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)

Pathological type complications associated with 46 cases of neurofibromatosis in children under 12 are reported. It is noted that in 65.2% of the cases there are mental retardation, usually serious. More than 50% (24 cases) had some type of tumoration. All were benign with the exception of a suprarenal neuroblastoma that caused arterial hypertension and histological characteristics of malignancy. Fifteen tumors were located in the optica ways, one in the mediastinum, one in the abdomen, one in the paravertebral area, one which was a craneal plexiform tumor and four of the moluscum pendulum type on the eyelids or in neighbouring regions. Twelve children suffered from some type of seizures (Salaam's spasms, tonic-clonic, myoclonic, atonic and versive). Radiological abnormalities were very frequent in the simple X rays as well as in those in which contrast medium was used. In four cases malformations of the midline were observed, three of which were non-communicating cysts of the septum pellucidum, the other agenesis of the corpus callosum. Neurofibromatosis was further seen associated iwth Bourneville's syndrome, Morquio's syndrome, Batten's type of lipofuscinosis, facial or generalized hemihypertrophia and stenosis of the aqueduct. Heredity was dominant autosomic in 16 cases, the rest being due to possible recent mutations.
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PMID:[Pathological complications in 46 cases of neurofibromatosis in children (author's transl)]. 82 74

Major syndromes in which cutaneous and extracutaneous nervous neoplasms are frequently associated include: 1) dysgenetic syndromes or phacomatoses (tuberous sclerosis and neurofibromatosis), 2) multiple schwannoma syndromes (schwannomatosis and Carney's complex), 3) multiple mucosal neuromas syndrome, 4) neurocutaneous pigmentary syndromes (Peutz-Jeghers-Touraine syndrome and neurocutaneous melanosis), and 5) sundry associations (cutaneous meningiomas and cutaneous metastases of neuroblastoma or carcinoid tumors). The early clinical and pathological recognition of these cutaneous neural and pigmentary associated lesions should stimulate the search for centrally located neural or neuroendocrine neoplasms, some of which might be life-threatening.
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PMID:Cutaneous neuropathology: neurofibromas, schwannomas and other neural neoplasms with cutaneous and extracutaneous expressions. 195 52

Neurofibroma and neuroblastoma both arise from the neural crest, and there has long been speculation regarding a pathogenetic relationship between them. Clinical characteristics do not necessarily distinguish these tumors, therefore the diagnosis of neuroblastoma should be considered in all children with neurofibromatosis 1 (NF-1) who have a rapidly growing or inaccessible mass. A careful physical examination, imaging studies, and urinary catecholamine measurement are indicated. In a child with NF-1 and malignancy, direct tissue examination may be necessary to differentiate malignant from nonmalignant tumor and guide therapy. Furthermore, with the significantly increased risk of certain types of childhood cancer in these patients, we recommend evaluation for this common heritable condition in all patients with malignancy.
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PMID:Neurofibromatosis 1: recognition and management of associated neuroblastoma. 212 42

Polyomavirus mutants selected for modified host range exhibit DNA sequence alterations in the regulatory region, which consist mainly of duplications and/or deletions. Single base pair mutations have also been observed, which create or abolish DNA sequence motifs recognized by DNA-binding regulatory factors. The present work deals with the molecular characterization of a Polyoma mutant (PyNB11/1), selected for its high efficiency of growth in neuroblastoma cells. The enhancer region of PyNB11/1 displays a 91 bp tandem duplication harbouring a novel DNA sequence motif created at the boundary of the duplicated fragment. This motif is absent in the wild-type enhancer and is specifically recognized by a nuclear factor that belongs to the NF-1 family of transcription factors. We also report the characterization of an as yet unidentified DNA sequence motif in the D domain of the viral enhancer, that represents the binding site for a nuclear factor that is ubiquitous and comparably abundant in several murine cell types.
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PMID:Protein recognition sites in polyomavirus enhancer: formation of a novel site for NF-1 factor in an enhancer mutant and characterization of a site in the enhancer D domain. 215 86

