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Results for “Neuroectodermal Tumors, Primitive, Peripheral”

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At least 613 records · Page 34Linked to original sources
PubMed1993

Human FLI-1 localizes to chromosome 11Q24 and has an aberrant transcript in neuroepithelioma.

The v-ets oncogene family shares a conserved motif, termed the ETS-domain, that mediates sequence-specific DNA binding. This motif is unique among transcription factor families. Using partially degenerate oligonucleotides to highly conserved amino acids in this motif as primers for the polymerase chain reaction, a novel ETS-domain cDNA fragment was generated. This fragment was subsequently used to clone both mouse and human full length cDNAs for this gene. The amino acid sequence of the longest open reading frame showed that this gene was homologous to the mouse FLI-I gene, an ETS family gene activated by Friend erythroleukemia virus insertion. The gene is normally expressed only in hematopoietic cells. The gene was localized to chromosome 11q24, a region of aberrations in Ewing's sarcoma and neuroepithelioma. In the neuroepithelioma cell line TC-32 the FLI-1 transcript is present but has an aberrant structure, indicating that it may be rearranged in neuroepithelioma.

Amino Acid Sequence↗
PubMed1988

Rhabdomyosarcoma of the maxillary sinus: review of the literature and report of a case.

A case of rhabdomyosarcoma of the maxillary sinus is reported. A brief review of the origin, histomorphology, classification, clinical presentation, behavior, prognosis, and treatment is given relative to occurrence in the maxillary antrum. Misinterpretation of the microscopic findings can delay the histologic diagnosis. An error in tissue diagnosis may be minimized by awareness of a chance encounter with this lesion.

Adult↗
PubMed1981

Influence of ethyl alcohol on carcinogenesis with N-nitrosodimethylamine.

This paper presents the results of an experiment on the combined action of nitrosodimethylamine and ethyl alcohol in C57BL mice. As shown, alcohol can act to change the target organ of that liver carcinogen by favouring development of olfactory neuroepitheliomas, which infiltrate the frontal lobe of the brain.

Animals↗
PubMed1988

Esthesioneuroblastoma: a comparison of two treatment eras.

This paper is a retrospective review of 30 patients with esthesioneuroblastoma, an uncommon malignancy of the upper nasal cavity, treated at a single institution from 1959 through 1986. Over the period of study, there has been a gradual evolution of treatment policy and technique with the introduction of craniofacial resections and complex field megavoltage radiation, as well as for Stage C disease, the addition of chemotherapy to radiotherapy and surgery. The 25 patients with a 2 year minimum follow-up are divided into 2 groups depending upon treatment era to determine the impact of modern aggressive therapy upon treatment results. Two-year survival for Group I (1959-1975) was 70% as compared with 87% for Group II (1976-85). For Stage C disease, there was a definite improvement in survival in the later era (88% versus 50%), although relapses did not appear to be circumvented. Salvage therapy has an important role in prolonging survival in this disease.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed1993

Ethmoidal cancers: a retrospective study of 22 cases.

From April 1978 to June 1990, 22 patients with ethmoidal cancer were treated at Fondation Bergonié by a combination of surgery and radiation therapy. The mean age was 59.6 years (range 34-79 years) and the sex ratio is 2.7 (16 males/6 females). Histologic types were: adenocarcinoma, 13 cases; squamous carcinoma, 4 cases; undifferentiated carcinoma, 3 cases and esthesioneuroblastoma, 2 cases. Exposure to wood dust was encountered in 11 patients, especially in cases of adenocarcinoma: 10/13 (77%). Staging according to the classification of the University of Florida was: Stage I, 10 patients; Stage II, 5 patients and Stage III, 7 patients. Resection was considered as complete in 16 cases and only one orbital exenteration was performed. The postoperative radiation therapy delivered a mean given dose of 55.7 Gy (range 50-70 Gy) expressed to the hot spot using a technique adapted to tumor location and extension. Complete remission was achieved in 20 cases. Median follow-up is 28 months. The 5-year overall and disease-free survival are 44% and 38%, respectively. Analysis of recurrences according to staging gives: 5/10 Stage I, 2/5 Stage II and 5/7 Stage III. Recurrence is pejorative since death occurs in all cases within an average of 6 months following salvage treatment, except for three patients still alive within less than 6 months and in second remission. Prognosis of ethmoidal cancer depends on staging and local control.

Adenocarcinoma↗
PubMed1994

Sarcomas of the hand and foot: analysis of local control and functional result with combined modality therapy in extremity preservation.

