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

A 6-day-old neonate with tuberous sclerosis having brain and cardiac tumors is reported. The patient presented with a seizure 20 minutes after birth. Cranial ultrasonography and computed tomography on admission showed a large and round high-density tumor lesion in the left frontal lobe. Echocardiography also showed a large tumor lesion beside the left ventricle wall, and a diagnosis of tuberous sclerosis was promptly made. Two months later, a periventricular calcified lesion was found on the demonstrated portion, and the tumor lesion initially observed in the brain had disappeared. These findings suggest that the initial tumor lesion found in the brain developed into a typical calcified lesion.
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PMID:Neonatal tuberous sclerosis with heart and brain tumors. 228 37

Renal angiomyolipoma (AML) is an uncommon, benign neoplasm of a hamartomatous origin that presents as multiple bilateral lesions occasionally associated with tuberous sclerosis, or as a single unilateral lesion. They are generally asymptomatic, and can manifest the following clinical triad: abdominal pain, palpable mass and hematuria. Retroperitoneal hemorrhage is a rare complication (9%). We report 3 cases of spontaneous rupture of renal AML with massive hemorrhage; two of these cases were associated with tuberous sclerosis. This complication was the presenting feature of renal AML in two cases. Radiologic diagnosis and management of this tumor type are highlighted.
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PMID:[Retroperitoneal hemorrhage as a complication of renal angiomyolipoma]. 233 Nov 62

The phenotypic characteristics of 7 subependymal giant cell astrocytomas (GSECG) (6 of these being associated with tuberous sclerosis) are studied using morphological and immunohistochemical methods with antiserums against vimentine, glial fibrillary acid protein (GFA), S100 protein, and neurofilaments. The glycoproteic secretion of the tumor cells was also analyzed after exposure to Concanavalin A (CON A) by a direct fluorescent method. Our results suggest that some GSECG originate from specialized ependyma (circum-ventricular organs). They have the same location (foramen of Monro), present some common ultrastructural features (cytoplasm with lumen containing cilia), are positive with certain immunohistochemical markers (staining with S100 protein in 4 cases, with vimentin in 3 cases) and show a strong glycoproteic secretion (positive with CON A). Therefore, some GSECG might be considered hamartomas of specialized ependyma, with a reduced evolutivity potential.
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PMID:[Histogenesis of subependymal glioma in Bourneville's tuberous sclerosis]. 234 68

Ten patients with clinical tuberous sclerosis were examined with CT and MR imaging, before and after IV contrast in order to determine the role of Gd-DTPA. Gd-DTPA enhancement occurred in eleven subependymal nodules which did not enhance on CT after IV contrast. As illustrated by previous CT and pathologic observations and related to the histologic similarity of the subependymal nodules and giant-cell astrocytomas, these hyperintense nodules could represent active lesions with the potential to evolve. Four giant-cell astrocytomas were detected both with CT and Gd-DTPA-enhanced MRI; tumor conspicuity and size assessment were improved by postcontrast MRI in two cases. No cortical tuber or heterotopic cluster enhanced; T2-weighted sequences therefore remain necessary for their detection. If pre and post-Gd-DTPA T1- and T2-weighted imaging is negative, CT is clearly the most sensitive modality in the detection of the small calcified subependymal nodules.
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PMID:Gadolinium-DTPA enhanced MR imaging in tuberous sclerosis. 235 31

Sacro-coccygeal teratomas (SCT) are rare congenital tumors (1/40,000 births) but the commonest neoplasms in the newborn, generally discovered at birth; they are well known by pediatric surgeons who operate on them during the first days of life because of the high risk of malignant transformation when the diagnosis is delayed after several months of life. Exceptionally these tumors are observed in the adult without being diagnosed during infancy. The case of a 67 year old man is reported; the tumor presented as a solid mass complicated by necrosis and ulceration. The operation involved en bloc resection of the tumor and its sacro-coccygeal bony attaches. The tumor was found to be benign on histological examination. The main problems concerning diagnosis are discussed: possible association with meningocele and intra-pelvic extensions; these two factors must be defined preoperatively as they may change the approach to treatment. TSC can only be treated surgically, and the classical inverted V incision performed with the patient in the prone position is discussed versus other procedures designed a midline sacral scar, including Z plasty. Radical resection of the tumor with its pedunculated last two sacral and coccygeal vertebrae must be performed to avoid recurrence that may be malignant even if the primary lesion was benign.
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PMID:[Giant sacrococcygeal teratoma in adults]. 247 9