The synchronous occurrence of neurofibromatosis and neuroblastoma has been labeled in the recent literature as a chance event. We report 2 cases of newborn infants with congenital neurofibromatosis and a similar midline pattern of multiple Schwann cell and neuroblastic tumors; other types of ectomesenchymal tumor differentiation are documented, along with supportive ultrastructural and immunohistochemical studies. The tumors may take an aggressive, fatal course despite maximal multimodality antitumor therapy. These 2 cases are reported, with additional literature review, to document a clinically recognizable neurocristopathy that links neuroblastic tumors and neurofibromatosis.
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PMID:Neurofibromatosis and associated neuroectodermal tumors: a congenital neurocristopathy. 301 62

Using the NIH two-phase microlymphocytotoxic test, lymphocytes of patients and control subjects were typed for HLA antigens of A and B loci: A1, 2, 3, 9, 10, 11, 28 (or Aw 19, A30, 31), B5, 7, 8, 12, 13, 14, 15, 17, 18, 21, w22, 27, 35, 40. Patients tested: 1. 75 patients with Wilms' tumour, thereof 35 had their whole families tested, 2. 20 patients with neuroblastoma, 3. 26 patients with neurofibromatosis, thereof 21 had their whole families tested, 4. 166 patients with testicular germinal tumours and 41 children with germinal tumours of diverse localization, 5. 48 individuals with haemangioma, 6. 64 women with breast cancer and 50 with dysplasia. We investigated 490 patients and, with the addition of family studies, another 193 individuals, altogether 683 persons. The results were compared with a group of 301 healthy non-related persons or with a group of 116 healthy non-related men, or with 100 healthy women and, in the family studies, with members of 47 healthy three-member families with healthy children. The chi 2 test with a Yates correction or also Fisher's exact test were used for the purpose. The resultant p was corrected using multiplication by the number of the antigens typed. In some cases we used the relative risk (RR) value. The results can be summed up in the following seven points: 1. Wilms' tumour was found to have no significant association either in our population or family studies. The latter seem to testify rather against this tumour's linkage with HLA. Our study was inconclusive as to the significance of the more frequent incidence of HLA-A1 and/or A9 in the diseased children of those families where one of the parents had at least one of those antigens. It cannot be ruled out as a sign of better prognosis. We regards as indispensable the typing of HLA antigens in patients with Wilms' tumour coincident with an inborn anomaly, as well as in members of those patients' families, and also a conclusive elucidation of the possible association with HLA-A1 and/or A9. No other centre has as yet undertaken any family studies. Consequently our possibilities of comparison with other teams' results were meagre. 2. We point to the possible conceivable relationship between neuroblastoma and HLA-B13. We found this association, albeit non-significant after correction, potentially important, especially after comparisons with the results of an only other existing study.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:HLA system and some neoplastic diseases. 307 Nov 20

During the period 1943-1980 a significant increase in the incidence of neuroblastoma was seen in Denmark. The incidence increased from a level corresponding to that in Finland to a level corresponding to that in the USA, and the increase appears to be continuing. The increase relates to children aged under 5 years, and is most pronounced in infants under 1 year. The incidence in the first year of life has, however, not yet reached the level of the USA. The increase in incidence is most likely a result of improved diagnosis, changes in the social composition of the population, and an increase in environmental carcinogens of importance in the induction of neuroblastomas. The incidence is lower in children of self-employed parents, and higher in infants of mothers aged under 20 or over 34 years. Aside from lower socio-economic circumstances for mothers under 20 years, no specific risk factors were revealed in this study. The observations of a family in which the mother has ganglioneuroma and both daughters have developed neuroblastoma, of a child who suffered from both neuroblastoma and neurofibromatosis von Recklinghausen, and of a significantly higher frequency of infants with signs of multicentric tumours in the offspring of mothers aged under 20 and over 34 years of age, is consistent with the two-hit theory of Knudson et al. (1972).
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PMID:Epidemiological investigations on neuroblastomas in Denmark 1943-1980. 309 20