PURPOSE: The records of 28 patients with sarcomas of the hand and foot treated at the National Cancer Institute (NCI) between 1977 and 1992 were reviewed to assess local control and functional results. METHODS AND MATERIALS: Histologic types included 15 cases of the Ewing's sarcoma family of tumors, 7 cases of alveolar rhabdomyosarcoma, and 6 cases of nonrhabdomyosarcoma soft tissue sarcomas. Median age of all patients was 18 years (range 4-61), with a median potential follow-up of 114 months following diagnosis. Surgery varied from incisional biopsies for Ewing's Sarcoma and rhabdomyosarcoma lesions to complete excision when possible for nonrhabdomyosarcoma soft tissue sarcoma lesions. Amputation was not primarily performed, except in two patients who underwent ray resections of hand lesions (patients 13 and 24). Radiotherapy generally consisted of 50 Gy/25 fractions (fx)/5 weeks for Ewing's Sarcoma, 54 Gy/30 fx/6 weeks for rhabdomyosarcoma, and 63 Gy/35 fx/7 weeks for nonrhabdomyosarcoma soft tissue sarcomas. Chemotherapy was administered on various NCI protocols. RESULTS: Actuarial local control for Ewing's Sarcoma was 84% at 5 and 10 years. All but one survivor are capable of hand/foot function for routine activities without orthotic requirements. Five of six patients (83%) who died of metastatic disease had functional distal extremities. Actuarial local control for rhabdomyosarcomas was 100%, with equivalent function. No patient developed a second malignancy in the treatment field. CONCLUSIONS: Although equivalent local control may be achieved in these lesions with either amputation or radiotherapy, a prudent management course would be to defer amputation for management of local recurrences. Many patients with these lesions fail in distant sites only and die without local failure. For these patients and for those who remain long-term survivors, we believe a functional hand and foot provides a better quality of life than a prosthesis.

Adolescent↗
PubMed1990

The neuroepithelioma breakpoint on chromosome 22 is proximal to the meningioma locus.

The recurrent translocation breakpoint on chromosome 22 of neuroepithelioma has been localized between two probes, D22S1 and D22S15, by both in situ hybridization and somatic cell hybrids. These two probes have further been shown to be genetically linked at theta = 0.0 and a lod score of 5.3. The two probes were unaffected by a partial deletion of the chromosome 22 long arm of a meningioma, showing that the meningioma locus is distal to that of the neuroepithelioma.

Blotting, Southern↗
PubMed1996

Endogenous beta 3- but not beta 1-adrenergic receptors are resistant to agonist-mediated regulation in human SK-N-MC neurotumor cells.

Although there is considerable interest in the regulation of the different beta-adrenergic receptor (AR) subtypes, most previous studies have utilized stably transfected cells expressing recombinant receptors under the control of viral promoters. Human SK-N-MC neurotumor cells appear to be novel, since they express both endogenous beta 1AR and beta 3AR based on radioligand binding and on functional response. Saturation binding of either the hydrophilic ligand (-)-[3H]CGP-12177 or the more hydrophobic (-)-[125I]iodocyanopindolol indicated the presence of two populations of binding sites with high and low affinities. With either ligand, the beta 1AR antagonist CGP-20712A preferentially inhibited binding to the high-affinity sites. This is consistent with the latter representing beta 1AR whereas the low-affinity sites represent beta 3AR. Both subtypes appeared to be functional on the basis of isoproterenol stimulation of cyclic adenosine monophosphate (cAMP) in intact cells and adenylyl cyclase activity in cell membranes in the absence and presence of CGP-20712A. SK-N-MC-IXC cells, derived by twice subcloning the parental cells, also expressed both beta AR subtypes, indicating that they co-exist in the same cell. SK-N-MC cells exposed to isoproterenol exhibited a rapid sequestration and a slower downregulation of beta 1AR. The latter subtype also underwent desensitization, as indicated by a rightward shift to less sensitivity in the EC50 for isoproterenol stimulation of adenylyl cyclase activity. In contrast, the beta 3AR subtype was resistant to agonist-mediated sequestration, downregulation, and desensitization. Thus, when endogenously expressed in the same cell line, human beta 1AR and beta 3AR display differences in their ability to be regulated by agonist.

Adrenergic beta-1 Receptor Agonists↗
PubMed1996

Intra- and intercellular calcium signaling in human neuroepithelioma cells.

