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Query: UMLS:C0027651 (tumor)
 685,946 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We report of a case of cardiac tumor in a three months old infant, examined with echocardiography because a murmur was noted on routine examination. Further investigations brought out the diagnosis of tuberous sclerosis. Spontaneous regression of the tumor mass occurred in the following months. No signs of cardiac dysfunction of CNS involvement appeared. The peculiarity of rhabdomyoma as an early manifestation of tuberous sclerosis is emphasized.
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PMID:[Cardiac rhabdomyoma in tuberous sclerosis. Presentation of a case]. 152 99

The cytomorphology of three subependymal giant cell astrocytomas (SEGA) is described. The tumors occurred in the left lateral ventricle of three males with tuberous sclerosis. The often-polarized spindle and epithelioid tumor cells possessed dense eosinophilic cytoplasm, eccentric nuclei and visible, occasionally prominent nucleoli. In addition, they displayed thick or hairlike processes and had a distinct tendency to form cohesive clusters as well as pseudorosettes. Occasional binucleate and multinucleate cells, as well as "strap" cells and nuclear cytoplasmic inclusions, were further features of this unique tumor. In cytologic terms the principal differential diagnostic considerations include gemistocytic astrocytoma, giant cell glioblastoma and ependymoma. Since, in isolation, SEGA may represent a "forme fruste" of tuberous sclerosis and since patients with tuberous sclerosis may have brain tumors other than SEGA, it is of diagnostic importance to recognize the cytomorphologic features of this essentially benign brain tumor.
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PMID:Cytomorphology of subependymal giant cell astrocytoma. 154 2

Five cases of hepatic tumor deemed unresectable by conventional techniques are presented to illustrate the potential benefit offered by complete hepatic vascular exclusion (Pringle maneuver associated with inferior vena cava clamping below and above the liver). The most extensive and difficult liver resections may be achieved, with possible venous reconstruction, if the clamping period is not interrupted. Hepatic vascular exclusion may exceed one hour, up to 85 minutes in this series, with a good liver tolerance, in the absence of preoperative liver dysfunction. The advantages and disadvantages of the ex situ extracorporeal liver resections performed under similar circumstances are discussed. The authors consider that the role of ex situ liver procedures should be very limited.
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PMID:[Is extracorporeal liver surgery useful for difficult hepatic resections?]. 155 Mar 15

From January 1986 to March 1989, 20 patients with stage III and IV cerebello-pontine angle neurinomas were treated with external fractionated radiation therapy; seven patients had phacomatosis. Indications for radiation therapy were as follows: (a) poor general condition or old age contraindicating surgery, 10 patients; (b) hearing preservation in bilateral neurinomas after contralateral tumor removal, 5 patients; (c) partial resection or high risk of recurrence after subsequent surgery for relapse, 4 patients; (d) non-surgical relapse, 1 patient. Most patients were irradiated with a 9 MV linear accelerator. A 3 to 4-field technique with 5 x 5 cm portals was used. Doses were calculated on a 95% isodose and were given 5 days a week for a mean total dose of 5140 cGy (180 cGy/fraction). Median follow-up from radiation therapy was 30 months (7 to 46); 4 patients died, 2 with progressive disease. Two patients underwent total tumor removal after radiation therapy (1 stable and 1 growing tumor). On the whole, 14 tumors remained stable, 3 decreased in size, and 3 progressed. CT scan or NMR tumor changes consistent with partial tumor necrosis appeared in four cases. Hearing preservation was obtained in 3/5 hearing patients with phacomatosis. When surgery is not indicated or incomplete, fractionated radiation therapy appears to be an effective and well-tolerated treatment for stage III and IV neurinomas. Hearing can be preserved.
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PMID:Fractionated radiation therapy in the treatment of stage III and IV cerebello-pontine angle neurinomas: preliminary results in 20 cases. 157 11

A case of tuberous sclerosis with congenital brain tumor was reported. The diagnosis was made on the basis of the pathological findings of subependymal giant cell astrocytoma, after surgery at the newborn period. After the neonatal period, the patient presented the classical triad of seizures, white spot of skin and mental retardation. The exact nature of tumor-forming giant cells remains controversial. We studied the nature of these cells using immunohistochemical method. GFAP, S-100 protein and NSE stains were all positive. This result suggested that the tumor cells had the features of both neurons and astrocytes.
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PMID:[A case of tuberous sclerosis with congenital brain tumor; an immunohistochemical study]. 159 Oct 26

