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

A case of renal angiomyolipoma with regional lymph node involvement is added to 20 cases previously reported. This represents the 11th case reported in a non-tuberous sclerosis patient. The electron microscopic features of the tumor are discussed and correlated with the light microscopic findings.
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PMID:Angiomyolipoma of the kidney with lymph node involvement. 175 10

We describe a 55-year-old woman with pulmonary lymphangioleiomyomatosis. The patient died of respiratory failure 9 years after diagnosis. An autopsy confirmed the diagnosis and disclosed multiple soft-tissue tumors, including a large solitary fibrous tumor of the lung, a huge cavernous hemangioma of the liver, a meningioma of the right pontocerebellar angle, and a focus of nodular stromal hyperplasia of the ovary. In addition, endocrine tumors, including a papillary carcinoma of the thyroid gland and a parathyroid adenoma, were also found. There was no evidence of tuberous sclerosis or angiomyolipoma of the kidney. The connection between pulmonary lymphangioleiomyomatosis and tuberous sclerosis is discussed.
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PMID:Pulmonary lymphangioleiomyomatosis. Report of a case with associated multiple soft-tissue tumors. 176 17

Angiomyolipoma is an uncommon benign tumor of the kidney. The tumor is composed of fat, smooth muscle, and blood vessels. The same pathological entity can appear in two clinically different forms, with or without tuberous sclerosis. We present two cases of renal angiomyolipomas with unusual manifestations. One was associated with tuberous sclerosis and both had the presentation of acute abdomen. Case one presented with epilepsy, angiofibroma, subungual fibroma, periventricular calcification, and bilateral renal angiomyolipomas. Tuberous sclerosis is characterized by these findings. Both cases had spontaneous hemorrhage with hypovolemic shock. Massive hemorrhage resulting in shock is uncommon and the incidence has been estimated to be about 10 per cent. In fact, many angiomyolipomas are clinically occult. The size of the tumor correlates well with the presence or absence of symptoms which include microhematuria, flank pain, hypertension and urinary tract infection. Abdominal CT is the preferred modality for diagnosis of angiomyolipoma. The most important finding is the presence of an intrarenal tumor with fat component which is recognized as a relative low density on CT. Our patients were hospitalized under the impression of angiomyolipoma after the CT study. In addition, the CT defined either the size of the tumor or the extension of the hemorrhage. Although many believe that renal angiography is not sufficient by itself to establish the diagnosis of angiomyolipoma, occasionally it is mandatory in the management of the tumor. The management is decided by two factors, the size of tumor and the clinical presentation. The attitude of management should include conservative treatment with regular follow-up, selective arterial embolization, enucleation, and partial or total nephrectomy.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Unusual presentations of angiomyolipoma]. 179 71

Subependymal giant-cell astrocytomas (SEGA) are rare brain tumors. They occur typically in the walls of the lateral ventricles and very seldom in the wall of the third ventricle. In our experience, all patients with SEGA have had tuberous sclerosis. Between January 1950 and May 1990, of our 345 patients with tuberous sclerosis 22 were found to have SEGA. The diagnosis being made histologically in symptomatic cases or by clinical or neuroradiologic methods. The histological, immunohistochemical and ultrastructural features of this unique tumor are discussed. To date, histopathologic methods have not resolved the issue of whether these tumors are exclusively astrocytic or neuronal in nature, or show features of both. Modern immunohistochemical and/or molecular-biological techniques will no doubt elucidate the histogenesis of this highly characteristic, albeit polymorphous tumor.
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PMID:[Subependymal giant cell astrocytoma]. 191 24

We describe a term newborn with tuberous sclerosis who presented with a neonatal brain tumor, diagnosed as a subependymal giant cell astrocytoma. We compare the various imaging modalities used in the diagnosis of this tumor.
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PMID:Neonatal subependymal giant cell astrocytoma associated with tuberous sclerosis: MRI, CT, and ultrasound correlation. 198 77

