SEARCH · Search PubMed
Results for “Neuroectodermal Tumors, Primitive, Peripheral”
Search indexed PubMed citations on genomics, clinical trials, systematic reviews and public health. Explore titles, authors and supplied subject terms, then open the PubMed record.
Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.
Esthesioneuroblastoma presenting as an intracranial mass.
Explore the source record for details and available documents.
Diagnosis and treatment of pediatric brain tumors.
Brain tumors are the most common form of solid neoplasm of childhood. Progress is clearly being made in the understanding and management of childhood brain tumors. There are possible biologic differences between histologically similar childhood and adult brain tumors. Chemotherapy is a major component of the treatment of many forms of childhood primary central nervous system tumors and is being used in attempts to improve survival and to delay or decrease the amount of radiotherapy needed. Despite the use of more aggressive radiotherapy approaches or intensified chemotherapy approaches, the outcome remains dismal for some forms of brain cancer. Many unsettled issues exist concerning the management of childhood low-grade gliomas.
Morpho-immunophenotypic-genotypic infidelity in synovial sarcoma.
Explore the source record for details and available documents.
Cytogenetic and molecular evaluation of clinically aggressive esthesioneuroblastoma.
We report a 16-year-old boy with esthesioneuroblastoma that presented with a unilateral tumor extending to the maxillary sinus and periorbital region. Despite initial therapy with gross resection, 5,682 cGy to the tumor bed and chemotherapy, the patient subsequently had a rapid local recurrence with distant metastases. Immunocytochemical, ultrastructural, cytogenetic, and molecular techniques were performed to determine if this tumor was biologically similar to childhood neuroblastoma. Urinary excretion of vanillylmandelic acid (VMA) and homovanillic acid (HVA) were markedly elevated. Chromogranin and neuron specific enolase immunostaining of tumor cells was positive, as seen in neuroblastoma. Electron microscopic studies showed cells that were closely packed and connected by occasional cell junctions. The cell cytoplasm contained moderate amounts of filaments and microtubules. Numerous electron dense granules were observed; however, these granules lacked distinct nucleoids and generally reacted strongly for acid phosphatase, indicating a lysosomal rather than a secretory function. Tumor cells contained near-pseudotetraploid chromosomes, with all chromosomes represented at least three times, and chromosome 5 was present in multiples of eight. Clonal structural abnormalities included 2q+ and 5q+ and multiple double minutes. Northern blot analysis revealed both c-myc and N-myc expression; however, N-myc amplification was not demonstrated, and c-myc expression appeared increased, unlike cases of rapidly progressive neuroblastoma. These results suggest that despite biologic similarities to neuroblastoma in catecholamine excretion and some ultrastructural features, molecular genetic abnormalities differ in this comparatively aggressive case of estesioneuroblastoma.
Therapy associated changes in childhood tumors.
Contemporary treatment regimens for the common solid tumors of childhood have led to increased numbers of post-treatment pathologic specimens from survivors. Current therapeutic strategies for childhood cancers in North America require an accurate pathologic diagnosis and stratify patients based on combinations of clinical, biological, and pathologic features. In several tumor systems, the pathologic response to therapy also modifies the treatment regimen. Accurate pathologic interpretation of such specimens is critical in providing useful prognostic information for therapeutic decisions. Standardized handling of post-therapy pathologic specimens, appropriate use of molecular and genetic studies, consideration of the differential diagnoses, and assessment of the potential biologic significance of therapy-induced pathologic changes are, therefore, critical for patient management and determination of treatment protocols.
Ewing sarcoma/peripheral primitive neuroectodermal tumor: adult abdominal tumors with an Ewing sarcoma gene rearrangement demonstrated by fluorescence in situ hybridization in paraffin sections.
