Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0027819 (neuroblastoma)
 27,800 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The clinicobiological feature of neuroblastoma is enigmatic because spontaneous regression often occurs in early stages of tumors of the patients under 1 year of age, while rapid growth usually occurs in the tumors of the patients over 1 year of age. Such difference in the clinical behavior may be caused by the difference in the pattern of gene expression among the subsets of neuroblastoma. To understand the molecular basis of neuroblastoma biology, we decided to identify the novel genes expressed differentially between favorable and unfavorable neuroblastomas. The oligo-capping cDNA libraries were constructed from different subsets of neuroblastomas. After random selection and DNA sequencing, the differentially expressed genes between favorable and unfavorable neuroblastomas were screened by reverse transcriptase-PCR. The clinical significance of gene expression was evaluated based on the results of Northern blot analysis. We have identified a novel gene Nbla03145 (alpha), also cloned and termed by another group as ECEL1, which encodes a new member of putative zinc-binding metalloendopeptidase (endothelin-converting enzyme) with unknown substrate. We also cloned a COOH-terminally truncated Nbla03145/ECEL1beta which is expressed only in thymus. In primary NBLs, the alpha isoform is more preferentially expressed than the beta isoform. High levels of Nbla03145/ECEL1 expression were significantly correlated with a younger age (p=0.0005), lower stages (p=0.0019), high level of TrkA expression (p</=0.00005), a single copy of MYCN (p<0.00005) and the tumors found by mass screening (p<0.00005). Decreased expression of Nbla03145/ECEL1 mRNA was significantly associated with poor prognosis (log-rank test: p=0.012). The present results have shown that expression of Nbla03145/ECEL1 is a novel prognostic marker of neuroblastoma. Further analysis of the gene may also give a cue to the understanding of the role of endothelin-like signaling in neuroblastoma and to the development of diagnostic and therapeutic strategies against aggressive tumors.
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PMID:High expression of the novel endothelin-converting enzyme genes, Nbla03145/ECEL1alpha and beta, is associated with favorable prognosis in human neuroblastomas. 1263 73

We present a casuistic revision of adrenal pathology, which was studied in our service during the period January 1977-July 2000. We reviewed 59.069 biopsies and 2.674 autopsies and we 84 cases. founded with the following findings: Primary tumors 25% Secundary tumors 51% Infectious diseases 11% Miscellaneous 12% Unsuitable for diagnosis 1% Hyperplasias, adenomas, pheochromocy-tomas, neuroblastoma, adenocarcinoma are included within primary tumors. The metastasic tumors corresponded to: lung, pancreas, mammary gland, kidney and carcinomas; endometrial adenocarcinoma, lymphoma, melanoma, hepatocarcinoma, gastric carcinoma, testicular teratocarcinoma, skin epidermoid carcinoma, uterus choriocarcinoma and a primary germinal tumor of the thymus. Within infectious diseases we founded tuberculosis, histoplasmosis, cryptococosis, hydatidosis. Miscellaneous included hematoma, hemorrhage, pseudocyst, Disseminated Intravascular Coagulation (DIC), athrophy, Wegener's granulomatosis, myelolipoma, hemorrhagic necrosis. There was only one case which was unsuitable for diagnosis due to insufficient material.
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PMID:[Casuistic revision of adrenal pathology during last 23 years]. 1293 68

Chylothorax and chylopericardium are rare conditions occurring in infants and children. Both may be traumatic or nontraumatic in origin. We reviewed our experiences with the management of sixteen pediatric cases (10 males, 6 females; 11-days to 14-years old) of chylothorax and chylopericardium from 1997 to 2003. There were fifteen cases of chylothorax (9 left, 2 right, 4 bilateral) and two cases of chylopericardium (1 isolated and 1 associated with chylothorax), and their incidences of occurrence after cardiothoracic surgery were 0.89% and 0.12%, respectively. Of the fifteen cases occurring after cardiothoracic surgery, thirteen patients had corrective or palliative surgery for complex congenital heart disease, and two patients had removal of thymolipoma and neuroblastoma, respectively. Included also in this review was an 11-day old preterm infant with hydrops fetalis and congenital heart disease who developed chylothorax. Characteristics of chylous effusion included a presence of whitish opaque fluid in the pleural cavity and the pericardial cavity, having a triglyceride content ranging from 59 to 1689 mg/dl which was higher than a plasma triglyceride, a protein content of 2.4 to 7.4 g/dl, and a presence of lymphocyte predominance. The average latent period for diagnosis of chylothorax or chylopericardium was 13 days (range 3-30). All patients were treated primarily with nutritional modification using medium-chain triglycerides (MCT) instead of long-chain triglycerides in their diet; and only a few cases needed bowel rest with total parenteral nutrition (TPN). Twelve patients completely responded to a MCT-rich diet; two cases resolved after switching to TPN and another case needed surgery for ligation of lymphatic vessels around the thymus gland. The mean duration of lymph drainage was 12.1 days (range 3-29) and the average length of time of continued conservative treatment (MCT-rich diet and TPN) was 29.8 days (range 18-47). Fourteen patients (87.25%) had good outcome, i.e. resolution from chylothorax or chylopericardium and return to normal diet. Two of the fourteen patients developed severe infections; one was diagnosed with suspected bacterial endocarditis and the other had candidemia. However, both responded well to antibacterial and antifungal drugs, respectively. One case succeeded after surgery. A case that had low compliance with dietary recommendations and required repeated placement of drainage devices died due to infection with enterococcal septicemia. Early and good compliance with MCT-rich diet is essential for achieving a favorable outcome in the management of chylothorax and chylopericardium in children.
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PMID:Management of chylothorax and chylopericardium in pediatric patients: experiences at Siriraj Hospital, Bangkok. 1592 38