Subependymal giant cell astrocytomas associated with tuberous sclerosis were studied with immunostains for glial fibrillary acidic protein (GFAP), myelin basic protein (MBP) and neuron-specific enolase (NSE). Tumor tissue was composed of three forms of cells; polygonal, ovoid and fusiform. A polygonal form was large in size and contained a vesicular nucleus, a distinct nucleolus and scarce Nissl granules resembling a nerve cell. An ovoid cell was similar to a gemistocytic astrocyte. Glial fibers stained with PTAH were observed to surround the tumor cell cluster. Immunocytochemically the polygonal cells were GFAP-and NSE-positive, the ovoid cells were GFAP-, MBP- and NSE-positive, and the fusiform cells were sometimes GFAP- and NSE-positive. The origin of tumor cells remains controversial, either glial or neuronal. This tumor occurs commonly in the subependymal region where germinal matrix cells appear at the early developmental stage. These results suggest that subependymal giant cell astrocytoma could have the totipotential to differentiate to astrocytic, oligodendrocytic as well as neuronal cells.
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PMID:[Immunocytochemistry of subependymal giant cell astrocytoma associated with tuberous sclerosis]. 247 62

Tuberous sclerosis (Bourneville-Pringle phacomatosis) has been known to be associated with cardiac rhabdomyoma, but apparently never previously with primary pericardial mesothelioma. We present an autopsy case of this condition in a 59-year-old man, who had been diagnosed as having tuberous sclerosis in view of the presence of facial sebaceous adenoma, mental retardation, intracranial calcification, cerebral ventricular dilatation and renal tumor. During the clinical course, characterized by heart failure due to cardiac tamponade, cardiac sarcoma was diagnosed by imaging techniques. Autopsy revealed biphasic-type primary pericardial mesothelioma. As to the tuberous sclerosis, atypical giant cells in the tubers of the cerebral cortex and the lateral ventricular wall were found, which were considered to be derived from neurons rather than glial cells on the basis of staining with Bodian, Holzer, and antibodies against NSE, GFA and S-100 protein. In old tubers protruding into the lateral ventricles, fibrous glias were present with dense calcospherite deposits, coinciding with the CT findings. The renal tumors were angiomyolipomas, which were present bilaterally and showed partially infiltrative growth, but seemed to have a benign nature because of the lack of metastasis and atypism of the leiomyocytes.
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PMID:An autopsy case of tuberous sclerosis associated with primary pericardial mesothelioma. 248 38

Between 1954 and 1984, 282 children with astrocytoma were included in the Manchester Children's Tumour Registry (MCTR), giving an overall incidence of 9.3 per million person-years. There were 110 children with adult astrocytoma and 172 children with juvenile astrocytoma. The five-year survival for adult astrocytoma was 15% and 75% for juvenile astrocytoma. There were no significant improvements in survival with time. There were 21 children with neurofibromatosis (NF) and 4 children had tuberous sclerosis. Some children had other recognized syndromes and others had major or minor abnormalities. Nine children had second tumors, mainly associated with NF, and seven siblings had malignant tumors. A number of mothers of these children were found to have breast cancer. Some of these families may represent examples of the Li-Fraumeni cancer family syndrome. We conclude that astrocytomas is an important problem in childhood and that a proportion of cases may have a genetic origin.
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PMID:A review of astrocytoma in childhood. 251 26

The terms, 'hamartoma', 'choristoma', 'phacoma' (mother-spot), and 'naevus', are used to describe benign developmental tumours or placoid lesions. Stedman's Medical Dictionary defines a hamartoma as: 'A focal malformation that resembles a neoplasm grossly and even microscopically, but results from faulty development in an organ; it is composed of an abnormal mixture of tissue elements, or an abnormal proportion of a single element, normally present in that site, which develop and grow at virtually the same rate as normal components, and are not likely to result in compression of the adjacent tissue (in contrast to neoplastic tissue).' A choristoma is defined as 'a mass formed by maldevelopment of tissue of a type not normally found at that site.' Phacoma is defined as, 'a hamartoma found in phacomatosis,' a group of hereditary diseases characterised by hamartomas of multiple tissues. A naevus is a, 'birthmark; a circumscribed malformation of the skin, especially if coloured by hyperpigmentation or increased vascularity; it may be predominantly epidermal, adnexal, melanocytic, vascular, or mesodermal, or a localised overgrowth of melanin-forming cells arising in the skin early in life.' Ophthalmologists have adopted the term to refer to developmental melanocytic lesions of the uveal tract, but heretofore have not used it to describe developmental melanocytic, glial, or vascular lesions of the retina. The purpose of this report is to summarise the author's observations and concepts concerning focal lesions that probably are developmental tumours and placoid lesions composed of either entirely or in part retinal pigment epithelium (RPE). Two of the lesions are tumefactions and may properly be termed 'hamartomas'. Four of the lesions are placoid and might better be described as naevi. Two are composed of hypertrophied RPE cells engorged with melanin granules, one is presumed to be hypertrophied RPE cells filled with a white pigment of unknown nature and one is composed of RPE cells containing no pigment. Three are widely recognised, three others are not.
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PMID:Focal congenital anomalies of the retinal pigment epithelium. 259 92

A case of a 12-year-old boy with tuberous sclerosis who developed life-threatening hematuria is presented. An angiomyolipoma of the lower two thirds of the left kidney resulted in a tumor hemorrhage that was successfully treated by partial nephrectomy.
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PMID:Angiomyolipoma causing life-threatening hematuria in a child with tuberous sclerosis. 259 64


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