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 detection of brain tumors using standard techniques of qualitative, relaxation-weighted magnetic resonance imaging (MRI) requires the application of contrast agents. We investigated whether or not it is possible to use diffusion-weighted MRI to localize tumors without contrast enhancement. Three different experimental rat brain tumors were studied: F98 glioma, RN6 Schwannoma and E376 neuroblastoma. We found a marked hypointensity in the region of the tumor and edema in heavily diffusion-weighted images, which corresponded well with the histological presentation. Quantitative maps of the apparent diffusion coefficient (ADC) allowed a better localization of the tumor than that obtained by regional presentation of T2 times, particularly under conditions in which peritumoral edema was absent. The ADC differences of the three tumor types were statistically not significant. Based upon regions-of-interest evaluations, tumor could be distinguished from peritumoral edema and normal brain tissue. However, a sharp demarcation between tumor and peritumoral edema was not possible, and this is attributed to a similar enlargement of interstitial space. It was concluded that diffusion-weighted MRI possesses a high potential for the detection of brain tumors but does not allow precise demarcation of the tumor border.
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PMID:Quantitative diffusion MR imaging of cerebral tumor and edema. 797 85

The potential of quantitative parameter images of the relaxation times T1 and T2, the proton density rho and the apparent diffusion coefficient (ADC) to characterize three different experimental rat brain tumors (F98 glioma, RN6 Schwannoma, and E376 neuroblastoma) was studied. All parameter values, as determined in histologically confirmed regions of interest (ROI), were higher in edema than in tumor, which in turn were elevated with respect to normal brain. ROI values of ADC and T2 delivered statistically significant (P < 0.01) differentiation between tumor and edema. Multidimensional parameter combinations improved differentiation between different tissues. However, the three tumor types could not be differentiated. All parameter maps allowed the identification of the whole tumor-edema area. On T2 images, edema could be identified best, whereas the tumor itself was hardly visualized. In many cases, tumor presentation using T1 maps corresponded best with histology, nevertheless suffering from a poor tumor-edema differentiation.
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PMID:High resolution quantitative relaxation and diffusion MRI of three different experimental brain tumors in rat. 859 10

Glia cell line-derived neurotrophic factor (GDNF), a recently cloned member of the transforming growth factor-beta (TGF-beta) superfamily, has been implicated in the survival, morphological and functional differentiation of midbrain dopaminergic neurons and motoneurons in vitro and in vivo. The factor may thus have utility in the treatment of various human neurodegenerative disorders. Mechanisms regulating expression of GDNF in normal and diseased brain as a possible means to increase the local availability of GDNF are only beginning to be explored. We have established and employed a competitive reverse transcriptase-polymerase chain reaction (RT-PCR) to study and compare levels of expression of GDNF mRNA in several cell types and to investigate its regulation. GDNF expression was clearly evident in primary cultured astrocytes, the glioma B49 and C6 cell, but less pronounced in the Schwannoma RN22 cell lines. Little or no signal could be observed in neuroblastoma cell lines (IMR32, LAN-1) or the pheochromocytoma cell line PC12, emphasizing the glial character of this factor. Using the C6 cell line we found that fibroblast growth factor-2 (FGF-2; bFGF) can increase GDNF mRNA levels, whereas FGF-1, platelet-derived growth factor (PDGF), and vasoactive intestinal polypeptide (VIP) are apparently ineffective. Several other factors (forskolin, kainic acid, triiodothyronine dexamethasone, GDNF, TGF-beta 1, and interleukin-6) appear to have slightly negative effects on GDNF mRNA levels at the concentrations tested. To further explore the relationship between FGF-2 and GDNF, we also addressed the question whether GDNF, like FGF-2, may have an effect on C6 cell proliferation. We conclude that (1) glial and glial tumor cells, rather than neuronal cell lines, express GDNF, (2) that FGF-2 has a prominent inductive effect on GDNF expression and (3) that GDNF stimulates C6 cell proliferation. Finally, these data suggest that neurotrophic actions of FGF-2 in mixed glial-neuronal cell cultures might be mediated in part by GDNF.
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PMID:GDNF mRNA levels are induced by FGF-2 in rat C6 glioblastoma cells. 888 50

Lymphoma, mediastinal cysts, and neurogenic neoplasms are the most common primary middle and posterior mediastinal tumors. Lymphoma may involve the anterior, middle and/or posterior mediastinum, frequently as lymphadenopathy or as a discrete mass. Foregut cysts are common congenital mediastinal cysts and frequently arise in the middle mediastinum. Pericardial cysts are rare. Schwannoma and neurofibroma are benign peripheral nerve neoplasms, represent the most common mediastinal neurogenic tumors, and rarely degenerate into malignant tumors of nerve sheath origin. Sympathetic ganglia tumors include benign ganglioneuroma and malignant ganglioneuroblastoma and neuroblastoma. Lateral thoracic meningocele is a rare cause of a posterior mediastinal mass.
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PMID:Primary mediastinal tumors: part II. Tumors of the middle and posterior mediastinum. 1008 19

Neurogenic tumors of the neck occur in children and adults. Important parameters to aid in the differential diagnosis are age at presentation, location, and a history of NF or multiple endocrine neoplasia. Schwannoma is the most common solitary neurogenic tumor in the neck and is usually seen in patients between 20 and 50 years of age. The plexiform neurofibroma and multiple localized neurofibromas are characteristic of NF1. MPNSTs are uncommon aggressive lesions that can arise de novo in patients with NF (10% to 30%) and postirradiation. Neuroblastic tumors consist of neuroblastoma, ganglioneuroblastoma, and ganglioneuroma. These tumors typically arise in the chest and abdomen but occasionally present as a primary neck mass. A neck mass with a histologic diagnosis of neuroblastoma is, however, more commonly metastatic from an abdominal neuroblastoma.
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PMID:Neurogenic tumors of the neck. 1105 70

