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At least 865 records · Page 48Linked to original sources
PubMed1998

Neurocytoma/rhabdomyoma (myoneurocytoma) of the cerebellum.

An unusual case of cerebellar neurocytoma with rhabdomyomatous differentiation in a 6-year-old boy is reported. Immunocytochemical and ultrastructural features of the tumour were studied. Abortive synapse formation, the presence of clear vesicles and synaptophysin immunoreactivity of the tumour cells indicated its intermediate neuronal differentiation, while the presence of myoblasts and myotubes and immunolabelling by desmin confirmed the rhabdomyomatous differentiation. In addition, the mesenchymal cells variably expressed neurofilament protein and glial fibrillary acidic protein, suggesting inductive interaction between the neuroectodermal and ectomesenchymal elements and persistence of the pleuripotential nature of the cells along the rhombic area of the brain stem.

Cerebellar Neoplasms↗
PubMed2006

Natural history of the Nihon (Bhd gene mutant) rat, a novel model for human Birt-Hogg-Dubé syndrome.

In the Nihon rat, an established model of hereditary renal cell carcinoma (RCC), the propensity for tumor development, is inherited as an autosomal dominant trait due to a single germline nucleotide insertion mutation in the rat Bhd ortholog. The Birt-Hogg-Dubé (BHD) syndrome is a rare autosomal dominant disease characterized by fibrofolliculoma, pulmonary cysts, spontaneous pneumothorax, and renal neoplasm. The renal lesions of the Nihon rat are characterized, and extrarenal lesions are also described in this work. The earliest lesion of the RCC was identified as an altered tubule at as early as 3 weeks of age and rapidly progressed through adenoma to carcinoma with the primary cell type being clear/acidophilic where some similarities were evident to RCCs in BHD syndrome. The Nihon rats demonstrate a heterotopic ossification within RCCs and three extrarenal lesions, clear cell hyperplasia/adenoma of the endometrium, clear cell change of the epithelium of striated portions of salivary glands, and cardiac rhabdomyomatosis. This rat model of hereditary RCC provides a useful tool for analyzing the series of events leading to renal tumorigenesis and for studying BHD gene functions.

Adenoma↗
PubMed1990

Surgical treatment of cardiac tumors: a 25-year experience.

From 1964 to 1989, we performed operations on 133 patients with cardiac tumors. There were 58 male and 75 female patients ranging in age from three days to 81 years; 101 were adults, and 32 were children (less than 12 years of age). Primary tumors (102 benign and 12 malignant) were found in 114 patients and metastatic tumors in 19. Symptoms included congestive heart failure, arrhythmias, emboli, and chest pain. Diagnosis was accomplished through angiography, echocardiography, computed tomography, and magnetic resonance imaging. Operative treatment encompassed techniques ranging from biopsy to complete excision (including hypothermic circulatory arrest and cardiac autotransplantation) depending on the site of disease and the extent of involvement. Overall operative survival was 91%. Twelve patients died early (within 30 days of operation), and follow-up was obtained for 110 (90.9%) of the remaining 121 survivors (total patient-years of follow-up, 572.8; mean follow-up, 5.2 years). Of the 20 patients who died late, 15 had malignant disease. Operative survival for patients with primary cardiac malignancies and for those with metastatic disease was 83% and 68.4%, respectively, with 3 and 5 patients, respectively, still living. We advocate an aggressive surgical approach, especially in patients with benign tumors, who can expect an excellent outcome. For patients with malignant or metastatic disease, palliation and cure are also possible if aggressive surgical actions are taken.

Adenocarcinoma↗
PubMed1995

Neonatal pulmonary autograft implantation for cardiac tumor involving aortic valve.

We diagnosed in a 4-day-old neonate a cardiac tumor involving the left atrium, left atrioventricular junction, left ventricular outflow tract, and aortic valve with severe subvalvular and valvular aortic stenosis. The critical involvement of the aortic valve and the scarcity of neonatal cardiac donors led us to perform a successful replacement of the aortic root with a pulmonary autograft, using a very small homograft for the native pulmonary valve (Ross operation).

Aortic Valve↗
PubMed1989

Coexistent cardiac tumours and malformations of the heart.

Cardiac tumours and anatomical malformations of the heart may produce similar clinical signs and symptoms. The coexistence of these two abnormalities complicates diagnosis and probably adversely affects prognosis. We present a review of four cases of this rare combination. In the first case, Ebstein's malformation was present in a child with tuberous sclerosis and cardiac rhabdomyomata. Right ventricular rhabdomyomata were associated with a hypoplastic tricuspid valve in the second case. In the third case, cardiac myxomas were detected in a child with a double-chambered right ventricle. The fourth case was a child with a fibroma of the right ventricle with pulmonary atresia. We propose that, in some circumstances, a space-occupying lesion may be associated with, or possibly induce, a malformation within the developing heart.

Ebstein Anomaly↗
PubMed1993

Surgical removal of a lipoma of the heart.

In a 29-year-old woman echocardiography revealed a tumour originating from the anterior wall of the right ventricle. Noninvasive findings aroused suspicion of a lipoma. The tumour was removed under cardiopulmonary bypass, the resulting defect in the right ventricular wall being covered with a Goretex patch. Histological examination classified the tumour as a rhabdomyolipoma.

Adult↗
PubMed1982

Two-dimensional echocardiography of intracardiac masses: echo pattern-histopathology correlation.

Two-dimensional echo patterns (EP) of 21 pathologically confirmed intra-cardiac masses (10 tumors, 6 thrombi and 5 vegetations) were correlated with histopathology. Three types of echo patterns were defined. Five tumors and 3 thrombi demonstrated a fine, speckled and uniform appearance (type AEP) and none had any hemorrhages or calcification on pathological examination. Three left atrial masses showed large and discrete echolucent areas (type B EP) and histopathological correlation revealed large hemorrhages in the tumors and a large area of clot lysis in the thrombus. Three tumors, 2 thrombi and 5 vegetations revealed either localized areas of bright echoes or appeared uniformly bright (type C EP) and on pathological examination, all had calcification and/or fibrosis. Two-dimensional echo patterns are useful in delineating some of the structural components of intracardiac masses.

Coronary Disease↗