We studied the clinical and pathologic features of 17 cardiac rhabdomyomas from 13 males and four females whose ages ranged from birth to 9 yr (mean, 36 wk). Eleven were multiple, and tumors were found throughout the heart. Four patients had congenital heart disease, and three had tuberous sclerosis; of the ten sporadic cases, four were surgical resections. Three of the four patients with surgical resections survived postoperatively. Two patients presented with sudden cardiac death. Immunohistochemical stains on seven tumor revealed diffuse positivity for myoglobin, actin, desmin, and vimentin, with negative results for S-100 protein, similar to adjacent cardiac muscle. We conclude that cardiac rhabdomyomas can be sporadic, can be associated with tuberous sclerosis, or can be seen with other cardiac malformations. They usually present early in life with a variety of symptoms, including sudden death, and attempts at resection may be successful.
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PMID:Cardiac rhabdomyoma: a clinicopathologic study. 202 Jun 64

Echocardiography was performed in 60 people with tuberous sclerosis (TSC) to ascertain the prevalence of cardiac rhabdomyomas at different ages. Twenty-five of 43 children, but only 3 of 17 adults, had tumors, the difference being highly significant. The number and absolute size of the tumors in those who had any were much the same throughout childhood, although the tumors in adults were smaller. Four cases of prenatal tumor recognition are also described; all four later showed signs of TSC, although none had tumors at birth. One of these four is already included in the series of 60, and one single tumor was removed surgically; in the other two the tumors regressed, as they did in the only three infants in the group of 60. The evidence presented herein, together with some published case reports, suggests that echocardiography may afford the most useful diagnostic test for TSC in early infancy and also that a conservative policy should be followed in the management of symptomatic tumors.
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PMID:Cardiac rhabdomyomas in tuberous sclerosis. 203 67

Rhabdomyoma is the most common intracardiac tumor in the newborn. The diagnosis is made by the clinical history and the physical examination, the electrocardiogram, chest X-ray, and the echocardiographic data. It has a poor prognosis, 80% dying in the first year of life because congestive heart failure or sudden death. Rarely, spontaneous regression has been described. We present the case of a newborn diagnosed of tuberous sclerosis, with an echocardiographic image of a rhabdomyoma in the right ventricle with a spontaneous total regression of the tumor 2 years later.
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PMID:[The spontaneous regression of a rhabdomyoma of the right ventricle]. 204 50

Rats with an adenocarcinoma of the colon implanted into the liver were treated by a bolus injection of adriamycin by the hepatic artery. In addition, vascular occlusions were performed in the following three ways. 1. The hepatic artery was ligated (HAL) immediately after adriamycin injection. 2. The portal venous branch nourishing the tumor was ligated immediately after adriamycin injection. 3. The Pringle maneuvre (clamping of the hepatic artery, the portal vein and the common bile duct) was performed during 5 min before and 10 min after injection. Tumor size was measured at operation and 6 and 12 days later in the first experiment, 7 days later in the other two experiments. The combination of adriamycin and HAL retarded tumor growth at day 6 as compared to controls, adriamycin alone and HAL alone. The differences were not significant at day 12. The other vascular occlusions did not improve the antitumor effect of adriamycin.
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PMID:Hepatic vascular occlusion combined with adriamycin given by the hepatic artery in rats with adenocarcinoma in the liver. 206 27

Of 345 patients with tuberous sclerosis complex evaluated at the Mayo Clinic from 1950 to 1989, 23 were identified as having brain tumors. In 20 of the 23, histological or clinical evidence showed the tumor to be a subependymal giant cell astrocytoma. A search of the Mayo Clinic tissue registry yielded 73 giant cell-containing astrocytomas and intraventricular gliomas exclusive of ependymomas. Reexamination revealed no further examples of subependymal giant cell astrocytoma in patients without features of the tuberous sclerosis complex. Considerable histological variation was observed in the 15 subependymal giant cell astrocytomas subjected to critical microscopic review. It is of note that no correlation was noted between either the histological features, such as atypia, mitoses, endothelial proliferations, necrosis, or the flow cytometric characteristics and the clinical course or the survival time of the patients.
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PMID:Subependymal giant cell astrocytoma: a clinical, pathological, and flow cytometric study. 206 10


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