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 clinical history and autopsy findings of a 22-week fetus with intestinal ganglioneuroblastoma, cardiac anomalies, omphalocele, and ileal atresia are presented. Ganglioneuroblastoma was confined to the large intestine and was not suspected prenatally despite ultrasonographic examination. Although enteric ganglia share neural crest lineage with other sites of congenital neuroblastoma, this is the first report of a primary intestinal ganglioneuroblastoma in a fetus or child. Various hypotheses are discussed to explain the coexistence of cardiac malformation and congenital neuroblastoma in this fetus and other cases in the literature. It is hypothesized that other malformations evident in this fetus were caused by the tumor, possibly as teratogenic effects of neuroblastoma-derived catecholamines during embryogenesis.
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PMID:Intestinal ganglioneuroblastoma in a 22-week fetus. 140 56

Neuropeptide Y (NPY) was investigated as a possible tumor marker in pediatric patients with tumors of the sympathetic nervous system. Seven patients with neuroblastoma, 3 patients with ganglioneuroblastoma, and 2 with ganglioneuroma, were compared with 12 matched healthy controls and 34 tumor controls. NPY-like immunoreactivity (NPYLI) was analyzed in extracted plasma using a competitive radioimmunoassay. At diagnosis, plasma NPYLI was significantly increased (p less than .001) in the neuroblastoma patients (352 +/- 99 pM; mean +/- SEM) when compared with healthy controls (36 +/- 4 pM) and tumor controls (30 +/- 2 pM). Ganglioneuroblastoma and ganglioneuroma patients had lower levels (57 +/- 8 pM) than neuroblastoma patients but still significantly higher than the controls. In all patients with sympathetic tumors, the NPYLI level was decreased after treatment. Five neuroblastoma patients relapsed; all had increasing NPYLI levels. In 3 of these patients, incresing NPYLI was the first sign of relapse. Plasma NPYLI correlated well to urinary levels of homovanillic acid. NPY in plasma (NPYLI) may be a clinically useful marker of pediatric neuroblastoma for diagnosis and differential diagnosis. NPYLI correlates well with the clinical course and can be the first sign of relapse. Plasma determinations of NPYLI make it possible to monitor rapid alterations of disease.
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PMID:Neuropeptide Y as a marker in pediatric neuroblastoma. 231 29

Ganglioside profile was evaluated in 19 samples of tumor tissue obtained from 13 surgical patients with various morphological patterns of neuroblastoma. In six of those cases, two samples from each proving most different in terms of cell maturity were selected for examination. The relative content of GD2 gangliosides was 27.0-37.6% in sympathoblastoma and as low as 5.1-14.8% in ganglioneuroblastoma. Ganglioneuroblastomas showed fairly high levels of GM1 and GD1a gangliosides which were almost completely absent in sympathoblastomas. Ganglioside profile variations seen within each tumor type were incomparable with differences in profile established between morphological patterns of neuroblastoma studied.
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PMID:[The immunological cellular phenotype and the prognosis in neuroblastoma in children]. 237 89

We present adult adrenal ganglioneuroblastoma with giant regional lymph node metastasis. The patient was a 21-year-old male, who had a left adrenal tumor diagnosed by ultrasonography incidentally. He was moderately developed and physical examination was almost normal. Blood pressure was within the normal range. Only the value of the vanilmandelic acid, which was 68.7 mg/day, in the urine was abnormal. Other laboratory data were almost normal. Neither chemical nor physical hormonal disorders were present. We carried out the radical surgical treatment by trans-thoracoabdominal approach under the diagnosis of left adrenal neuroblastoma. Pathological diagnosis was ganglioneuroblastoma. After the operation, the patient received both systemic chemotherapy of vincristine and cyclophosphamide and immunotherapy of OK-432 by subcutaneous administration. Furthermore, 40 Gy of the regional irradiation of 60Co to the operative space after the surgery was performed. At eight months after the operation, he is alive with no evidence of disease. Ganglioneuroblastoma is a neuroblastic tumor including neuroblastoma, which commonly occurs in childhood. The prognosis of the neuroblastic tumors which occur either in the adrenal gland of a young child or in an older child is poor. On the other hand, the biological activity of the adult neuroblastic tumors is different from that of the pediatric neuroblastic tumors. In the adult, the adrenal ganglioneuroblastoma does not always have a poor prognosis. The multimodality treatment which we performed is effective for the neuroblastic tumor. Ultrasonography is both effective and adequate for the screening of the retroperitoneal lesion such as in our case.
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PMID:[Multimodality treatment of adrenal ganglioneuroblastoma: a case report]. 307 Nov 24

