Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0035412 (rhabdomyosarcoma)
 6,156 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The transcriptional activity of plasmid pCOL-KT, in which human pro alpha 1 (I) collagen gene upstream sequences up to -804 and most of the first intron (+474 to +1440) drive expression of the chloramphenicol acetyltransferase (CAT) gene [Thompson, Simkevich, Holness, Kang & Raghow (1991) J. Biol. Chem. 266, 2549-2556], was tested in a number of mesenchymal and non-mesenchymal cells. We observed that pCOL-KT was readily expressed in fibroblasts of human (IMR-90 and HFL-1), murine (NIH 3T3) and avian (SL-29) origin and in a human rhabdomyosarcoma cell line (A204), but failed to be expressed in human erythroleukaemia (K562) and rat pheochromocytoma (PC12) cells, indicating that the regulatory elements required for appropriate tissue-specific expression of the human pro alpha 1 (I) collagen gene were present in pCOL-KT. To delineate the nature of cis-acting sequences which determine the tissue specificity of pro alpha 1 (I) collagen gene expression, functional consequences of deletions in the promoter and first intron of pCOL-KT were tested in various cell types by transient expression assays. Cis elements in the promoter-proximal and intronic sequences displayed either a positive or a negative influence depending on the cell type. Thus deletion of fragments using EcoRV (nt -625 to -442 deleted), XbaI (-804 to -331) or SstII (+670 to +1440) resulted in 2-10-fold decreased expression in A204 and HFL-1 cells. The negative influences of deletions in the promoter-proximal sequences was apparently considerably relieved by deleting sequences in the first intron, and the constructs containing the EcoRV/SstII or XbaI/SstII double deletions were expressed to a much greater extent than either of the single deletion constructs. In contrast, the XbaI* deletion (nt -804 to -609), either alone or in combination with the intronic deletion, resulted in very high expression in all cells regardless of their collagen phenotype; the XbaI*/(-SstII) construct, which contained the intronic SstII fragment (+670 to +1440) in the reverse orientation, was not expressed in either mesenchymal or nonmesenchymal cells. Based on these results, we conclude that orientation-dependent interactions between negatively acting 5'-upstream sequences and the first intron determine the mesenchymal cell specificity of human pro alpha 1 (I) collagen gene transcription.
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PMID:The transcriptional tissue specificity of the human pro alpha 1 (I) collagen gene is determined by a negative cis-regulatory element in the promoter. 152 Feb 67

Between 1981 and May 1986 31 children with solid abdominal tumor masses were observed in our clinic. The first diagnostic procedure was a sonographic examination, followed by further radiological investigations if necessary. 30 cases were examined histologically; in one case the sonographic findings were confirmed by an angiography. The most frequent abdominal masses were neuroblastomas and Wilms tumors (7 cases each). A mesoblastic nephroma was diagnosed in 3 cases, a lymphoma, a hepatoblastoma and a rhabdomyosarcoma 2 times each. One time we found a pancreas carcinoma, a teratoma, a hemangiomatosis of the liver, a malignant Schwannoma, a Ewing sarcoma, an adenoma of the adrenal gland, a pheochromocytoma and an osteosarcoma. According to our own experience and recent reports in the literature it seems possible in most cases, to predict the correct diagnosis of solid abdominal masses using the informations of sonographic imaging. Sonography is a highly specific non-invasive diagnostic tool for planning treatment (e.g. early surgery, cytostatic therapy and/or radiation) of solid abdominal masses. Nevertheless the histological examination should be performed in every case to confirm the definitive diagnosis.
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PMID:[Sonographic diagnosis of solid space-occupying abdominal lesions in childhood]. 303 88

While magnetic resonance imaging (MRI) is not a screening examination in the search for metastatic disease, it has already demonstrated its value when performed as a carefully directed study to detect focal hepatic lesions. In the past 18 months we have encountered nine patients (six men, three women--ages 14-66) with a variety of primary tumors (renal cell carcinoma, two; colon carcinoma, two; hepatocellular carcinoma, two; pancreatic carcinoma, one; pheochromocytoma, one; rhabdomyosarcoma, one) whose MR scans revealed hepatic metastases after normal or nondiagnostic computed tomographic (CT) scans. The lesions were most clearly demonstrated using spin echo (SE) technique with recovery times (TR) of 2.0 seconds and delay times (TE) of 56 msec; metastases usually imaged with greater signal intensity than surrounding liver parenchyma. Lesions were solitary in three patients, multiple in six, and were found in all hepatic lobes without any specific location predominating. Pathologic confirmations of MR diagnoses were available in six of nine patients. In two patients, CT scans were of poor technical quality because streak artifacts from surgical clips obscured portions of the liver. In one patient iodinated contrast medium could not be given. In the remaining six patients CT scan quality was acceptable. This report is not a comparison study of the two modalities. We simply present nine patients in whom metastases were not detected by CT for various reasons but were found by MRI using SE technique.
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PMID:Magnetic resonance imaging diagnosis of hepatic metastases in the presence of negative CT studies. 408 52

