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Results for “Neuroectodermal Tumors, Primitive, Peripheral”

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At least 1,495 records · Page 83Linked to original sources
PubMed1994

[Clinicopathological features of dysembryoplastic neuroepithelial tumor (DNT): four cases report].

We reported four cases of dysembryoplastic neuroepithelial tumor (DNT) with intractable complex partial seizures, and analyzed their clinical and pathological features. The age of patients ranged from four to nineteen years old. Three were boys and one was a girl. The age of seizure onset ranged from eight months to five years. They did not have any neurological deficits. In all cases, CT scan revealed a low density mass in the temporal lobe. Two cases demonstrated calcifications, and another two cysts. The mass demonstrated low signal intensity in T-1 weighted MR images with mild contrast enhancement by Gd-DTPA. Long-term video-EEG monitoring revealed the epileptogenic focus in the temporal lobe on the side of the mass lesion. Anterior temporal lobectomy with total removal of the tumor was carried out in all cases. Hippocampectomy was performed in three cases in which intraoperative electrocorticogram demonstrated spike discharges in the hippocampus. In the surgical specimens, the tumor consisted of increased astrocytes, oligodendrocytes, and some neurons. A cortical nodule appeared in one case, and cortical dysplasia appeared in two cases. It was difficult to distinguish the tumor of case 3 from ganglioglioma because of mild neuronal atypia. The tumor of case 4 demonstrated increased capillary vessels and was also difficult to differentiate from vascular anomalies. The pathological diagnosis of DNT was still difficult. Consequently, we speculated that DNT included some transitional types between congenital anomalies and ganglioglioma.

Adolescent↗
PubMed1994

The ETS oncogene family in development, proliferation and neoplasia.

V-ets was found as an oncogenic sequence in the E26 avian retrovirus, which produced leukemias in chickens. There are a large number of mammalian homologues of v-ets, all sharing a common sequence called the ETS domain. These mammalian homologues have been found to be transcriptional activators, and the ETS domain is the DNA binding portion of the gene. This family has been found to be important in regulating embryonic development and the response to growth stimuli. In addition, PU.1 and FLI-1 are dysregulated by Friend leukemia virus insertion in mice resulting in erythroleukemia. Significantly, the DNA binding portion of FLI-1 is the 3' part of an oncogenic fusion transcript (termed EWS-FLI) in human Ewing's sarcoma and neuroepithelioma.

Amino Acid Sequence↗
PubMed1993

[Malignant teratoid medulloepithelioma of the ciliary body. Apropos of a case and review of the literature].

A case of ocular medulloepithelioma is reported and the authors review the literature concerning these rare tumors. This report concerns a ten-month-old girl in whom ophthalmic examination showed a large tumor arising in the ciliary body with such rapid progression that enucleation was necessary. Pathological findings were compatible with a malignant teratoid medulloepithelioma. According to preceding descriptions, slit lamp examination and ultrasonography are the best approach to the diagnosis. Medulloepithelioma is a tumor composed of poorly differentiated neuroepithelial cells and the predominant histological feature is a net-like pattern. Teratoid forms contain cartilage or brain tissue or bundles of skeletal muscle. The main criterion for malignancy is the presence of neuroblastic cells. Local excision is sufficient when the tumor is mall, otherwise enucleation or even exenteration may be necessary. The prognosis is generally good even when the tumor presents malignant features on pathological examination, provided treatment is instituted rapidly.

Ciliary Body↗
PubMedDate not supplied

Dysembryoplastic neuroepithelial micro-tumor in a 75-year-old patient with long-standing epilepsy.

Dysembryoplastic neuroepithelial tumors (DNT) are rare, cortically located neoplasma associated with intractable partial complex seizures. This entity was first described by Daumas-Duport et al. [1988], who also coined the term DNT. In that report, at the time of operation, the patients' ages ranged from 3 to 30 years and the duration of symptoms ranged from 2 to 18 years. In contrast, we observed a histologically comparable tumor in a 75-year-old female with a 60 year epilepsy history. Autopsy revealed an 8 mm large tumor in the right hippocampus. In view of the extremely prolonged course of the patient's symptoms and the complete lack of excessive cell proliferation, the differentiation of a genuine tumor versus a hamartoma remains equivocal.

