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Ultrastructural pleomorphism in medulloepithelioma of the ciliary body: a comparative study of tumour cells and fetal ciliary epithelium.

A locally aggressive nonteratoid intraocular medulloepithelioma occurring in a two-year-old boy was studied by electron microscopy. For comparison the primitive pars plana region was examined in human fetal eyes obtained at 16, 18 and 22 weeks of gestation. The tumour was polymorphic with neural, intermediate and epithelial components. The neural component of the tumour resembled the zonulae adherentes dominant cell junction seen in the apical part of the unpigmented epithelium at 16 weeks, while the well-developed junctional complexes and interdigitations seen in the unpigmented epithelium at 22 weeks resembled those in the epithelial component of the tumour. Intermediate cells which exhibited characteristics of both neural and epithelial components, particularly in the cell junctional features, were found. Pleomorphism in nonteratoid medulloepithelioma might be accounted for by the developmental stage at which the tumour is initiated.

Ciliary Body↗

Importance of phenotypic and molecular characterization for identification of a neuroepithelioma tumor cell line, NUB-20.

A neuroblastic-like cell line (NUB-20) was derived from a case of histopathologically diagnosed metastatic neuroblastoma. The metastatic tumor and nude mouse heterotransplant resembled neuroblastoma by histological criteria, in contrast to the primary tumor, which was differentially classified as Ewing's sarcoma. However, the cell line demonstrated a unique phenotype in culture with respect to morphology, immunohistochemical markers, and sensitivity to a battery of differentiation modulators. These characteristics, together with the presence of a chromosomal translocation (11;22),(q24;q12) and amplification with enhanced expression of the c-myc protooncogene rather than N-myc, established this tumor as neuroepithelioma. Neuroepithelioma is a tumor type distinct from, but related to, neuroblastoma in its development from the neural crest lineage. These results emphasize the growing importance of cytogenetic and molecular markers in the classification and characterization of human tumors.

Bucladesine↗

Antisense inhibition of single copy N-myc expression results in decreased cell growth without reduction of c-myc protein in a neuroepithelioma cell line.

The N-myc gene is transiently expressed during normal embryonic development and abnormally expressed in several tumors of neuroendocrine origin. Little is known of the function of the N-myc gene product in either normal or neoplastic tissue. We utilized synthetic antisense oligodeoxynucleotides to specifically inhibit N-myc gene expression in the neuroepithelioma cell line CHP100. These cells contain single copy N-myc alleles but overexpress c-myc. N-myc antisense oligomer treatment was found to be growth inhibitory without affecting levels of c-myc protein. N-myc antisense oligomer-treated cells also lost the characteristic cellular heterogeneity displayed by CHP100 in vitro.

Cell Division↗

Selective down-regulation of the pro-enkephalin gene during differentiation of a multiple neuropeptide-co-expressing cell line.

Regulation of co-expression of three neuropeptide genes, i.e. genes encoding enkephalin, cholecystokinin, and gastrin-releasing peptide, was studied in human neuroepithelioma cells. In nondifferentiated state, the continuous cell line SK-N-MC displayed an equally high level of expression of the enkephalin, cholecystokinin, and gastrin-releasing peptide genes. By culturing in medium containing endothelial cell growth supplement the SK-N-MC cells differentiated morphologically into a cell type with neurite-like processes. After 3 days the expression of the enkephalin gene in endothelial cell growth supplement-differentiated cells was significantly reduced by 75% as compared to the nondifferentiated cells, while there was no change in the expression of the cholecystokinin and gastrin-releasing peptide genes during differentiation. The results show that the enkephalin gene is selectively down-regulated during differentiation of neuroepithelioma cells. It is suggested that the down-regulation is related to the transient expression of the enkephalin gene in developing brain and other organs. Thus the neuroepithelioma cell line may provide a cellular model to study the underlying molecular mechanism.

Blotting, Northern↗

[Immunohistochemical examination of olfactory neuroblastoma].

The results of the immunohistochemical examinations by means of PAP method in 3 olfactory neuroblastomas were described. In the neoplastic cells there were cytokeratin as well as neuronal enolase. The same was recently encountered in the merkel cell tumor, which have the analogous histogenesis. The usefulness of the immunohistochemical method of examinations are essential in correct diagnosis.

Adult↗

[Olfactory neuroblastoma--tumor entity or complex clinical picture?].

Clinical and radiological findings in seven cases of olfactory neuroblastoma are reviewed and discussed in the light of the international literature. The report provides further evidence of difficulties related to the predictability of the condition's clinical course, diagnosis, and therapeutic consequences. Moreover, the lack of uniform CCT and NMR features indicates that the discussion about the genesis, the biological action, the terminology, and therapy of these tumors has not yet reached a conclusion.

Adolescent↗

[Clinicopathological features and immunohistological diagnosis of olfactory neuroblastoma].

Nasal olfactory neuroblastoma and other malignant neoplasms showing spherical cell are easily confused in clinical and pathological diagnosis. We have made immunohistochemical staining in 7 olfactory neuroblastoma cases. Results showed that they were all positively stained by NSE, and negatively by keratin, leucocyte common antigen, desmin and S-100 protein (except 1 positive by desmin). We believe that the olfactory neuroblastoma cells are uniform morphologically. The cells are round, elliptic, having scant cytoplasm; with coarse or fine chromatin. Neurofibers can be found among tumor cells. Their histological structures vary greatly. They may form nest or garland, and they may also form striation, cleft or discrete structure; but rosette or pseudo-rosette structure is uncommon.