Ganglioneuroblastoma a transitional tumor of sympathetic origin has not yet been described as involving orbit. It is characterized by a mixture of cells ranging from primitive neuroblast to well differentiated ganglion cells within a neurofibromatous tissue. The prognosis is uncertain, as the tumor may either undergo maturation into a ganglioneuroma or may metastasize widely and rapidly as in neuroblastoma. We may postulate a relationship between ganglioneuroblastoma and Recklinghausen's neurofibromatosis in view of the development of the tumor in conjunction with the phacomatosis.
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PMID:[Ganglioneuroblastoma of the orbit]. 392 90

Neuroblastic tumors have the unique ability to differentiate and mature. This family of tumors is composed of the neuroblastoma, ganglioneuroblastoma, and ganglioneuroma. These tumors are derived from primordial neural crest cells that form the sympathetic nervous system. The purpose of this study was to characterize the ultrastructural features of neuroblastic tumors in a pediatric population. Forty-five neuroblastic tumors (15 neuroblastomas, 15 ganglioneuroblastomas, and 15 ganglioneuromas) were examined using standard transmission electron microscopic techniques. Undifferentiated neuroblastomas were composed of primitive cells with rare neurite-like processes containing clear secretory vesicles and no Schwann cell differentiation. Poorly differentiated and differentiating neuroblastomas showed more frequent neuritic processes with infrequent dense core granules and infrequent immature Schwann-like cells. Ganglioneuroblastomas possessed an admixture of cell types, including immature ganglion cells without associated satellite cells, intermediate cells, and differentiating neuroblasts. The neuropil contained immature Schwann cells encasing haphazardly arranged neuritic processes. Ganglioneuromas were composed of mature ganglion cells with occasional binucleation. The neuropil contained mature Schwann cells with well-organized neuritic processes and abundant collagen deposition. Differentiation or maturation of tumor cells, neuritic processes, and Schwann cells may thus be discerned ultrastructurally in primary neuroblastic tumors in pediatric patients.
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PMID:Comparison of ultrastructural features among neuroblastic tumors: maturation from neuroblastoma to ganglioneuroma. 757 Oct 90

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

Neuroblastoma, ganglioneuroblastoma, and ganglioneuroma are tumors of the sympathetic nervous system that arise from primitive sympathogonia and are referred to collectively as neuroblastic tumors. They arise wherever sympathetic tissue exists and may be seen in the neck, posterior mediastinum, adrenal gland, retroperitoneum, and pelvis. The three tumors differ in their degree of cellular and extracellular maturation; immature tumors tend to be aggressive and occur in younger patients (median age, just under 2 years), whereas mature tumors occur in older children (median age, approximately 7 years) and tend to behave in a benign fashion. The most benign tumor is the ganglioneuroma, which is composed of gangliocytes and mature stroma. Ganglioneuroblastoma is composed of both mature gangliocytes and immature neuroblasts and has intermediate malignant potential. Neuroblastoma is the most immature, undifferentiated, and malignant tumor of the three. Neuroblastoma, however, may have a relatively benign course, even when metastatic. Thus, these neuroblastic tumors vary widely in their biologic behavior. Features such as DNA content, tumor proto-oncogenes, and catecholamine synthesis influence prognosis, and their presence or absence aids in categorizing patients as high, intermediate, or low risk. Treatment consists of surgery and, usually, chemotherapy. Despite recent advances in treatment, including bone marrow transplantation, neuroblastoma remains a relatively lethal tumor, accounting for 10% of pediatric cancers but 15% of cancer deaths in children.
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PMID:Neuroblastoma, ganglioneuroblastoma, and ganglioneuroma: radiologic-pathologic correlation. 1211 Jul 23