Novel trinuclear complexes C23H31N6O6CuSn2Cl5 [1], C23H31N6O6CuZr2Cl5 [2], C23H31N6O6ZnSn2Cl5 [3], and C23H31N6O6ZnZr2Cl5 [4] were synthesized and characterized by spectroscopic (IR, 1H, 13C, 2D COSY, and 119Sn NMR, EPR, UV-vis, ESI-MS) and analytical methods. In complexes 1-4, the geometry of copper and zinc metal ions were described as square-based pyramidal with l-tryptophan coordinated to copper/zinc via carboxylate group while Sn/Zr was present in the hexacoordinate environment. The interaction of 1 and 2 with calf thymus DNA in Tris buffer was studied by electronic absorption titration, luminescence titration, cyclic voltammetry, circular dichroism, and viscometric measurements. The emission quenching of these complexes by [Fe(CN)6]4- depressed greatly when bound to DNA. Observed changes in the circular dichoric spectra of DNA in presence of 1 and 2 support the strong binding of complexes with DNA. The relative specific viscosity of DNA bound to 1 and 2 decreased, indicating that the complexes bind to DNA via covalent binding. The results reveal that the extent of DNA binding of 1 was greater than that of 2. To evaluate the mechanistic pathway of DNA inhibition, counting experiments and MTT assay were employed to assess the induction of apoptosis by 1. Western blot analysis of whole cell lysates and mitochondrial fractions with Bcl-2 and p-53 family proteins and caspase-3 colorimetry assay were also carried out on a human neuroblastoma cell line SY5Y.
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PMID:DNA binding studies of novel Copper(II) complexes containing L-tryptophan as chiral auxiliary: in vitro antitumor activity of Cu-Sn2 complex in human neuroblastoma cells. 1737 49

Neuroblastoma (NBL) is the most common solid tumor in children. Tumors in advanced stage or with positive risk factors still have a poor prognosis. Thy1 (CD90) is a membrane glycoprotein expressed in thymus, retinal ganglionic cells, and several types of stem cells. The aim of this study was to assess Thy1 expression in NBL and analyze the correlation with clinical outcome. Sixty-three specimens of NBL were stained for Thy1 on a tissue microarray by immunohistochemistry. Fresh frozen tumor tissues were used for RNA isolation, and RT-PCR analysis for Thy1-mRNA expression was performed. Patients' survival data were correlated with Thy1 status using a log rank test and a Cox regression multivariate analysis. Thy1 was expressed on 51 (81%) of the tumors. Kaplan-Meier survival analysis showed a significantly impaired survival in patients with NBL missing Thy1 (P < 0.005 by log-rank test). A multivariate Cox regression showed an independent prognostic value of Thy1 status for overall survival (P < 0.05). In addition, the frequency of events and deaths was significantly higher in the group of patients with Thy1 negative tumors, as assessed by ANOVA analysis (P < 0.05 by F-test). The data showed that Thy1-negative NBL patients have a significantly impaired overall survival compared with Thy1-positive NBL patients. Thus, Thy1 seemed to be a marker with a specific prognostic value in NBL patients. Future studies are aiming at the biological role of this marker in the tumor cell differentiation.
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PMID:Lack of Thy1 (CD90) expression in neuroblastomas is correlated with impaired survival. 1795 44