Five children with tuberous sclerosis (TS), a polysystemic disease, had cardiac tumors detected by B-mode echocardiography. In a fetus, sonography performed at the eighth month showed the presence of a cardiac mass and of polycystic kidneys: the diagnosis of TS was confirmed postnatally because of the presence of calcified cerebral nodules. A newborn dichorial twin had paroxysmal tachycardia at ten days. B-mode echocardiography showed the presence of seven intracardiac tumors, and cerebral CT the presence of multifocal periventricular calcifications. An absence in a nine month old baby prompted an electrocardiogram that proved abnormal; a B-mode echocardiography showed large apical cardiac tumor; again cerebral CT showed periventricular calcified nodules. Two girls, 9 and 10 y.o. respectively, affected by TS, with normal electrocardiograms, both presented a small, asymptomatic, intracardiac mass demonstrated sonographically. In recent years, prenatal sonography and B-mode echocardiography in patients with TS demonstrated with increasing frequency the association of cardiac tumors (rhabdomyomas) with TS. Tumor detection often precedes the appearance of the cutaneous and neurological signs typical of the disease. This paper emphasizes the role of echocardiography in detecting cardiac tumors, as an early sign for the diagnosis of tuberous sclerosis, and to depict the natural history of cardiac rhabdomyoma with its variable clinical presentation and prognosis.
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PMID:[Cardiac rhabdomyoma in tuberous sclerosis. A report of five cases and review of the literature]. 163 32

We reported 29 kidneys with angiomyolipoma in 23 patients (14 women and 9 men). One of these cases was associated with tuberous sclerosis. Their age ranged from 20 to 82 years (mean 48.9). Six patients had bilateral renal involvement and 7 had multiple lesions in one kidney. Synchronous renal tumors were noted in three patients. One patient was associated with renal cell carcinoma, and the other two patients with transitional cell carcinoma. Nineteen tumors in 16 patients were larger than 4 cm. Among them 93.8% (15/16 patients) were symptomatic and tumor hemorrhage was found in 57.9% (11/19 kidneys). Of these patients, 17 renal units underwent surgical intervention (including 8 total nephrectomy, 7 partial nephrectomy and 2 enucleation of tumor). Preoperative diagnosis was made in 82.4% (14/17) with combined imaging of sonogram and CT scan. One patient received embolization only. There were two mortalities: one patient with tumor rupture who received emergent nephrectomy but died of irreversible hypovolemic shock, and the other with tuberous sclerosis and pulmonary fibrosis who received embolization for spontaneous rupture of tumor but expired due to respiratory failure. Among nine patients receiving renal preserving operation, the post-operative effective renal plasma flow of the affected kidneys ranged from 96 to 184 ml/min (mean 131.7 ml/min). There was no evidence of recurrence during a mean followup of 2.6 years. Eight asymptomatic patients with 9 renal masses had incidental angiomyolipoma and did not undergo exploration. We did not note any progression of the lesions in the cases under observation for a mean followup of 2.6 years.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Renal angiomyolipoma: experience of 23 patients. 165 40

We report on a case of intracardiac rhabdomyoma diagnosed prenatally from two-dimensional echocardiograms. The tumor was diagnosed by intrauterine echocardiography at the 38th week of gestation. Autopsy proved that the intracardiac tumor was a rhabdomyoma. Tuberous sclerosis spots in the brain tissue were also found.
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PMID:Prenatal diagnosis of intracardiac rhabdomyoma: a case report. 168 63

The authors report a case of subependymal giant cell astrocytoma associated with tuberous sclerosis in a 15-year-old boy. Computed tomographic scans showed a large intraventricular mass with peritumoral calcification and a cyst in the left lateral ventricle. Left dominant unilateral hydrocephalus was also revealed. Magnetic resonance images clearly demonstrated the lesion. The tumor was subtotally removed and a ventriculoperitoneal shunt was performed because of the hydrocephalus. The proliferation potential was assessed by measuring the bromodeoxyuridine (BUdR) labeling index employing the in vitro labeling method, and determining the deoxyribonucleic acid (DNA) content by flowcytometry. BUdR-positive cells were found to be rare, and the DNA histogram demonstrated no evidence of high proliferative activity or aneuploidy.
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PMID:Subependymal giant cell astrocytoma associated with tuberous sclerosis: with special reference to cell kinetic studies--case report. 170 61

The etiology of hemiplegic cerebral palsy has been elucidated by surveys of affected children using CT. MR imaging has contributed to the more exact diagnosis of the nature and extent of hypoxic ischemic damage and allows the accurate estimation of the time of the insult. Serial MR imaging and MR spectroscopy may be useful for predicting the prognosis for cerebral palsy and neurodevelopmental delay. The damage caused by intrauterine infection is best appreciated on MR imaging, though CT is important in showing calcification. Similar considerations apply to tuberous sclerosis, and new observations may facilitate MR imaging diagnosis. Gadolinium-enhanced MR imaging is the most accurate modality for detection of tumor recurrence and metastatic spread. Enhancement due to reactions to and complications of surgery can usually be distinguished. The reliability of MR imaging in showing many congenital anomalies provides evidence of the incidence of these lesions and contributes to understanding the disordered embryology. Most of the changes described in inherited metabolic disorders are nonspecific, but they may suggest a particular diagnosis, and MR imaging is the most sensitive modality for monitoring progress. Lifesaving intervention for arteriovenous malformations involving the vein of Galen requires detailed noninvasive imaging for selecting the most effective approach. Technical developments in diffusion and in flow imaging promise to further increase our diagnostic capabilities and to limit the application of invasive techniques.
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PMID:Imaging of pediatric and congenital brain disease. 173 98


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