated a case of intravenous leiomyomatosis with extension from the inferior vena cava into the right atrium. Three operations--exploratory laparotomy, debulking of the pelvic mass and resection of the intracardiac leiomyoma--were performed. Since cells of the resected leiomyomatosis were estrogen receptor positive, we postoperatively administered GnRH agonist (leuprorelin acetate) for six months to prevent regrowth of the residual mass in the pelvis. The residual mass began to enlarge immediately after cessation of leuprorelin acetate. The same medication was readministered, and regrowth of the residual mass was completely inhibited for 15 months, until this writing. CONCLUSION: Intravenous leiomyomatosis seems to be hormone dependent, as in the case of uterine leiomyomas. In the absence of total resection, functioning ovarian tissue may remain. Therefore, long-term treatment with GnRH agonist may be useful in preventing recurrence of this disease.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
3/12. Left atrial myxosarcoma with systemic metastasis: a case report.The term myxosarcoma is currently not used in standard classification for soft tissue tumors, but restricted to cardiac tumors. Primary cardiac myxosarcoma is a very rare disease and is difficult to differentiate from myxoma clinically and pathologically. We report a case of left atrial myxosarcoma with widespread systemic metastasis in a 21-yr-old male. The patient presented with sudden onset of intermittent dyspnea and orthopnea. echocardiography showed a mobile, pedunculated tumor, 7.5x5x2 cm in size, at left atrium. Histologically, the excised tumor showed an amorphous finely fibrillar and mucinous stroma, in which irregular cords and clusters of lepidic cells and large stellate cells with plump vesicular nuclei resembled the usual type of cardiac myxoma were noted. And it showed focally cellular area with great nuclear pleomorphism and frequent mitoses. The patient received combination chemotherapy, peripheral blood stem cell collection transplantation and operations for systemic metastases in the brain, skeletal muscle and lung. He is alive at present 37 months after initial diagnosis and has no more new metastatic lesion.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
4/12. An unusual cause of new-onset atrial flutter: primary cardiac lymphoma.Primary cardiac lymphoma is a rare disease with a high mortality rate due to the advanced stage of myocardial involvement at presentation. The diagnosis is extremely difficult to make because of the rarity of the disease, variability of clinical manifestations, limited noninvasive diagnostic techniques available, and difficulties and/or delays in the use of invasive measures. The incidence of the disease is increasing, especially among immunocompromised patients, with those suffering from acquired immunodeficiency syndrome accounting for the greatest increase. We report the case of an immunocompetent 76-year-old black woman who presented with near-syncopal episodes. Transthoracic echocardiogram revealed a right atrial mass. Surgical resection was performed, and a diagnosis of large B-cell non-Hodgkin's lymphoma was made. Primary cardiac lymphoma should be considered in any patient with a cardiac mass. Prompt diagnosis and treatment of primary cardiac lymphoma is imperative for survival.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
5/12. Spontaneous rupture of a right atrial angiosarcoma and cardiac tamponade.Primary cardiac angiosarcoma is a rare disease of difficult diagnosis and poor prognosis frequently associated with recurring hemopericardium. We report the case of a 30-year-old female with a right atrial angiosarcoma and spontaneous rupture to the pericardial cavity, who was diagnosed during an emergency exploratory thoracotomy, whose indication was cardiac tamponade. This is the 8th case reported in the literature. Clinical findings are discussed and a literature review is provided.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
6/12. Primary cardiac Burkett's type lymphoma: transthoracic echocardiography, multidetector computed tomography and magnetic resonance findings.Although primary cardiac lymphoma is an extremely rare disease and is associated with high mortality, it is treatable when diagnosed appropriately. We describe the transthoracic echocardiography, 16-row multidetector computed tomography (16-MDCT) and magnetic resonance findings of primary cardiac lymphoma in an immunocompromised patient and review of the literature.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
7/12. Cardiac myxoma as the cause of death in an infant.Cardiac myxoma is a rare disease most frequently encountered in adults, usually localized in the left atrium. It rarely occurs in infants. We report a 3 1/2 month old girl whose sudden death was caused by a large atrial myxoma, completely occluding the tricuspidal orifice. The importance of possible prodromal symptoms suggesting a cardiac disease is stated, and the role of echocardiography as an excellent examination technique is discussed.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
8/12. Intracaval and intracardiac leiomyomatosis of uterine origin.Intracaval leiomyomatosis of uterine origin is a rare disease. Extension to the right heart is exceptional. Based on the review of 11 cases reported in the literature and the case presented herein, which was treated successfully, the diagnostic and therapeutic problems are discussed. diagnosis should be suggested when a female patient operated on previously for myofibroma of the uterus by hysterectomy, presents with a picture of cardiac myxoma. diagnosis can be confirmed by iliocavogram and computerized tomography of the abdomen. Excision calls for a cardiac procedure under extracorporeal circulation and caval exploration which may be performed either simultaneously or as a two stage procedure.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
9/12. Malignant schwannoma of the heart.Primary cardiac tumor is an extremely rare disease entity. Only three cases of primary malignant cardiac schwannoma, the subject of this report, have been recorded in japan. Recently, we encountered a case of malignant schwannoma in which retention of pericardial effusion was the first clinical finding. This case was a 30-year-old female, who had dyspnea at work, general fatigue, and fever. Striking cardiac expansion was seen, with a cardiothoracic ratio (CTR) of 69% on chest x-ray. Two-dimensional echocardiograms showed a large volume of pericardial effusion between the side wall of the left ventricle and the epicardium, and the presence of a parenchymatous tumor. An increase in tumor size was detected on chest computer tomography (CT) scan. Using a pump oxygenator, median sternotomy was performed to reach the epicardium. A pale yellow, soft tumor was seen in the left atrium near the left ventricle. Histologically, the patient was diagnosed as having a malignant schwannoma. We have reported a case of primary malignant schwannoma which was surmised to have arisen from the boundary between the atrium and the ventricle.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
10/12. Primary angiosarcoma of the heart. Report of a case and review of the literature.We report on a 44-year-old male with primary cardiac angiosarcoma who died 11 months after onset of nonspecific symptoms (thoracic pain and general fatigue) of intracerebral metastases. A right atrial tumor mass and a pericardial effusion could be demonstrated by transthoracic and transesophageal echocardiography. Cardiac angiography showed a right atrial hemangioma, fed by the right coronary artery. In a review of 108 cases of primary cardiac angiosarcoma we summarize clinical features, diagnostic means, therapeutic approaches and life expectancy of this rare disease.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
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