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Results for “Neuroectodermal Tumors, Primitive, Peripheral”

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PubMed2004

[Primary neuroectodermal tumour of the kidney invading the wall of the inferior vena cava. Surgical strategy].

Primary neuroectodermal tumours (PNET) are very aggressive malignant tumours, rapidly progressing to metastasis and death. They rarely involve the kidney, as only 23 cases have been described in the literature. Renal tumours, regardless of their histology, spread to the inferior vena cava (IVC) in 4% to 10% of cases. Invasion of the inferior vena cava wall by tumour thrombus is rare and, when it occurs, is often limited to the ostium of the renal vein. The authors report a case of primary neuroectodermal tumour of the kidney with thrombus in the inferior vena cava ascending to the right atrium (level IV), invading the wall of the IVC and associated with a non-infiltrating papillary urothelial tumour of the ureter. They describe the surgical management of this tumour and present a review of the literature.

Aged↗
PubMed1992

[Stereotaxy, MRI and independent computer. 85 punctures].

After 150 stereotactic biopsy with CT, the team of Val-de-Grâce has made these ones with MRI and simulation of the complete procedure with an independent computer. The result of 85 biopsies is reported. This one imply the acquisition of 3DFT millimetrics slices, the transfer by Ethernet network on a computer where a specific software treat the data and allow to establish the better course for the needle biopsy. The constraints of quality are very important and some test must be executed. The diagnosis are established, avoiding an unless trepanation.

Adult↗
PubMed2005

Molecular detection of EWS-Ets fusion transcripts and their clinicopathologic significance in Ewing's sarcoma/peripheral primitive neuroectodermal tumor.

BACKGROUND: Ewing's sarcoma/peripheral primitive neuroectodermal tumor (ES/pPNET) is often difficult to distinguish from other small round cell tumors. The EWS-Ets gene fusions that result from chromosomal translocations in this tumor provide potential molecular diagnostic markers. To apply these molecular markers to commonly available archival materials, we evaluated the feasibility of detecting EWS-Ets including EWS-Fli1 and EWS-ERG fusion transcripts in paraffin-embedded tissues and its diagnostic value for detecting ES/pPNET. METHODS: Thirteen paraffin-embedded samples of ES/pPNETs were retrieved from archives. Thirteen cases of other tumors with small round cell features (including rhabdomyosarcoma, neuroblastoma, lymphoma, small cell carcinoma, and desmoplastic small round cell tumor) were used as negative controls. Beta-actin and beta2-microglobulin were used as internal controls. A nested reverse transcriptase-polymerase chain reaction (RT-PCR)-based assay was performed to detect the EWS-Fli1 and EWS-ERG fusion transcripts. RESULTS: Beta-actin and beta2-microglobulin were detected in 10/13 and 13/13 ES/pPNETs, respectively. EWS-Fli1 fusion transcripts were detected in 11 of 13 (85%) ES/pPNETs. Three chimeric transcripts, all EWS-Fli1, were detected in ES/pPNET samples. Among 11 EWS-Fli1-positive cases, 7 cases had a type I fusion transcript involving fusion of EWS exon 7 with Fli1 exon 6, 2 cases had a type II fusion transcript involving EWS exon 7 with Fli1 exon 5, and 2 cases expressed fusion transcripts involving EWS exon 7 and Fli1 exon 8. Type I EWS-Fli1 fusion predominated over other types. Fusion types could not be distinguished in the remaining 2 cases. Thirteen negative controls did not show detectable chimeric messages. There was a significant relationship between EWS-Fli1 fusion transcripts and CD99 expression. CONCLUSIONS: Molecular detection of EWS-Fli1 fusion transcripts in formalin-fixed paraffin-embedded material by nested RT-PCR is feasible and is useful for the diagnosis and differential diagnosis of ES/pPNETs.

Adolescent↗
PubMed2005

[Clinicopathological study of Ewing's sarcoma/primitive neuroectodermal tumor in oral and maxillofacial region].