Aged↗
PubMed1993

Directed expression of an oncogene to the olfactory neuronal lineage in transgenic mice.

The mammalian olfactory system provides a useful model to understand the cellular and molecular mechanisms governing the development of the nervous system. The olfactory neuroepithelium undergoes continual turnover in the adult animal, resulting in a neural tissue containing cells at various stages of neurogenesis. We have generated a transgenic mouse line to examine the effects of directed expression of an oncogene within the olfactory neuronal lineage. A hybrid oncogene was constructed utilizing the regulatory elements for the olfactory marker protein gene to direct the olfactory neuronal-specific expression of simian virus 40 T-antigen, a potent oncogene. The resulting transgenic mouse line expressed T-antigen only in olfactory neurons. Ten-month-old transgenic mice displayed significant hypoplasia of the neuronal elements in the olfactory neuroepithelium. The transgenic mice developed neuroblastomas of olfactory neuronal origin at a low frequency. Distinct clonal lines were derived from the primary culture of the tumor. GAP-43, a growth-associated neuronal marker, was expressed by some of the cell lines. One of the cell lines, 2.2, appeared to be responsive to neurotrophic effects from the presumptive target tissue, the olfactory bulb.

Animals↗
PubMed1993

Esthesioneuroblastoma: diagnosis, prognosis, and treatment.

Esthesioneuroblastoma is a rare neoplasm arising from the olfactory epithelium and characterized by slow growth, frequent local recurrence, and metastasis. This tumor has no known etiologic cause and has been studied from clinically retrospective analysis. The historic treatment of esthesioneuroblastoma has included surgical resection, adjuvant radiation therapy, and chemotherapy for advanced disease. We describe a case of esthesioneuroblastoma which illustrates the diagnostic, prognostic, and therapeutic aspects of this uncommon neoplasm.

Aged↗
PubMed1993

Characterization of insulin-like growth factor 1 in human primary brain tumors.

Insulin-like growth factor 1 (IGF-1) is involved in the regulation of brain development and has been suggested as an autocrine stimulator of brain tumor cell proliferation. This study demonstrates the expression of IGF-1 in tumor tissue from human gliomas and one esthesioneuroblastoma. Using immunohistochemistry, expression of an IGF-1-like peptide was localized in tumor cells of 6 of the 9 gliomas examined as well as the esthesioneuroblastoma. From one anaplastic oligodendroglioma (which showed strong IGF-1 immunostaining) the IGF-1 transcripts were characterized after isolation of mRNA followed by amplification using the reverse transcriptase-polymerase chain reaction. Two IGF-1 complementary DNAs resulting from alternative splicing of the IGF-1 primary transcript were identified. These transcripts encode two different precursor proteins which correspond to Ea IGF-1 and Eb IGF-1. The significance of IGF-1 alternative mRNA splicing pathways remains to be determined.

Amino Acid Sequence↗
PubMed1977

Esthesioneuroblastoma. Case report.

We present a case of esthesioneuroblastoma, and we emphasize the importance of considering this tumor in the differential diagnosis of primary intranasal carcinomas. Although the tumor is uncommon, we feel that many cases have been misdiagnosed histologically. The typical history in these patients is one of intermittent recurring epistaxis or nasal destruction and frequently, at the time of the initial examination, the tumor has reached a considerable size with evidence of local invasion.

Diagnosis, Differential↗
PubMed1995

[Dysembryoplastic neuroepithelial tumor].

The dysembryoplastic neuroepithelial tumor is a benign central nervous system neoplasm of children and young adults manifesting almost exclusively in complex partial seizures. The authors report the case of an 8-year-old boy presenting with characteristic clinical and radiologic features who subsequently underwent surgery. Light microscopic, immunohistochemical and ultrastructural traits of the tumor are described demonstrating pluripotential differentiation of tumor cells and architectural features suggesting a dysontogenic lesion. A brief literature review on the biology and histologic diagnosis of the dysembryoplastic neuroepithelial tumor is provided. Although this uncommon tumor accounts for only a minority of intracranial neoplasms, its pathogenetic role has to be considered in the differential diagnosis of temporal lobe epilepsies. This is the first report on this recently described, rare neoplastic condition in Hungary.

