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

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.
 ...
PMID:GDNF mRNA levels are induced by FGF-2 in rat C6 glioblastoma cells. 888 50

A retrospective review of 16 patients operated upon for primary mediastinal tumours was carried out. Anterior mediastinal tumours seen included retrosternal goitre (2), benign cystic teratoma (1), benign thymoma (1), malignant thymoma, spindle cell type (1) and Hodgkin's lymphoma, nodular sclerosing type (1). Mid-mediastinal tumours included bronchial cyst (1), mediastinal granuloma (1), and pulmonary arterio-venous fistula (1). Neurilemmoma (2), neuroblastoma (1), ganglioneuroma (1), Askin tumour (1), neurofibroma (1) and benign histiocytoma (1) constituted the tumours of the posterior mediastinum. The tumours were successfully resected with minimal operative morbidity and no mortality.
 ...
PMID:Primary mediastinal tumours in the southern region of Saudi Arabia. 901 77

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.
 ...
PMID:Primary mediastinal tumors: part II. Tumors of the middle and posterior mediastinum. 1008 19

This is a comprehensive immunohistochemical study of selected archival tumors of the nervous system applying human anti-neuronal nuclear autoantibodies of types 1 and 2 (ANNA-1 and -2), serum markers of paraneoplastic syndromes associated primarily with small cell lung cancer (SCLC). Neither ANNA-1 nor ANNA-2 bound to glial tumors regardless of histological grade and subtype; instead they labeled neurons in overrun normal parenchyma. Central neurocytomas and the neuronal components of mixed glioneuronal tumors were also immunoreactive for both. In addition, varying proportions of tumor cells were stained in dysembryoplastic neuroepithelial tumor, subependymal giant cell astrocytoma (SEGA), tuber and neuroblastoma. All other tumors were nonreactive, namely choroid plexus papilloma, pituitary adenoma, pineocytoma, pheochromocytoma, thymic and pulmonary carcinoid, chordoma, meningioma, schwannoma and metastatic melanoma. SCLC was immunonegative for ANNA-1 and ANNA-2 in paraffin preparations, but displayed strong immunoreactivity for both in frozen sections: this discrepancy was not observed in other tumors studied. In conclusion, the human IgG autoantibodies ANNA-1 and ANNA-2 provide novel tools for studying the cytogenesis of tumors of the nervous system in that they permit the identification of both normal and neoplastic, poorly differentiated and small neuronal cells that may escape detection using commercially available anti-neuronal antibodies.
 ...
PMID:Anti-neuronal nuclear autoantibodies, types 1 and 2: their utility in the study of tumors of the nervous system. 979 96

Neurilemomas and neurofibromas appear as round soft tissue masses at CT. Variable enhancement with either homogeneity or heterogeneity is seen in neurilemomas. Attenuation of the tumors on enhanced CT depends on histology: the extent of Antoni A or B tissue and the amount of myxoid or cystic degeneration or hemorrhage. Neurofibromas are usually homogeneous low attenuation lesions on unenhanced CT. They show homogeneous enhancement or early central blush on enhanced scan. The extent of enhancement depends on the proportions of tumor components: nerve sheath cells, collagen bundles, and areas of myxoid degeneration. Malignant nerve sheath tumors show variable attenuation. Bony destruction, pleural effusion, and metastatic pulmonary nodules may also occur. Ganglioneuromas appear as oblong homogeneous low attenuation lesions on both enhanced and unenhanced CT. This low attenuation is due to their pathologic components, with an abundant amount of myxoid matrices and a relatively small amount of ganglion cells. Neuroblastomas appear as aggressive soft tissue lesions with calcification. Ganglioneuroblastomas may appear with features in between those of ganglioneuromas and neuroblastomas. The typical location of the posterior mediastinum or the aortopulmonary window and high enhancement with administration of contrast medium at CT suggest the diagnosis of paragangliomas.
 ...
PMID:Spectrum of neurogenic tumors in the thorax: CT and pathologic findings. 1034 46

The coexistence of pheochromocytoma and other tumor types in a single adrenal gland has been rarely documented. This type of pheochromocytoma is designated "composite" or "mixed," depending on whether the pheochromocytoma and the nonpheochromocytoma components show the same embryologic origin. The nonpheochromocytoma components reported in the composite pheochromocytoma include ganglioneuroma, ganglioneuroblastoma, neuroblastoma, and malignant schwannoma. The components found in the mixed pheochromocytoma include adrenal cortical neoplasms and spindle cell sarcoma. We report a unique case of composite pheochromocytoma in which the nonpheochromocytoma element is a neuroendocrine carcinoma. The histologic and the immunohistochemical profiles of the 2 distinct components of this tumor were typical for those of pheochromocytoma and neuroendocrine carcinoma. This dual differentiation was also supported by ultrastructural findings. This case not only broadens the morphologic spectrum of composite pheochromocytoma but also provides some additional insight into the histogenesis of this rare but fascinating type of tumor.
 ...
PMID:Pheochromocytoma associated with neuroendocrine carcinoma. A new type of composite pheochromocytoma. 1058 35

