Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0035412 (rhabdomyosarcoma)
 6,156 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

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.
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PMID:Unusual primary tumors of the heart. 1080 31

The present report describes the case of a 61-year-old woman with malignant fibrous histiocytoma of the left atrium originating from the left atrial free wall, operated on in emergency for a suspected large left atrial myxoma that, at the echo scan, was consistently protruding through the left atrioventricular orifice at each diastole and was almost completely occluding the left ventricular inflow, causing signs of congestive heart failure and severe dyspnea. Surgery was performed as radically as possible, but the histological examination of the specimen revealed the exact diagnosis of the neoplasm. About 75% of primary tumors are benign and 75% of these are atrial myxomas. 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. The long-term results for sarcomas are very poor and there are few survivors after several months from surgery due to the extent of local spread and invasion or because of the frequent distant metastases. Malignant fibrous histiocytoma constitutes about 2% of all cardiac malignancies, which might grow within several localized areas, occasionally in the heart. Echocardiography represents the best examination procedure for both diagnosis and follow-up of patients with cardiac tumors.
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PMID:[A rare cardiac tumor: the malignant fibrous histiocytoma. Description of a case]. 1555 23

Primary and secondary neoplasms of the canine and feline heart are uncommon. During a 2-year period, 83 dogs suffering from primary cardiac (n=11), extracardiac benign (n=6) or malignant (n=66) tumours and 30 cats with primary cardiac (n=1) or extracardiac (n=29) malignant tumours were examined. Echocardiography revealed four cases of primary cardiac neoplasms in dogs, but secondary heart tumours were not detected. After necropsy, tissue samples from the heart and tumours were examined histologically and immunohistochemically. In dogs, primary neoplasms included seven haemangiosarcomas, two chemodectomas, one rhabdomyosarcoma, and one neurofibrosarcoma. In 24 of 66 dogs examined, metastases of extracardiac neoplasms were found in the heart (15 carcinomas, six malignant lymphomas, three haemangiosarcomas). In cats, one case of primary haemangiosarcoma of the pericardium and five cases of secondary cardiac tumours (two malignant lymphomas, three carcinomas) occurred. Cardiac neoplasms in cats were not identified clinically but were detected by detailed gross sectioning of the heart (n=2) or histopathological examinations (n=3). This study showed an unexpectedly high number (36%) of dogs with cardiac metastases.
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PMID:Primary and secondary heart tumours in dogs and cats. 1727 Feb 4

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. The classic symptoms of cardiac tumours are intracardiac obstruction, signs of systemic embolisation, and systemic or constitutional symptoms. However, serious complications including stroke, myocardial infarction and even sudden death from arrhythmia may be the first signs of a tumour. Echocardiography and angiography are essential diagnostic tools for evaluating cardiac neoplasms. Computed tomography and magnetic resonance imaging studies have improved the diagnostic approach in recent decades. Successful treatment for benign cardiac tumours is usually achieved by surgical resection. Unfortunately, resection of the tumour is not always feasible. The prognosis after surgery is usually excellent in the case of benign tumours, but the prognosis of malignant tumours remains dismal. In conclusion, there are limited published data concerning cardiac neoplasms. Therefore, a high level of suspicion is required for early diagnosis. Surgery is the cornerstone of therapy. However, a multi-treatment approach, including chemotherapy, radiation as well as evolving approaches such as gene therapy, might provide a better palliative and curative result.
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PMID:Malignant tumours of the heart: a review of tumour type, diagnosis and therapy. 1769 68

We conducted a retrospective study to analyze the role of the cervical and lateral skull base approaches in the surgical excision of parapharyngeal space (PPS) tumors. Our study population was made up of 34 patients-15 males and 19 females, aged 13 to 73 years (mean: 50.6)-who had presented to us with a PPS tumor during a 9-year period. The 34 cases included 23 benign tumors and 11 malignancies. The 23 benign tumors consisted of 11 pleomorphic adenomas, 5 schwannomas, 2 paragangliomas, 2 Warthin tumors, 1 oncocytoma, 1 hamartoma, and 1 osteochondroma. The 11 malignancies included 3 cases of recurrent nasopharyngeal carcinoma, 2 cases of mucoepidermoid carcinomas, and 1 case each of carcinoma ex pleomorphic adenoma, liposarcoma, lymph node metastasis from nasopharyngeal carcinoma, lymph node metastasis from follicular thyroid carcinoma, rhabdomyosarcoma, and cranial nerve (CN) X neurofibrosarcoma. Among the benign tumors, 14 were removed via the transparotid approach, 3 via the transcervical approach, 3 via the transcervical-transparotid approach, 2 via the petro-occipital-trans-sigmoid approach, and 1 via the Fisch type A infratemporal fossa approach. During postoperative follow-up, 5 of the 23 patients with a benign tumor exhibited facial nerve paresis, 2 developed CN IX to XII palsy, and 1 each developed Frey syndrome and CN X palsy. In the malignant tumor group, 4 of the 11 patients were treated via the transcervical-transparotid approach, 3 via the Fisch type C infratemporal fossa approach, 3 via the transcervical-transmandibular approach, and 1 via the transcervical-lateral petrosectomy approach. The neural deficits observed during the postoperative period were more extensive among the patients with a malignant tumor. We conclude that the transparotid and transcervical approaches were adequate for excising most benign tumors. For malignant tumors, large tumors, and tumors with skull base involvement and transcranial extension, the transparotid-transcervical, transcervical-transmandibular, infratemporal fossa, and petro-occipital-trans-sigmoid approaches were necessary.
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PMID:Surgical management of parapharyngeal space tumors: The role of cervical and lateral skull base approaches. 2792 99


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