We have extended our analysis of human tumors using antibodies specific for each of the five types of intermediate filaments to neuroblastoma, ganglioneuroblastoma, pheochromocytoma, ependymoblastoma, and alveolar soft part sarcoma. Tumor cells in the three cases of neuroblastoma, as well as in the single case of alveolar soft part sarcoma, did not react positively with sera directed against any of the five intermediate filament types. We suppose, therefore, that neuroblastoma at least may be derived from a cell type - possibly present in peripheral neurones - which in vivo has very few or no intermediate filaments. In ganglioneuroblastoma and in pheochromocytoma the tumor cells were positive when tested with antibodies directed against neurofilaments and negative with those directed against other intermediate filament types. The ependymoblastoma was positive when tested with antibodies directed against glial fibrillary acidic protein (GFA) and negative when tested with antibodies against other intermediate filament types. Use of antibodies to the different intermediate filament types appears to be a valid way in which to classify tumors, and so far the data presented here and elsewhere support the hypothesis that tumor cells retain the intermediate filament type typical of their cell of origin. Wider use of these sera would seem particularly useful in cases such as neuroblastoma, rhabdomyosarcoma or lymphoma where diagnosis is currently difficult using conventional histological stains.
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PMID:Various sympathetic derived human tumors differ in neurofilament expression. Use in diagnosis of neuroblastoma, ganglioneuroblastoma and pheochromocytoma. 612 32

Three enolase isozymes (alpha alpha, alpha gamma, and gamma gamma) and S-100 protein in the extract of neuroendocrine tumors (neuroblastoma, ganglioneuroblastoma, ganglioneuroma, and pheochromocytoma) and nonneuroendocrine tumors (Wilms' tumor, rhabdomyosarcoma, and hepatoblastoma) were determined by means of enzyme immunoassay systems. All of the tumors examined showed a high level of alpha alpha-enolase (1.71 to 19.0 micrograms/mg protein). Levels of nervous system-specific enolases (NSE; alpha gamma and gamma gamma) in the neuroendocrine tumors were also rather high (alpha gamma, 1.64 to 7.45 micrograms/mg protein; gamma gamma, 0.052 to 5.56 micrograms/mg protein). However, the NSE concentration in the extract of nonneuroendocrine tumors was low (alpha gamma, less than 0.88 micrograms/mg protein; gamma gamma, 0 microgram/mg protein). The level of S-100 protein was relatively high in ganglioneuroma (greater than 500 ng/mg protein) and ganglioneuroblastoma (greater than 100 ng/mg protein), but low in neuroblastoma (less differentiated neuroendocrine tumor) and nonneuroendocrine tumors. Serum levels of enolase isozymes were also determined in neuroblastoma patients before and after resection of primary tumor or effective chemotherapy. The elevated level of serum NSE (alpha gamma and gamma gamma) was markedly decreased with little change in the alpha alpha level by the treatment.
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PMID:Determination of three enolase isozymes and S-100 protein in various tumors in children. 631 26

Computed tomographic (CT) equipment capable of high-resolution, rapid-sequence scanning allows detection of intracardiac and intrapericardial masses. Two patients with intrapericardial masses (pheochromocytoma, organized hematoma) and three patients with intracardiac masses (right ventricular rhabdomyosarcoma, right atrial metastasis, and left atrial thrombus) are presented. CT is the imaging method of choice for displaying pericardial masses directly and may be superior to echocardiography and angiocardiography in the detection of ventricular thrombi. In patients with cardiac tumors, CT evaluates extent of disease including invasion of contiguous vessels and pulmonary metastases better than echocardiography. Dynamic scanning after bolus intravenous injection of contrast material is recommended for the evaluation of patients with suspected masses involving the heart or pericardium.
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PMID:CT of intracardiac and intrapericardial masses. 660 31