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.
 ...
PMID:Neurogenic tumors of the neck. 1105 70

Inactivating germline mutations of the novel putative tumor-suppressor gene LKB1/STK11 at 19p13.3 have been shown to cause Peutz-Jeghers syndrome (PJS), an autosomal dominantly inherited disease characterized by a predisposition to mucocutaneous pigmentations, as well as various benign and malignant neoplasms. To elucidate the role of LKB1/STK11 in the carcinogenesis of primary and secondary human brain tumors, a total of 309 tumors were analyzed for loss of heterozygosity (LOH) at microsatellite loci D19S886, DI9S878, and D19S565. Low LOH rates were observed for glioma (17.3%, n = 139), meningioma (5.3%, n = 57), schwannoma (0%, n = 21), pituitary adenoma (18.8%, n = 16), primary CNS lymphoma, neuroblastoma, plasmocytoma, medulloblastoma, germinoma, and papilloma of the choroid plexus (6.6%, n = 15). In contrast, brain metastases exhibited a mean LOH frequency of 42.6% (n = 61), with breast (56.3%) and lung cancer metastases (58.3%) being most frequently affected. Genomic DNA sequencing of the complete coding region of LKB1/STK11 was performed in all brain metastases exhibiting LOH (n = 26); no mutation was revealed, but we did find a germline mutation in a PJS patient. Despite high LOH fiequencies at the 19p13.3 locus in carcinoma metastases to the brain and occasional mutations reported for certain primary carcinomas, there are no mutations in LKB1/STK11. This fact suggests that alterations of LKB1/STK11 occur relatively early in tumorigenesis and are rarely involved in the development of carcinoma metastases. Based on these findings, the genes adjacent to LKB1/STK11 may be relevant for the development of metastases to the brain from certain carcinomas.
 ...
PMID:Frequent loss of heterozygosity at the 19p13.3 locus without LKB1/STK11 mutations in human carcinoma metastases to the brain. 1121 97

We report five new cases of rhabdomyosarcoma (RMS) in Costello syndrome. These cases, combined with those previously reported, increase the number of solid tumors to 17 (10 RMSs, 3 neuroblastomas, 2 bladder carcinomas, 1 vestibular schwannoma, 1 epithelioma), in at least 100 known Costello syndrome patients. Despite possible ascertainment bias, and the incomplete identification of all Costello syndrome patients, the tumor frequency could be as high as 17%. This is comparable to the 7-21% frequency of solid tumors in Beckwith-Wiedemann syndrome (BWS), and may justify tumor screening. Based on the recommendations for screening BWS patients, we propose a screening protocol consisting of ultrasound examination of the abdomen and pelvis every 3-6 months until age 8-10 years for RMS and abdominal neuroblastoma; urine catecholamine metabolite analysis every 6-12 months until age 5 years for neuroblastoma; and urinalysis for hematuria annually for bladder carcinoma after age 10 years. These recommendations may need to be modified, as new information becomes available. Potential criticism of the tumor screening protocol concerns the lack of evidence for improved outcome, and possible overestimation of the tumor risk. The ability of RMSs to occur at various sites complicates tumor screening, but 8 of the 10 RMSs in Costello syndrome patients originated from the abdomen, pelvis and urogenital area. Prior diagnosis of Costello syndrome is a prerequisite for the implementation of any screening protocol. The diagnosis of Costello syndrome should also be considered in individuals with RMS and physical findings suggestive of Costello syndrome.
 ...
PMID:Five additional Costello syndrome patients with rhabdomyosarcoma: proposal for a tumor screening protocol. 1269 63

In the publication by the World Health Organization of the histological classification of central nervous system tumors in 1993, several new tumor types were added, including pleomorphic xanthoastrocytoma (PXA), dysembryo-plastic neuroepithelial tumor (DNT), and desmoplastic infantile ganglioglioma (DIG). Referring to these newly classified tumors of the brain, the present article describes the neuroradiological findings of relatively rare tumors of the brain such as PXA, subependymoma, gangliocytoma, dysplastic cerebellar gangliocytoma (Lhermitte-Duclos disease), DIG, central neurocytoma (CN), neuroblastoma, desmoplastic medulloblastoma, atypical teratoid/rhabdoid tumor (ATRT), glossopharyngeal schwannoma, and malignant fibrous histiocytoma (MFH). PXA and DIG affect the cerebral hemisphere and appear to be cystic masses with a solid component on CT and MRI. Gangliocytomas commonly occur in the floor of the third ventricle and the temporal lobe. CT and MRI typically show a mass lesion with no vasogenic edema. In Lhermitte-Duclos disease, T2-weighted MR images reveal characteristic enlarged folia. Desmoplastic medulloblastomas tend to favor the cerebral hemisphere in the adolescent. ATRTs are rarely seen in infants or children and resemble medulloblastomas or primitive neuroectodermal tumors. Intracranial MFHs are also rare tumors and sometimes resemble the meningiomas.
 ...
PMID:[Neuroradiological findings of relatively rare tumors of the brain]. 1223 62


<< Previous 1 2 3 4 5 6 7 8 9 Next >>