OBJECTIVE: To study the clinicopathological features and diagnostic criteria of Ewing's sarcoma/primitive neuroectodermal tumor (Ewing's sarcoma/PNET) in oral and maxillofacial region. METHODS: There were 15 patients with Ewing's sarcoma/PNET in the last 35 years at our hospital, aged 1-49 years and mean 14.5 years. The most common manifestation was swelling of the affected region. The cases were analyzed and histological and immunohistochemical studies were also conducted to examine CD99 (12E7), Vimentin, NSE, S-100, Syn, CD45 (LCA), desmin. RESULTS: (1) The most common histological pattern of Ewing's sarcoma/PNET was a lobular arrangement of uniform, small, hyperchromatic cells in a fibrous background. Some of these tumors were rich in cytoplasmic glycogen. (2) Of the 7 cases, IHC was positive for CD99 (12E7) and Vimentin and negative for lymphoid (CD45), muscle (desmin) markers. S-100 was positive in 4 cases and NSE negative in 3, Syn positive in 1. NSE, S-100, Syn were all negative in 2 cases. CONCLUSIONS: Ewing's sarcoma/PNET is more common in teenagers and young people. Immunohistochemistry is essential to distinguish Ewing's sarcoma/PNET from other small round cell tumors. Immunohistochemistry is useful in the diagnosis.

Adolescent↗
PubMed1992

[Olfactory esthesioneuroblastoma].

The AA. present 2 cases of olfactory Neuroblastoma in different stages of histologic differentiation, which are compared. Bibliographic survey of the entity.

Adult↗
PubMed1975

An experimental mouse testicular teratoma as a model for neuroepithelial neoplasia and differentiation. I. Light microscopic and tissue and organ culture observations.

The various stages of divergent neuroepithelial differentiation were studied in the solid transplants of a transplantable mouse testicular teratoma (OTT-6050) maintained in both ascitic and solid forms. They included: a) areas of undifferentiated medullary epithelium corresponding to the rare human medulloepithelioma; b) areas of neuroblastic differentiation corresponding to neuroblastoma, with more mature neuronal differentiation corresponding to ganglioneuroma or, when mixed with glial elements, to ganglioglioma; and c) more mature neuroglial areas resembling astrocytoma, oligodendroglioma or ependymoma, as well as more primitive areas corresponding to ependymoblastoma. In tissue culture using collagen-coated coverslips, astrocytic differentiation was found in the outgrowth zone after 15 days, confirmed by immunofluorescence with antibodies to an astroglia-specific protein. In organ culture systems, glial components, including ependymal structures, were preserved in tumor explants, and astrocytic differentiation, as expressed by glial fiber formation, was increased after 4 to 6 weeks in vitro. No neuronal differentiation was demonstrable, however. The neuroepithelial component of this experimental teratoma may provide a model for the study of neoplastic neuroepithelial differentiation.

Animals↗
PubMed1991

[Anterior craniofacial resection in the treatment of malignant nasal-paranasal sinus tumors with intracranial extension].

Nasal and paranasal sinus tumors extending through the cribriform plate to the overlying dura of the frontal lobe can be successfully treated by anterior craniofacial resection. During the period from 1986 to 1990, 14 patients (11 males, 3 females, age 18-67) with nasal/paranasal tumors extended to the nasal basis underwent craniofacial resection. From a histological point of view these patients were classified as follows: --8 adenocarcinomas --2 squamous cell carcinomas --2 esthesioneuroblastomas --1 cylindroma --1 haemangiopericytoma. In 6 of the 14 patients post-operative complications were encountered, 3 being resolved. Radical surgery was realised in 12 cases; post-operative radiotherapy was performed in 7. No contraindications were found because of previous chemo- and/or radiotherapy, even if it had been performed as treatment. Four of the patients died because of recurrences between the 6th and 20th month after surgery; 5 patients (all adenocarcinoma subjects) are disease-free respectively 8, 16, 17, 22 and 24 months after surgery.

Adenocarcinoma↗
PubMed2006

[Application of fluorescence in-situ hybridization and reverse transcription-polymerase chain reaction in molecular diagnosis of Ewing's sarcoma and primitive neuroectodermal tumor].

OBJECTIVE: To detect tumor specific chromosome translocations and associated fusion transcripts in paraffin-embedded tissue by interphase fluorescence in-situ hybridization (FISH) and reverse transcription polymerase chain reaction (RT-PCR), respectively, and to evaluate their diagnostic values for Ewing's sarcoma/primitive neuroectodermal tumor (ES/PNET). METHODS: Nuclei of the tumor cells and total RNA were extracted from 10 cases of ES/PNET. Interphase FISH was utilized to analyze the EWS gene translocation with a dual color, break apart probe (Vysis company). RT-PCR was used to detect t (11; 22) (q24; q12) and t (21; 22) (q22; q12) fusion transcripts. RESULTS: Among 10 cases of ES/PNET, the EWS-FLI1 fusion transcript was detected in 8 by RT-PCR. EWS-ERG fusion transcript was not detected in any of the cases. EWS gene translocation was found in 9 of 10 cases by FISH. CONCLUSIONS: Interphase FISH and RT-PCR can be reliably applied to paraffin-embedded tissues for molecular diagnosis of ES/PNET. Between the two approaches, interphase FISH provides a more sensitive and stable result.