Brain Neoplasms↗
PubMed1996

Coexpression of platelet-derived growth factor alpha and beta receptors on medulloblastomas and other primitive neuroectodermal tumors is consistent with an immature stem cell and neuronal derivation.

Medulloblastomas and related childhood primitive neuroectodermal tumors (PNET) are currently thought to originate from a multipotent stem cell that can give rise to a variety of different tumor types, representing different lines of differentiation and stages of maturation of the original stem cell. The proliferation and differentiation of different cell types in the central nervous system are influenced by growth factors, such as platelet-derived growth factor (PDGF). In this study, we investigated the expression of the two types of PDGF receptors, alpha and beta, on 14 cases of PNET by immunohistochemistry and in situ hybridization. To characterize the tumor cells according to their differentiation and maturation, we performed immunohistochemistry with Ab against several members of the intermediate filament family, such as neurofilament, glial fibrillary acidic protein, vimentin, and the embryonal marker nestin. For the cerebral PNET, we found that the phenotype of multidirectional differentiation (with coexpression of neurofilament, glial fibrillary acidic protein, vimentin, and in some cases nestin) was associated with the expression of both PDGF alpha and beta receptors on the tumor cells. Medulloblastomas (i.e., cerebellar PNET), however, often showed the phenotype of partially committed neuron-like precursor cells, and this was associated with the predominant expression of PDGF alpha receptors on the tumor cells. Our findings show that the PDGF alpha receptor, previously known to play a role in the normal development of cells of the glial lineage, either exclusively or in combination with the PDGF beta receptor, is expressed on tumor cells of neuronal derivation. This "aberrant" expression of PDGF alpha receptors on PNET may reflect a feature of the malignant phenotype of these tumors. Further studies are needed to study the biologic effects of PDGF-A and its receptor in PNET.

Brain Neoplasms↗
PubMed1996

Isolation of a novel oncogene, NET1, from neuroepithelioma cells by expression cDNA cloning.

We generated a cDNA expression library from a human neuroepithelioma cell line for detection of novel oncogenes by focus formation assay in NIH3T3 cells. A morphologically unique focus was identified upon transfection and the transforming plasmid was isolated. The transforming gene, designated NET1, encoded a predicted protein species of 54 kDa containing the Dbl-Homology (DH) motif. This motif is also present in other growth regulatory molecules including Bcr, Cdc24, Vav, Ras-Grf, Ect2, Ost, Tim and Tiam1, which have been implicated as regulators of small GTP-binding proteins. NIH3T3 cells transfected with NET1 expression plasmid showed altered growth properties in vitro and were tumorigenic when injected into nude mice. In addition, a 2.5 kb cDNA was isolated from a normal human cDNA library which represented the NET1 proto-oncogene contained a 5' extended open reading frame. The fact that the proto-oncogene failed to induce transformation in NIH3T3 cells suggested that the original NET1 oncogene was activated by 5'-truncation. The 3.0 and 2.4 kilobasepair (kb) transcripts of the NET1 gene was ubiquitously expressed in all tissues examined. Using fluorescence in situ hybridization, we localized the NET1 gene to the short arm of human chromosome 10 at band p15.

3T3 Cells↗
PubMed1995

[Malignant neuroectodermal tumor of the arm in a 15-year-old girl successfully treated with chemo- and radiotherapy].

In a 15-year-old girl with right upper arm tumor diagnosed at first as neuroblastoma, the second histopathological examination revealed PNET. In spite of the advanced stage of disease (IIIrd or IVth) surgical excision was not performed but chemotherapy (8 courses of VADRAC and 8 courses of VP16 + CDDP) and radiotherapy (40 Gy) was administered. After 4 initial cycles all symptoms of the disease disappeared. The child remains in CCR for 60 months, including 40 months of therapy.

Adolescent↗