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)

Two hundred and two benign and malignant soft tissue lesions were studied for the presence of S-100 protein by means of the peroxidase-antiperoxidase technique on formalin-fixed, paraffin-embedded tissue. Virtually all benign nerve sheath tumors (neurofibroma, neurilemoma, and granular cell tumor) contained numerous immunoreactive S-100-positive cells. Only one-half (18 of 36) of malignant schwannomas contained the protein, suggesting that its presence is an expression of differentiation in Schwann cell tumors. S-100 protein was not identified within pure neuroblastic tumors (neuroblastoma, neuroepithelioma) but could be identified within rare cells of the ganglioneuroblastoma and within the Schwann cell component of ganglioneuroma. It was also identified within most melanocytic tumors (cellular blue nevus, clear cell sarcoma, and melanoma). In fact, its constant presence in melanoma indicates that it may prove to be an independently reliable method for diagnosing amelanotic forms. It is also sporadically present within a variety of mesenchymal lesions including lipoma, liposarcoma, synovial chondromatosis, chondrosarcoma, fibromatosis, histiocytosis X, and chordoma. Although S-100 protein is highly characteristic of neural crest-derived tumors, it is not restricted to them and, consequently, must be interpreted cautiously. It may prove helpful in select situations such as the distinction of (a) benign nerve sheath tumors from other benign mesenchymal tumors such as fibrous histiocytomas, (b) cellular neurilemomas from malignant schwannomas, (c) malignant schwannomas from conventional fibrosarcoma (d) malignant melanomas from many carcinomas, and, possibly (e) juvenile xanthogranulomas from histiocytosis X.
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PMID:Value of S-100 protein in the diagnosis of soft tissue tumors with particular reference to benign and malignant Schwann cell tumors. 631 Feb 27

Ectopic intrathoracic kidney is an extremely rare congenital disorder. It is generally asymptomatic, being discovered as incidental finding on a routine chest radiograph. Diagnosis is established by an intravenous pyelogram demonstrating an abnormally shaped excretory system with longer than usual ureters, which also helps to differentiate it from other posterior mediastinal tumors, such as neurogenic masses, including neuroblastoma, ganglioneuroma, neurofibroma, neuroenteric cysts, meningoceles and hernia of the foramen of Bochdalek.
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PMID:[Congenital intrathoracic right kidney]. 649 97

Immunohistochemical analysis of five paraffin wax-embedded neoplasms was performed to elucidate the characteristics of bovine nervous-tissue tumours. In case 1 (peripheral neuroblastoma), the neoplastic tissue was characterized by the formation of true and Homer-Wright rosettes and the existence of neuron-specific enolase. The neoplastic cells were possibly more immature than those of common neuroblastomas, because similar features are observed in human malignant neuroepitheliomas. The neoplastic cells in case 2 (ganglioneuroblastoma) ranged from large cells with abundant neurofilaments to immature small cells, rarely with neurofilaments or glial fibrillary acidic protein (GFAP). Such expression suggests the presence of pluripotential cells. The neoplastic tissue in case 3 (anaplastic ganglioglioma) was strikingly polymorphous, and had five elements; neuronal, astrocytic, oligodendrocytic, spindle cell and small oval cell. The neoplastic neurocytes and astrocytes were, respectively, characterized by neurofilament and GFAP positivity. The neoplastic oligodendrocytes made a honeycomb appearance, and the neoplastic spindle cells and small oval cells were considered to be less differentiated. The tumours of cases 2 and 3, which contained poorly differentiated cells and revealed both neuronal and glial differentiation, may be specific to calves. In case 4 (schwannoma), almost all the neoplastic cells were positive for S100 protein, while S100-negative fibroblasts were present in many areas of case 5 (neurofibroma). These two tumours were readily distinguished histologically and immunohistochemically.
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PMID:An immunohistochemical study of peripheral neuroblastoma, ganglioneuroblastoma, anaplastic ganglioglioma, schwannoma and neurofibroma in cattle. 796 22

The histological localization of the ret proto-oncogene (proto-ret) product was examined in neural crest-derived and neuronal tissues together with their neoplastic counterparts by immunohistochemistry using a polyclonal antibody. Schwann cells, neurons, sympathetic ganglia, and cells of the adrenal medulla were positive for the proto-ret product, whereas melanocytes were negative. Positive results were obtained from neural crest-derived tumours such as schwannoma (69 per cent, 11/16), neurofibroma (59 per cent, 13/22), neuroblastoma (80 per cent, 4/5), phaeochromocytoma (100 per cent, 3/3) and medullary thyroid carcinoma (100 per cent, 3/3). The antibody reacted with all of the 22 astrocytomas examined. With negative proto-ret expression in melanocytic tumours, proto-ret expression was considered to correlate with the differentiation of some lineages of neural crest-derived cells.
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PMID:Expression of the ret proto-oncogene product in human normal and neoplastic tissues of neural crest origin. 819 28

Neurofibroma type 1 tissue was investigated for the presence of growth-promoting activity on human neuroblastoma cells. The activity was isolated by gel filtration and reversed-phase column chromatographs from neurofibroma type 1 extracts. An adenosine-containing dinucleotide (adenylyl(3'-5')cytidine-3'-phosphate) was identified as one of the major components of the activities by its enzymatic fragmentation and liquid chromatography/mass spectrometry. Synthetic adenosine-containing dinucleotide derivatives such as cytidyl(3'-5')adenosine, cytidyl(2'-5')adenosine, adenylyl(3'-5')cytidine, and adenylyl(2'-5')cytidine showed a similar action. Cytidyl(3'-5')adenosine, cytidyl(2'-5')adenosine, and adenylyl(2'-5')cytidine, which are able to release a free adenosine through enzymatic hydrolysis, in particular elicited a strong activity corresponding to that of adenosine with the highest action. These results suggest that neuroblastoma cells are able to use adenosine-containing dinucleotides as well as mononucleotides for their survival and proliferation.
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PMID:Growth-promoting action of adenosine-containing dinucleotide on neuroblastoma cells: detection of adenosine-cytidine dinucleotide (ApCp) in neurofibroma (NF1) extracts. 837 96

Human glioma cell extracts were found to elicit a marked growth-promoting activity on human neuroblastoma cells. This activity was also detected in the extracts of neurofibroma type 1 (NF1; von Recklinghausen neurofibromatosis) comprising aberrant Schwann cell growth. The purified substance from the NF1 extracts by HPLC on ODS columns was identical to a pyrimidine nucleoside, uridine, the chemical structure of which was identified by gas chromatography-mass spectrometry. The authentic uridine showed a strong growth-promoting activity on human neuroblastoma cells. Other purine or pyrimidine nucleotides, their derivatives, and ribose sources for their syntheses were employed to test the activity; a purine nucleoside, adenosine, showed a stronger activity than uridine. The current study raises the possibility that human neuroblastoma cells may be affected by dysfunctions of the de novo pathway of both purine and pyrimidine nucleotide biosyntheses.
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PMID:Neuroblastoma growth factors derived from neurofibroma (NF1): participation of uridine in a neuroblastoma growth. 841 51

Cutaneous ganglioneuroma is rare. Only five cases have been reported, and in all patients the lesions developed after birth. We describe a congenital cutaneous ganglioneuroma. The differential diagnosis includes well-differentiated cutaneous metastases of neuroblastoma and ganglion cells entrapped by a neurofibroma in patients with neurofibromatosis.
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PMID:Primary congenital cutaneous ganglioneuroma. 869 26

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

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.
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PMID:Spectrum of neurogenic tumors in the thorax: CT and pathologic findings. 1034 46


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