Neuroblastoma (Nb) is a malignancy of the sympathetic nervous system which affects children in their first decade. It is the most common extra-cranial solid tumor in children with an incidence of approximately 1 in 8-10 000 live births annually and accounts for approximately 10% of all children's cancers. Ganglioneuroblastoma is a relatively benign form of Nb and consists of a mixture of fibrils, mature and maturing ganglion cells, as well as undifferentiated neuroblasts. During routine cytogenetic analysis of patients with different manifestations of neuroblastoma we have identified one patient with ganglioneuroblastoma that carries an apparently balanced t(1:13)(q21:q12) reciprocal translocation. Positional cloning of the translocation breakpoint on chromosome 13 resulted in the mapping of the breakpoint between coding exon 2 and exon 3 of WAVE3, a member of WASP gene family. Although the breakpoint region on chromosome 1 was localized to within 2 kb of genomic sequence, no gene was found to be interrupted on this chromosome. The WAVE3 transcript is mainly expressed in the nervous system and, like all the members of the WASP gene family, WAVE3 is a key element in actin polymerization and cytoskeleton organization. WAVE3, therefore, is important for cell differentiation and motility and its expression is lost in a number of low grade and stage 4S tumors. From analysis of its expression pattern and function, WAVE3 is a candidate tumor suppressor gene, at least in some forms of neuroblastoma.
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PMID:WAVE3, an actin-polymerization gene, is truncated and inactivated as a result of a constitutional t(1;13)(q21;q12) chromosome translocation in a patient with ganglioneuroblastoma. 1218

Neuroblastic tumours are a group of paediatric cancers with marked morphological heterogeneity. Neuroblastoma (Schwannian stroma-poor) (NB-SP) is composed of undifferentiated neuroblasts. Ganglioneuroblastoma intermixed (Schwannian stroma-rich) (GNBi-SR) is predominantly composed of Schwannian stromal (SS) and neuroblastic (Nb) cells. There are contrasting reports suggesting that SS cells are non-neoplastic. In the present study, laser capture microdissection (LCM) was employed to isolate SS and Nb cells. Chromosome 1p36 deletion and MYCN gene amplification were found to be associated in two out of seven NB-SPs, whereas no abnormalities were observed in five GNBi-SRs. In some cases, loss of heterozygosity (LOH) at 1p36 loci was detected in Nb cells but not in the bulk tumour by LCM; furthermore, LOH was also identified in both SS and tumour tissue of a GNBi-SR. DNA gain and loss studied by comparative genomic hybridization were observed at several chromosome regions in NB-SP but in few regions of GNBi-SR. Finally, gene expression profiles studied using an oligo-microarray technique displayed two distinct signatures: in the first, 32 genes were expressed in NB-SP and in the second, 14 genes were expressed in GNBi-SR. The results show that NB-SP is composed of different morphologically indistinguishable malignant cell clones harbouring cryptic mutations that are detectable only after LCM. The degree of DNA imbalance is higher in NB-SP than in GNBi-SR. However, when the analysis of chromosome 1p36 is performed at the level of microdissection, LOH is also observed in SS cells. These data provide supportive evidence that SS cells have a less aggressive phenotype and play a role in tumour maturation.
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PMID:Genome analysis and gene expression profiling of neuroblastoma and ganglioneuroblastoma reveal differences between neuroblastic and Schwannian stromal cells. 1617 58


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