The characteristics of intra- and intercellular Ca2+ signaling in human SK-N-MCIXC neuroepithelioma cells have been examined by means of Fura-2 digital imaging microfluorimetry. When cells were exposed to maximally effective concentrations of either endothelin-1, ATP, norepinephrine or oxotremorine-M, the Ca2+ signals that accompany an increase in phosphoinositide turnover could be differentiated on the basis of their magnitude, shape and duration. When individual cells were microinjected with inositol 1,4,5-trisphosphate, a rise in [Ca2+]i was observed not only in the target cell, but also in neighboring cells. This intercellular propagation of Ca2+ signals was found to be mediated via the release of nucleotide di- and triphosphates which subsequently activate purinergic receptors linked to Ca2+ homeostasis on neighboring cells. These results indicate: (1) that agonist-specific Ca2+ 'signatures' are generated in SK-N-MCIXC cells; and (2) that an intercellular propagation of Ca2+ signals is triggered by a rise in [Ca2+]i.

Calcium↗
PubMed1989

Beam's-eye-view aided treatment planning for a nasopharyngeal lesion: a case report.

We report on the application of CT-based multilevel treatment planning to achieve complete and uniform dose distribution over the entire target while sparing critical structures. Treatment strategy and parameters are chosen on the slice containing the isocenter. Target coverage and organ sparing is achieved on all other slices by independently adjusting the asymmetric field width at each level, stimulating the effects of custom blocks. The optimized field borders are back projected using beam's eye views (BEV) from each treatment angle. The BEV printouts are used to assist the physician in the delineation of field blocking on the simulation films.

Humans↗
PubMed1995

Is neuro-ectodermal differentiation of Ewing's sarcoma of bone associated with an unfavourable prognosis?

Among Ewing's sarcoma (ES) of bone and related entities are tumours with neuro-ectodermal features that could represent a biologically distinct type. In order to assess the prognostic significance of the various forms of ES, a retrospective joint study involving three cancer centres in Europe and the U.S.A. was initiated. The material from 315 primary ES was reviewed by a panel of five pathologists and classified as typical ES (220 cases), atypical ES (48 cases) or ES with neuro-ectodermal features (47 cases). Prognostic factor analysis on treatment failure-free survival was performed using the Cox model. It included histopathological classification, initial patient characteristics, clinical presentation and treatment type. After multivariate analysis, in addition to treatment type (P < 0.001), metastases (P = 0.003) and proximal tumour location (P = 0.006), two histopathological parameters correlated with poor treatment failure-free survival, the presence of filigree pattern (P = 0.044) and dark cells (P = 0.043). We conclude that ES with neuro-ectodermal features does not appear to have a different outcome to the other subtypes.

Adolescent↗
PubMed2005

Examination of the distribution of the transferrin homologue, melanotransferrin (tumour antigen p97), in mouse and human.

Melanotransferrin (MTf) is a transferrin homologue initially identified in melanoma cells. Serum transferrin (Tf) contains two iron (Fe)-binding sites and plays a vital role in Fe transport. However, human MTf has only a single, high affinity, Fe-binding site. Furthermore, while isolated MTf can bind Fe, it plays little role in Fe uptake by cells and its function remains elusive. To further understand the biological role of this molecule, we examined the expression profile of mouse MTf (mMTf) and human MTf (hMTf) and the splice variant of the latter. Analysis of mMTf in 18 normal mouse tissues and 4 embryonic stages (7-17 days) using an RNA dot blot demonstrated it was expressed at high levels in the pancreas, salivary gland and epididymis of the adult, while embryonic tissues showed low expression. The expression pattern was very different from that of mouse transferrin receptor 1 (TfR1) mRNA, which was found at high levels in the spleen and embryo. Using the more sensitive RT-PCR technique, mMTf expression was demonstrated across all 24 normal mouse tissues assessed. Analysis of the mMTf genomic sequence predicted only one mMTf transcript, although two putative transcripts were found in the testis using Northern blotting. An alternate hMTf transcript, h delta MTf, has been identified by others, although its tissue distribution was not previously examined. In human heart and skeletal muscle, three putative hMTf transcripts were identified at approximately 2, 3 and 4 kb, the smallest transcript being consistent with h delta MTf. The two larger transcripts were also found in 10 other human tissues. The h delta MTf transcript was detected using RT-PCR and Southern blotting in tumour-derived cell lines, with the highest expression being identified in melanoma cells. Immunohistochemistry showed that hMTf was expressed primarily within epithelia. In fact, the most pronounced expression was within the epidermis of the skin, tubules of the kidney and the ducts of sweat and salivary glands. The distribution of MTf and its splice variants may provide clues to their possible biological roles.