Adolescent↗
PubMed1990

Expression of the neuronal form of pp60c-src in neuroblastoma in relation to clinical stage and prognosis.

The expression of the protooncogene c-src has been studied in specimens of childhood tumors with special reference to neuroblastoma and other tumors of neuronal origin. For comparison c-src gene expression was studied in seven neuroblastoma and neuroepithelioma cell lines. The structurally distinct neuronal product of the gene, pp60c-srN, expressed during normal development in neuroblasts and neurons, was identified by immunoblotting technique together with the fibroblast form, pp60c-src. While pp60c-src was found in most tumors studied, the neuronal form was restricted to neuroblastomas (23 of 27) and retinoblastomas (3 of 3) and could not be detected in the other childhood tumors. A dominance of the neuronal form, pp60c-srcN, was exclusively found in the infant cases of neuroblastoma (9 of 12), estimated to have good prognosis. These results indicate that pp60c-srcN might be a diagnostic marker in primitive childhood tumors. When expressed in higher amounts than pp60c-src, pp60c-srcN may be a positive prognostic marker in neuroblastoma, especially useful in the evaluation of infants. In addition, lack of pp60c-srcN seems to be incompatible with low stage neuroblastoma.

Aging↗
PubMedDate not supplied

[Askin tumor. Consideration on a case].

The authors present the case of a male patient of 9 years of age admitted for thoracic pain. The clinical evaluation and the imaging exams (chest X-ray, ultrasonography exam of the chest and thoracic computer tomography) reveal a tumor of the thoracic wall. The child was referred to the surgery. It was revealed an invasive tumoral mass of bone origin. At the pathological exam the tumor presented an aspect of Askin tumor. The authors discuss the theoretical aspects correlated with the diagnosis of Askin tumor, based on the presented case.

Child↗
PubMed1975

An experimental mouse testicular teratoma as a model for neuroepithelial neoplasia and differentiation. II. Electron microscopy.

The electron microscopic features of the stages of divergent neuroepithelial differentiation in the solid implants of a transplantable mouse testicular teratoma (OTT-6050) are presented and compared to the sequential stages of cytogenesis that have been described in the developing avian and mammalian central nervous system. Primitive neuroepithelial tumor cells showed the features of undifferentiated multipotential matrix (or ventricular) cells of the neural tube. They formed primitive medullary rosettes, from which various transitions were traced to more differentiated, cilia-containing ependymoblastomatous rosettes; the transitional features included increased granular endoplasmic reticulum and microvilli formation. Glial differentiation was characterized by the presence of mature ependymal rosettes and of astrocytes containing glial filaments. Neuronal differentiation included the development of synapses and the presence of dense-core vesicles in nerve cell processes. No intermediate cell forms were found that suggested multiple lines of differentiation occurring within a single cell.

Animals↗
PubMed1992

Expression of p53 in human neuroblastoma- and neuroepithelioma-derived cell lines.

Overexpression of the nuclear phosphoprotein p53 has been detected in many different transformed human cell lines and primary adult tumors. Elevated steady-state levels of p53 appear to be the result of an increase in the stability of the protein and, in adult cancers, high levels of the protein are associated with mutation of the p53 gene. In this study, overexpression of p53 was detected in 4 out of 5 human neuroblastoma-derived cell lines. The protein expressed by each of these four lines had a significantly prolonged half-life relative to the p53 protein in immortalized rodent fibroblasts and normal bovine adrenal medullary cells. However, no mutations were detected in the highly conserved regions of the p53 gene in these four neuroblastoma lines and the protein being expressed was not recognized by the mutant-specific anti-p53 monoclonal antibody, PAb 240. Upon retinoic acid-induced differentiation of the LA-N-5 neuroblastoma cell line, the level of p53 protein declined, as did the level of p53 mRNA, but the half-life of the protein remained unchanged. The high level of protein observed in the undifferentiated cell lines appears to result from expression of a stable wild-type p53 protein and increased transcription. In contrast, p53 protein was undetectable in two neuroepithelioma-derived cell lines; the p53 gene in one of these lines contained a nonsense mutation, while the other transcribed truncated p53 mRNA.

Base Sequence↗