Only about 2% of the urinary tract are not of urothelial origin. Our knowledge of their morphology and biology is mainly based on single case reports, and therefore apart from a few exceptions very poor. Generally, the most often affected site is the urinary bladder (79.2%), followed by the urethra (12.7%), pelvis (4.9%) and ureter (3.2%). The urinary bladder also is the only organ in which all different histological types of these tumors were described. According to their histogenesis non-urothelial tumors (NUT) can be classified by the following main groups: soft tissue tumors, mixed epithelial and non epithelial tumors (carcinosarcomas), neuroendocrine carcinomas, carcinoids, malignant lymphomas, malignant melanomas and extragonadal germ cell tumors. Moreover some very interesting tumor-like lesions, like malakoplakia and inflammatory pseudosarcoma, mainly occur in this region. About 75% of all NUT of the urinary tract belong to the soft tissue tumors. Rhabdomyosarcomas in children and leiomyomas and -myosarcomas in adults are the more frequent histological types. Leiomyosarcomas can easily be confused with other tumor types or even with inflammatory pseudotumors. The use of immunohistochemistry to achieve a correct diagnosis is mandatory but not always successful. A relatively frequent tumor occurring in the bladder of young adults is the paraganglioma (pheochromocytoma), which usually produces typical symptoms of catecholamine excess. Carcinosarcomas of the urinary bladder contain both epithelial and mesenchymal components. They have to be distinguished from collision tumors (coexistent but separate carcinoma and sarcoma), spindle cell transitional carcinomas as well as from carcinomas with osseous or cartilaginous metaplasia. Carcinoids and neuroendocrine carcinomas developed from the neuroendocrine cells scattered all over the transitional epithelium of the bladder. Neuroendocrine carcinomas of the bladder are also called "oat cell carcinomas" since they show the same histological features and immunoreactivity as the oat cell carcinomas of the lung. They share also the same poor prognosis. The affection of the urinary tract in generalized malignant lymphomas and leukemias occur in more than 30% of cases. Lymphomas, primarily localised in the urinary bladder are, however, extremely rare. The most frequent ones are low grade non Hodgkin lymphomas, although 3 cases of Hodgkin disease and a few cases of primary extramedullary plasmacytoma of the bladder have been reported, too.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:[Non-urothelial tumors of the urinary tract]. 751 Dec 78

The distribution of the neural cell adhesion molecule (N-CAM; CD56) was studied immunohistochemically in acetone-fixed frozen sections of 83 soft tissue tumors and selected normal mesenchymal tissue using Leu-19 monoclonal antibody and avidin-biotin peroxidase immunostaining. Positive N-CAM immunostaining was found in gastrointestinal and uterine cells but not in vascular smooth muscle cells. Normal skeletal muscle was negative, but atrophic muscle fibers within tumors were positive. Strong and consistent immunoreactivity was seen in nerves, pheochromocytoma, malignant schwannoma, and spindle cells, but not in epithelial-like cells of synovial sarcoma, hemangiopericytoma, benign leiomyoma, and rhabdomyosarcoma. Variable staining was seen in benign schwannoma and malignant fibrous histiocytoma. Limited, usually focal N-CAM immunoreactivity was seen in desmoid tumor, dermatofibrosarcoma, and leiomyosarcoma. Although the N-CAM is consistently seen in neural tumors, it is not cell lineage specific. Changes on malignant transformation (comparing corresponding benign and malignant lesions) do not show any consistent pattern. Rather, there is neoexpression of the N-CAM in rhabdomyosarcoma, whereas there is loss of the N-CAM in leiomyosarcoma. Immunohistochemistry of the N-CAM can be a useful adjunct for characterization of soft tissue tumors, and its possible correlation with tumor behavior has to be examined in further studies.
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PMID:Neural cell adhesion molecule distribution in soft tissue tumors. 767 94

The 14 tumors reported in Rubinstein-Taybi syndrome since 1989, when added to the 22 previously reported, are beginning to show a pattern of neural and developmental tumors, especially of the head, which is malformed in the syndrome. Among the neoplasms were 12 of the nervous system: 2 each of oligodendroglioma, medulloblastoma, neuroblastoma, and benign meningioma, a pheochromocytoma, and 3 other benign tumors; 2 of nasopharyngeal rhabdomyosarcoma; and 1 each of leiomyosarcoma, seminoma, and embryonal carcinoma. Among the other benign tumors were an odontoma, a choristoma, a dermoid cyst, and 2 pilomatrixomas.
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PMID:Tumors in Rubinstein-Taybi syndrome. 955 2

Radioiodinated meta-iodobenzylguanidine (131I-MIBG) has been widely used for the diagnosis of neuroblastomas, pheochromocytomas, paragangliomas and medullary carcinomas of thyroid. We have developed a procedure for preparation of 131I-MIBG and studied its utility in diagnosis of primary and metastatic neural crest tumours. Studies were carried out in 54 patients. Of them 39 cases were of neuroblastomas, 1 pheochromocytoma; 6 operated medullary carcinomas; 5 paragangliomas; 2 Ewing's sarcoma and 1 Rhabdomyosarcoma; The sensitivity for the detection of primary tumours of neuroblastomas was 94% and for the detection of metastasis was 83%; while in the case of paragangliomas and medullary carcinoma, the sensitivity was 75% and 70% respectively. Our experience in the present study shows that 131I-MIBG scintigraphy is a sensitive and specific diagnostic tool to localise primary and metastatic disease of neural crest tumours.
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PMID:131I-MIBG scintigraphy in neural crest tumours. 792 41


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