We describe a rare case of thymic neuroblastoma with the syndrome of inappropriate secretion of antidiuretic hormone (SIADH). A 60-year-old male patient was admitted to our hospital for further examination and treatment of anterior mediastinal tumor found at a regular health check-up. On examination there was hyponatremia, decrease in plasma osmolarity and elevation of plasma antidiuretic hormone (ADH) level. Thus, he underwent total thymectomy under the diagnosis of thymoma with SIADH. The tumor was located in the right lobe of the thymus and the final diagnosis was thymic neuroblastoma. To our knowledge, this is the first reported case of thymic neuroblastoma in which production of ADH by tumor cells is demonstrated immunohistochemically. This case highlights the need to consider functional activity of thymic neuroblastoma and complete resection of the tumor is warranted for treatment.
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PMID:Thymic neuroblastoma with the syndrome of inappropriate secretion of antidiuretic hormone. 1966 Nov 18

Presence of thymus in the normal position as well as in the posterior mediastinum is an unusual phenomenon. We report here a case of posterior mediastinal mass in a 20-month old male child who presented with dysphagia and dry cough. Investigations revealed it to be a solid posterior mediastinal mass, suspected to be either lymphoma or a neuroblastoma. Excision of the mass followed by histopathologic examination revealed the mass to be a normal thymus. This case indicates that a benign mass, an accessory thymus, though rare, can be located in the posterior mediastinum and cause symptoms similar to solid mediastinal tumors.
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PMID:Accessory thymus in posterior mediastinum. 2001 97

We report the isolation of a novel Tumor-Cells Apoptosis Factor (Nerofe). We found that cDNA of this protein is expressed mainly in the human thymus and partially in the colon and in the frontal lobe of brain. Immunohistochemical studies localize Tumor-Cells Apoptosis Factor (TCApF) to the medulla and Hassal's corpuscles of the thymus gland, which are responsible for negative selection. Treatment of mice with induced AML terminates the cancer development and completely eliminates metastatic cell colonies from the bone marrow and the spleen that reduces probability of the cancer return. We find that TCApF binds to the T1/ST2 receptor and activates caspases 8, 9 and 3 mediated apoptosis, together with activation of JNKinase and p38 MAPKinase. Application of TCApF to cells induced apoptosis in acute myeloid leukemia proliferating cells (U937 premeyloid cells), in human breast carcinoma (MCF7), human glioblastoma, human neuroblastoma, human prostate cancer and human lung cancer proliferating cells. In contrast, TCApF was unable to induce apoptosis in non-proliferating cells. The selectivity of TCApF-induced apoptosis is related to the level of T1/ST2 receptor expression. This is the first report linking the T1/ST2 receptor to apoptosis.
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PMID:NEROFE--a novel human hormone-peptide with anti-cancer activity. 2122 65

The autors report original date on the combined use of videosurgery and open surgical intervention in 3 patients. One (1 yr 10 mo) had neuroblastoma in the posterior mediastinum spreading to the retroperitoneal region, another (5 yr) presented with neuroblastoma in the thoracic aperture region spreading to the neck, the third one (14 yr) suffered limphoepithelioma-like cancer of the thymus. The combined treatment permits to optimize the surgical procedure and avoid additional use of thoraco- and laparotomy.
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PMID:[Combined use conventional and videoendosurgery in pediatric oncology]. 2271 73

Neck masses are frequently encountered in pediatric medicine, and can present a diagnostic dilemma for the clinicians involved. There are several means by which neck masses in children can be subdivided, for example by age at presentation, anatomical location including compartments and fascia of the neck, their classical appearance when imaged, or by etiology. When imaging children the clinicians must be mindful of radiation exposure and as such ultrasound (US) is often attempted first. Cross sectional imaging can be helpful for problem solving with CT being particularly useful for assessing the patient in more acute scenarios, for example when there is airway compromise. Nuclear medicine scintigraphy has a role in specific circumstances and can aid in staging in the presence of malignancy. If required, additional acquisition by means of magnetic resonance imaging (MRI) and computed tomography (CT) can be considered. This pictorial review describe the diagnostic imaging of (I) congenital and Developmental Pathologies, including thyroglossal duct cyst, branchial cleft cyst, cystic hygroma, dermoid cyst, thymic cyst and ectopic thymus; (II) neoplastic lesions, including hemangiomas and vascular malformations, pilomatrixoma, neurofibroma, neuroblastoma, rhabdomyosarcoma, papillary thyroid cancer, lymphoma & leukemia; (III) neck masses of Infective causes, including lymphadenitis, retropharyngeal and peritonsilar abscess, salivary gland inflammation; and (IV) other miscellaneous lesions, including ranula, sternocleidomastoid fibromatosis coli, and goiter. Neck masses are common in the pediatric population with a broad and varied differential; malignant etiologies are less frequently encountered when compared with adults but an awareness of its potential is important when reviewing imaging.
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PMID:Diagnostic imaging of benign and malignant neck masses in children-a pictorial review. 2794 80


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