Adolescent↗

[Errors in the early diagnosis of malignant tumors of the ethmoid sinus].

This paper discusses the factors responsible for delayed diagnosis of malignant tumors of ethmoidal sinuses. It presents 9 cases: keratinizing type (6 cases) and nonkeratinizing type (1 case) of squamous cell carcinoma, nasopharyngeal poorly differentiated carcinoma (1 case), esthesioneuroblastoma (1 case). The diagnosis was made on the basis of a detailed study of case histories (spontaneous nasal bleeding and recurrent polyposis) and X-raying (total, contrast and tomographic). The clinical investigation was supplemented with operational green filter optics that helped detect tumor specific lesions. The final diagnosis was made on the basis of histological examinations.

Adult↗

[The value of radiotherapy in the treatment of esthesioneuroblastoma].

Three cases of aesthesioneuroblastoma highlight the problems of treatment of these extremely rare tumours. These tumours of the olfactory epithelium arise in the nasal cavity and invade the paranasal sinuses, the orbit and the skull base. As symptoms occur late in the disease, most cases are advanced when diagnosed. Early cases can be treated by surgery, by irradiation or by a combination of, but the local control rate is about 60% regardless of the treatment. Chemotherapy should be used in addition to operation and irradiation, especially in advanced cases with nodal and distant metastases. An irradiation dose of 40-60 Gy is recommended. CT-aided treatment planning, sophisticated irradiation techniques, the use of high-energy photons and reliable immobilization measures are indispensable. A 5-year survival rate for all stages of 50% is reported. The recurrence rate in advanced stages is high, so that the benefits and risks of the different, combined treatment modalities must be evaluated carefully.

Adolescent↗

[Esthesioneuroblastoma--treatment of recurrences using the remote-control afterloading technic. A case report].

At the Radiological Hospital of the Heidelberg University, one patient with a local recurrence of an esthesioneuroblastoma situated on the left-hand side of the skull base was treated between February 11 and March 18, 1986, by an afterloading contact therapy with curative intent. After preceding surgery and percutaneous radiotherapy, repeated local recurrences and locoregional lymph node metastases had occurred. The contact therapy was performed after adjusting an individual face mask in which the afterloading probe was placed and brought into its position in the tumor region. The irradiation scheme was based on the transformation of the source coordinates form the stereoscopic X-ray localization system into the coordinate system of the computed tomogram by means of X-ray tight reference points. The irradiation planning by computed tomography allows to optimize the arrangement of sources within the tumor region. The principles of the method as well as the treatment result are presented.

Brachytherapy↗

[Treatment of olfactory neuroblastoma: a report of 14 cases].

Olfactory neuroblastoma is a rare malignant tumor arising from the nasal cavity. From 1982 to 1987 fourteen cases had been treated in this hospital. The average age was 34 years with a range from 15 to 65 years, male to female ratio was 1:1. At the time of diagnosis, 3 tumors were of kadish stage A-involvement of the nasal cavity only, 5 of stage B-involvement of the nasal cavity and one or more paranasal sinuses, and six of stage C-extension beyond the nasal cavity, including the orbit, base of skull or intracranial cavity, or metastasis to cervical nodes or distant sites. All were treated with radiation therapy and 7 also by surgery. The three and five-year survival rates were 50% and 30% respectively. Seven patients died, six because of distant metastases within one year. It shows that the disease is aggressive from the beginning, and tends to metastasize early. A treatment regimen for prophylactic cervical node irradiation combining chemotherapy is proposed.

Adolescent↗

In vitro differentiation of human neuroblastoma cells caused by vasoactive intestinal peptide.

Neuroblastoma, a tumor of the sympathetic nervous system, is the most common solid malignancy of childhood outside the central nervous system. Vasoactive intestinal peptide (VIP) is produced by some of these tumors, and elevated serum levels correlate with tumor cell differentiation and a favorable prognosis. It has previously been demonstrated that human neuroblastoma cell lines LA-N-5 and IMR-32 will differentiate in vitro when exposed to retinoic acid. It is now shown that VIP also induces in vitro differentiation of these neuroblastoma lines. LA-N-5 or IMR-32 cells were grown in the presence of different concentrations of VIP. Cell proliferation was suppressed, as measured by cell count, incorporation of [3H]thymidine, and measurement of the proliferation index. The degree of suppression correlated with the concentration of VIP, and the effect was indistinguishable, on a molar basis, from that seen when cells were treated with retinoic acid. Similarly, the morphological changes seen in the VIP-treated cells were the same as those seen in retinoic acid-treated ones. The effects of VIP on both cell lines, like those of retinoic acid, are reversible. The human neuroepithelioma line CHP-100, is much less sensitive to either agent. Vasoactive intestinal peptide is the first substance shown to cause differentiation of neuroblastoma cells in vitro which is also known clinically to have a specific association with that tumor. It is postulated that VIP may play a key role in the well-documented maturation of these tumors in vivo and in the normal development of the sympathetic nervous system. These findings may also have therapeutic implications for the management of this frustrating childhood malignancy.

Breast Neoplasms↗