Dystrophic calcification of the fetal myocardium.
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Intramural cardiac masses were detected antenatally in three fetuses by echocardiography. The masses were initially thought to be rhabdomyomas. All three pregnancies were terminated and histology showed dystrophic calcification in all, with no evidence of tumour. Therefore, dystrophic calcification of the fetal myocardium may have a similar appearance to single or multiple rhabdomyomas. This should be considered when counselling parents after detection of masses in the fetal heart, particularly when considering the risk of associated tuberous sclerosis. (+info)
Spectrum of cystic variants of Wilm's tumour: cystic nephroma (multilocular cyst) and cystic partially differentiated nephroma--a report of four cases.
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Two cases of cystic nephroma (multilocular cyst of the kidney), and one case each of cystic partially differentiated nephroblastoma (CPDN) and rhabdomyomatous Wilms' tumour are described. All were male and in the pediatric age group. Grossly tumours were unilateral, unicentric and multiloculated. The need for proper designation of these lesions is highlighted because of difference in the treatment and prognosis of these tumours. (+info)
Ultrastructural changes in medullomyoblastoma. Similarities with foetal rhabdomyoma.
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The light and electronmicroscopic changes are described in two cases of medullomyoblastoma, and compared with the changes seen in a case of foetal rhabdomyoma. The medullomyoblastomas in two children aged 8 and 5 years, consisted predominantly of classical type of medulloblastoma cells, along with few to many 'strap cells' or 'myoid cells' which, on closer examination, showed clear cross striations, consistent with muscle fibres or myofibrils. The primitive myoid cells were similar to those encountered in larger numbers in a post-auricular rhabdomyoma, possibly of foetal origin in a 40 day old infant. The four pathogenetic mechanisms i.e. (i) an embryonal stage of myofibrillar differentiation; (ii) a malformative factor; (iii) a teratoid factor on account of the presence of mesenchyme derived striated muscle tissue in the obviously predominant ectodermal medulloblastoma; and (iv) metaplasia of the vascular smooth muscle cells in the medullomyoblastoma, are discussed. (+info)
Prenatal detection of cerebral lesions in a fetus with tuberous sclerosis.
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We report a newborn, diagnosed prenatally with both cardiac rhabdomyomas and a brain tumor. To the best of our knowledge, this is the first report of central nervous system (CNS) lesions detected prenatally in a child with tuberous sclerosis with term follow-up. At 36 months, the child has normal growth and is developing appropriately. Thus the finding of CNS tumors on fetal ultrasound examination can help in the prenatal diagnosis of tuberous sclerosis but does not necessarily indicate a poor prognosis. (+info)
Oesophageal rhabdomyoma.
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Extracardiac rhabdomyomas are rare benign tumours showing striated muscle differentiation. Seventy percent of these lesions occur in the head and neck region. The most common sites for these lesions are the larynx, pharynx, and the floor of the mouth. There has been only one previous report of a rhabdomyoma of the oesophagus; two further cases are described. (+info)
Perinatal diagnosis of cardiac tumors.
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OBJECTIVE: As fetal cardiac tumors are a rare condition, we report the perinatal diagnosis and ultrasound findings of 12 cases. METHODS: In 10 cases the tumors were identified prenatally by fetal echocardiography; gestational age at detection ranged from 22 to 34 weeks. In two symptomatic infants cardiac tumors were diagnosed on the first day postpartum; prenatal ultrasound evaluation at 26 and 38 weeks of gestation did not reveal a cardiac lesion. RESULTS: Six fetuses had singular tumors, in six fetuses they were multifocal. The left ventricle was most often affected. Termination of pregnancy was chosen in three cases, one in association with trisomy 21 and tuberous sclerosis. One intrauterine and three neonatal deaths due to cardiac failure occurred. Histopathologic examination revealed cardiac rhabdomyoma in six fetuses and fibroma in one case. In the five surviving fetuses the size of the tumors spontaneously decreased postpartum. Rhabdomyomata were associated with tuberous sclerosis in four out of 11 cases. CONCLUSION: Cardiac tumors are detectable as early as 22 weeks of gestation. Presenting symptoms may be arrhythmia, dysfunction of the atrioventricular valves, pericardial effusion and fetal hydrops. The most common perinatal diagnosis is rhabdomyoma, which is often associated with tuberous sclerosis. Sequential examination in high risk patients should be considered as most tumors increase in size during pregnancy and may become evident in late second and third trimester of pregnancy. Postpartum, however, regression of tumor size is common. (+info)
Surgical treatment of primary cardiac tumors: 28 years' experience in Kanazawa University Hospital.
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To examine the clinical features of primary cardiac tumors, 34 patients who underwent surgical treatment from 1973 to 2000 at the Kanazawa University Hospital were analyzed and the literature was reviewed. The 34 patients were divided into 3 categories: (i) myxomas; (ii) benign non-myxomas; and (iii) malignant tumors. Twenty-three patients (70%) were diagnosed with myxomas, including 22 left atrial myxomas and 1 right atrial myxoma. Seven patients (18%) were diagnosed with benign non-myxoma tumors, including 3 hemangiomas, 1 fibroma, 1 rhabdomyoma, 1 pheochromocytoma, and 1 lipoma. Four patients (12%) were diagnosed with malignant tumors, including 2 angiosarcomas, 1 rhabdomyosarcoma, and 1 malignant fibrous histiocytoma. Among the myxoma patients, in-hospital mortality was 9% (2/23), late mortality was 10% (2/21), and no recurrent myxomas have been identified. Among benign non-myxoma patients there were no perioperative deaths; however, 1 patient died 11 years after surgery, with no linked cause. No recurrent tumors have been identified. Among malignant tumor patients, 1 patient died the day following surgery and the rest died within 14 months. Early and late results of surgery were acceptable for those patients with benign tumors, while the prognosis for patients with malignant tumors was very poor. (+info)
Serum creatine kinase levels parallel the clinical course for rhabdomyomatous Wilms tumor.
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A right-sided renal mass in an 11-month-old girl was diagnosed by percutaneous needle biopsy as Wilms tumor, which on histologic examination was found to be predominantly rhabdomyomatous. As part of the examination, serum creatine kinase (CK) and CK-MB levels were measured and were significantly elevated at 994 U/L (reference range, 42-180 U/L) and 40 U/L (reference range, 0-3 U/L), respectively. Subsequently, an 8-month-old girl was admitted to the hospital with septicemia and was found to have an abdominal mass. A diagnosis of bilateral Wilms tumor was made following percutaneous biopsy of both kidneys; histologic examination confirmed that the tumor was predominantly rhabdomyomatous. Serum CK and CK-MB levels also were measured and were significantly elevated at 685 U/L and 84.4 U/L, respectively. In both cases, the serum CK and CK-MB levels reflected the clinical course; elevation in serum levels was associated with tumor recurrence, infarction, or chemotherapy-related necrosis. We conclude that these enzymes have clinical usefulness as markers for Wilms tumor showing rhabdomyomatous morphologic features. (+info)