Alternative Splicing↗
PubMed2006

Investigating biological activity spectrum for novel quinoline analogues.

The lack of the wide spectrum of biological data is an important obstacle preventing the efficient molecular design. Quinoline derivatives are known to exhibit a variety of biological effects. In the current publication, we tested a series of novel quinoline analogues for their photosynthesis-inhibiting activity (the inhibition of photosynthetic electron transport in spinach chloroplasts (Spinacia oleracea L.) and the reduction of chlorophyll content in Chlorella vulgaris Beij.). Moreover, antiproliferative activity was measured using SK-N-MC neuroepithelioma cell line. We described the structure-activity relationships (SAR) between the chemical structure and biological effects of the synthesized compounds. We also measured the lipophilicity of the novel compounds by means of the RP-HPLC and illustrate the relationships between the RP-HPLC retention parameter logK (the logarithm of capacity factor K) and logP data calculated by available programs.

Antifungal Agents↗
PubMed2004

Two successful spontaneous pregnancies in a patient with a primary primitive neuroectodermal tumor of the ovary.

OBJECTIVE: To describe a patient with primary primitive neuroectodermal tumor of the ovary with two successful spontaneous pregnancies. DESIGN: Case report. SETTING: Tertiary center for gynecologic oncology. PATIENT(S): A 25-year-old woman with two spontaneous pregnancies 5 months after and 2 years after conservative treatment of International Federation of Gynecology and Obstetrics stage IC primary primitive neuroectodermal tumor of the ovary. INTERVENTION(S): Assessment of extraovarian spread with staging laparotomy. Four courses of BEP (bleomysin, etoposide, cisplatin) and, for recurrent disease, six courses of salvage VIP (vinblastin, iphosphamide, mesna, cisplatin) chemotherapy. MAIN OUTCOME MEASURE(S): Two successful deliveries and no residual ovarian cancer. RESULTS(S): A healthy, normal female infant weighing 3600 g was delivered by cesarean section at 38 weeks' gestation. Sixteen months later another infant, a healthy, normal male weighing 3500 g, was delivered by cesarean section at 38 weeks' gestation. No residual cancer was detected at follow-up 12 months after the last delivery. CONCLUSION(S): Conservative fertility-preserving treatment might be considered in patients with primary primitive neuroectodermal tumor of the ovary. Without any assisted reproductive technologies, spontaneous pregnancies might occur.

Adult↗
PubMed2006

Immunohistochemistry of primary malignant neuroepithelial tumors of the kidney: a potential source of confusion? A study of 30 cases from the National Wilms Tumor Study Pathology Center.

Ewing sarcoma/peripheral primitive neuroectodermal tumor (pPNET) is a rare primary tumor of the kidney with morphologic features similar to those of other primitive tumors. Previous studies have shown that these tumors frequently stain positively with immunostains against CD99 and FLI-1 and negatively with stains against WT-1, suggesting that these markers may be used for the distinction between Wilms tumor and pPNET. We present 30 cases of primary malignant neuroepithelial tumor with immunohistochemical profiles and reverse transcriptase polymerase chain reaction (RT-PCR) analysis and show that immunophenotypic overlap exists between Wilms tumor and pPNET. A subset of 30 neuroepithelial tumors from the National Wilms Tumor Study originally categorized as putative pPNETs of the kidney was stained with FLI-1, WT-1, and thyroid transcription factor-1. Bicolor fluorescence in situ hybridization studies were performed on 19 of the cases. Other data on these tumors were available from a previous study (Am J Surg Pathol 2001;25:133). Of 7 primary tumors that had the EWS/FLI-1 fusion transcript by RT-PCR, 6 exhibited strong immunopositivity for FLI-1. Nine that were negative by RT-PCR stained positively with the FLI-1 stain. Five fusion-negative cases stained with both FLI-1 and WT-1. Three fusion-negative cases were negative for FLI-1 but positive for WT-1. Five fusion-negative cases were negative for both FLI-1 and WT-1. Of the 30 cases, 29 were positive for CD99. Seven cases that were negative for the EWS-FLI-1 fusion by RT-PCR were positive by fluorescence in situ hybridization. All cases were negative for thyroid transcription factor-1. Reliance upon immunohistochemistry as the sole means of ancillary diagnosis in renal pPNET can lead to confusing results. We recommend molecular fusion studies for clarification of primitive renal tumors with unexpected immunophenotypic results.

Adult↗