We report a case of schwannoma (neurilemmoma) predominantly composed of small cells arranged in rosettes around central collagenous cores and discuss the differential diagnosis of this unusual variant. Schwannoma with giant rosettes, previously designated as neuroblastoma-like schwannoma by Goldblum et al., must be differentiated from neuroblastoma, peripheral neuroectodermal tumors, and malignant change in a schwannoma.
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PMID:Neuroblastoma-like schwannoma: a case report and review of the literature. 1254 97

Schwannoma and neurofibroma account for the majority of cutaneous benign peripheral nerve sheath tumors and usually pose little diagnostic difficulty in their classic forms. In rare instances, however, benign peripheral nerve sheath tumors may display epithelioid morphology and lack otherwise usual features of schwannoma or neurofibroma, making classification difficult. These unusual changes may prompt consideration of other benign neoplasms or a malignancy. Benign epithelioid peripheral nerve sheath tumor (BEPNST) is a somewhat non-specific term recently proposed to describe these neoplasms of imprecise histogenesis. Also diagnostically challenging, rare BEPNST with unusual arrangements of extracellular collagen have been described and reported as neuroblastoma-like schwannoma and collagenous spherulosis. We report a unique case of cutaneous BEPNST with a peculiar arrangement of abundant extracellular collagen, different than the previously observed patterns. Specifically, the neoplastic cells in this tumor were nearly obscured by the collagen, which formed large nodules and compressed the majority of the few remaining tumor cells to the periphery of the lesion. This excessive collagen production emphasizes the importance of adequate sampling to ensure a correct diagnosis.
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PMID:Collagen-rich variant of benign epithelioid peripheral nerve sheath tumor of the skin. 1819 Apr 49

We report a rare case of a 45-year-old African American woman with Neuroblastoma-like Schwannoma (Neurilemmoma) occurring in the posterior mediastinum as a pleural-based mass noted on computed tomography (CT) scan. A CT-guided core biopsy of the mass was performed and core biopsy imprints were prepared during the procedure. A Diff-Quik stain was performed for on-site evaluation of specimen adequacy. The hematoxylin and eosin (H&E) staining was evaluated subsequently. Immunohistochemistry panels were applied to the same specimen. The cytologic findings of the core biopsy imprints showed hypercellular smears with a predominance of small cells with atypical features including hyperchromatic, round nuclei and occasional nucleoli. Neurocytic rosettes were particularly appreciated on H&E stain. The immunohistochemical results exhibited strong and diffuse immunoreactivity for S-100 and vimentin. This case enriches the cytopathology literature by providing awareness of this tumor presenting as a posterior mediastinal mass.
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PMID:Pleural-based neuroblastoma-like schwannoma: a case report with cytologic findings and review of literature. 2594 Jan 1

Sarcomas are malignancies of mesenchymal origin that occur in bone and soft tissues. Many are chemo- and radiotherapy resistant, thus conventional treatments fail to increase overall survival. Natural Killer (NK) cells exert anti-tumor activity upon detection of a complex array of tumor ligands, but this has not been thoroughly explored in the context of sarcoma immunotherapy. In this study, we investigated the NK cell receptor/ligand immune profile of primary human sarcoma explants. Analysis of tumors from 32 sarcoma patients identified the proliferative marker PCNA and DNAM-1 ligands CD112 and/or CD155 as commonly expressed antigens that could be efficiently targeted by genetically modified (GM) NK cells. Despite the strong expression of CD112 and CD155 on sarcoma cells, characterization of freshly dissociated sarcomas revealed a general decrease in tumor-infiltrating NK cells compared to the periphery, suggesting a defect in the endogenous NK cell response. We also applied a functional screening approach to identify relevant NK cell receptor/ligand interactions that induce efficient anti-tumor responses using a panel NK-92 cell lines GM to over-express 12 different activating receptors. Using GM NK-92 cells against primary sarcoma explants (n = 12) revealed that DNAM-1 over-expression on NK-92 cells led to efficient degranulation against all tested explants (n = 12). Additionally, NKG2D over-expression showed enhanced responses against 10 out of 12 explants. These results show that DNAM-1+ or NKG2D+ GM NK-92 cells may be an efficient approach in targeting sarcomas. The degranulation capacity of GM NK-92 cell lines was also tested against various established tumor cell lines, including neuroblastoma, Schwannoma, melanoma, myeloma, leukemia, prostate, pancreatic, colon, and lung cancer. Enhanced degranulation of DNAM-1+ or NKG2D+ GM NK-92 cells was observed against the majority of tumor cell lines tested. In conclusion, DNAM-1 or NKG2D over-expression elicited a dynamic increase in NK cell degranulation against all sarcoma explants and cancer cell lines tested, including those that failed to induce a notable response in WT NK-92 cells. These results support the broad therapeutic potential of DNAM-1+ or NKG2D+ GM NK-92 cells and GM human NK cells for the treatment of sarcomas and other malignancies.
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PMID:Boosting Natural Killer Cell-Mediated Targeting of Sarcoma Through DNAM-1 and NKG2D. 3208 16


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