The precise role of the nerve growth factor protein (NGF) during the growth and development of the human nervous system is not determined. Although it appears to influence a number of neural functions, its mechanism of action is poorly understood. A number of researchers have proposed that NGF may be involved in several pathological conditions including cancer. It has been shown that NGF is secreted by certain sarcoma (23), neuroblastoma (113), and glioma (7,102,136) cell lines and can bind to neuroblastoma and metastatic melanoma cell lines (42). Neuroblastoma (136,181) and pheochromocytoma (165) cells in vitro can be induced by NGF to differentiate toward a morphologically "more benign" state and appropriate NGF treatment of rats can reduce the number of chemically induced gliomas and neurinomas (174,178). NGF can also reduce the growth of intracerebrally inoculated anaplastic glioma cells (172). Anti-NGF treatment of rats (178) and mice (179) can alter the tumor distribution observed following ethylnitrosourea or benzo(a)pyrene treatment (10). In humans, it has been reported that serum levels of NGF are usually elevated in persons "at risk" for neurofibromatosis (156). The precise nature of the NGF role is not known in these instances. Further understanding of the action of NGF could be of clinical importance.
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PMID:Nerve growth factor and neural oncology. 630 Apr 14

Sixteen cases of malignant peripheral nerve tumors that were recorded in the files of the Department of Orthopedics, National Cancer Center Hospital, Tokyo, between 1972-July 1983 were studied clinicopathologically. The patients' ages ranged from 24-51 years, and both sexes were affected equally. Histologically, in 13 cases of nerve sheath tumors the tumors were spindle-cell type, two of these patients had manifestation of multiple neurofibromatosis (von Recklinghausen's disease). One malignant epithelioid schwannoma was found to arise from the tibial nerve. Other two cases were of primitive neuroectodermal tumors (primary malignant peripheral neuroblastoma) which showed rosette formation. The common primary symptoms in all patients were a noticeable mass which increased in size over a variable period of time, with or without associated pain and tenderness. Ultrastructural findings of spindle-cell type (in 7 tumors examined) and epithelioid type (1 tumor) showed evidence of Schwann cell differentiation of the tumors in all cases. Immunohistochemically, by the PAP method (Sternberger), staining for S-100 protein was positive in 3 of 14 tumors. Ultrastructural findings in two S-100 protein-positive cases showed evidence of Schwann cell differentiation better than the S-100 protein-negative cases, such as pronounced interdigitation of cytoplasmic processes, presence of fibrous long-spacing collagen and well-developed basal lamina. Local recurrence occurred in nine patients, and metastasis was found in five. The total 5-year survival rate was 58.5%. Tumors associated with multiple neurofibromatosis and primary peripheral neuroblastomas had the worst prognosis. Complete removal of the tumor by means of wide excision as primary treatment seemed to be the most important factor in decreasing the morbidity and mortality rates.
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PMID:Malignant peripheral nerve tumors: a clinicopathological and electron microscopic study. 642 67

We are studying the biological activity and regulation of mammalian Ras protein in tumours and in physiological signalling. We have shown that GAP (the GTPase-activating protein) is a potent negative regulator of normal Ras in cells. Reduction or loss of the NF1 gene product neurofibromin, in association with genetic abnormalities of the NF1 locus, has been identified in schwannoma cell lines from patients with neurofibromatosis and in melanoma and neuroblastoma lines from patients without neurofibromatosis. Although loss of neurofibromin in the schwannoma lines was associated with a high proportion of normal Ras protein in the active GTP-bound state, Ras-GTP appeared to be appropriately regulated in the melanoma and neuroblastoma lines, which contain normal levels of GAP. Therefore the GTPase-activating activity of neurofibromin is not essential for negative regulation of Ras in some cell types and the putative tumour suppressor function of neurofibromin in such cell types is independent of its GTPase-activating activity. Mitogen activation of Ras in fibroblasts is mediated primarily by exchange factors, which probably interact with a region on the Ras protein distinct from the region required for interaction with GAP. Multiple full-length cDNAs have identified a mouse gene whose products are related to yeast CDC25 guanine nucleotide exchange factor.
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PMID:Cell transformation by ras and regulation of its protein product. 829 27


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