The differential diagnosis of small round cell tumors is exhaustive and requires ancillary studies. Relatively recently, fluorescence in situ hybridization (FISH) using probes for specific gene rearrangements has gained wide acceptance. This technique is particularly useful in the differential diagnosis of Ewing sarcoma/primitive neuroectodermal tumor (ES/PNET) and desmoplastic small round-cell tumor (DSRCT). In ES/PNET, the EWS gene is juxtaposed to the FLI-1 gene in 85% of cases and to the ERG gene in another 7% of cases; the EWS gene is juxtaposed to the WTI gene in DSRCT. Documentation of the EWS gene rearrangements in EWS/PNET has previously been demonstrated in frozen tissue. We report 2 unusual cases of EWS/PNET diagnosed in abdominal tumors in adults. Although the immunohistochemical results supported a diagnosis of ES/PNET, 1 case morphologically resembled DSRCT. The diagnosis in these 2 cases was confirmed by the FISH demonstration of EWS/FLI-1 gene fusion in paraffin-embedded tissue. Thus, the usefulness of FISH demonstration of an EWS gene rearrangement with these specific probes in such unusual cases is supported and is demonstrated in paraffin-embedded tissue.
Primitive neuroectodermal tumor of the prostate.
Explore the source record for details and available documents.
Tumor heterogeneity affects the precision of microarray analysis.
Microarray-based analysis of global gene expression patterns defines groups of genes that correlate with specific tumor types and prognosis, but the identified genes may not all be of equal clinical utility due to technical factors that affect the precision of their measurement. To analyze how technical variability in measured expression levels may impact microarray-based analysis in a clinical setting, we used Ewing sarcoma/peripheral neuroectodermal tumor (EWS/PNET) in a model system that replicates the clinical scenario in which microarray-based analysis of gene expression will likely occur, namely analysis of a fresh tumor sample by a single chip. By comparing variability of measured expression due to purely technical factors with variability due to biologic factors, we confirm that variability is dependent on the level of gene expression. We also demonstrate that the variability in expression level from either cell line or tumor samples is significantly higher than can be attributed to specific probe sets that have an intrinsically poor performance. These results have significant impact on the application of cDNA microarray chip for molecular analysis performed in a clinical setting.
Molecular diagnosis of Ewing's sarcoma/primitive neuroectodermal tumor in formalin-fixed paraffin-embedded tissues by RT-PCR and fluorescence in situ hybridization.
Recent studies have suggested that formalin-fixed paraffin-embedded (FFPE) tissues can be used for molecular analyses by fluorescence in situ hybridization (FISH) and RT-PCR. We analyzed 18 cases of ES/PNET for the t(11;22)(q24;q12) and t(21;22)(q22;q12) fusion transcripts by RT-PCR and analyzed for EWS translocation by interphase FISH with a dual color fusion probe to compare these two approaches directly. RT-PCR detected 13 (72%) EWS-FLI-1 fusions (type I=10, type II=3) and 2 (11%) EWS-ERG fusions. Three cases could not be evaluated because the housekeeping gene phosphoglycerate kinase (internal mRNA control) was not amplified. FISH was diagnostic in 15 of 18 cases (83%). There were three discordant cases between RT-PCR and FISH (concordance of 83%). Using a combination of RT-PCR and FISH, the results were complementary. One advantage of RT-PCR analysis was that subtypes of EWS translocation could be determined specifically (type I, type II and ERG). These findings indicate that because of the difficulties and limitations associated with the molecular analysis of FFPE tissues, a combination of RT-PCR and FISH may be a better approach to enhance the sensitivity and accuracy of detecting ES/PNET translocations in FFPE tissues with suboptimally preserved nucleic acids.
Clearance of unusual muscular uptake of F-18 FDG in the lower lumbar region on a repeat FDG-PET scan after oral chlorzoxazone intervention.
Explore the source record for details and available documents.
Cytokine production in five tumor cell lines with activity to induce cancer cachexia syndrome in nude mice.
To identify the so-called toxohormone, which is a tumor-derived factor with activity to induce cancer cachexia syndrome in tumor-bearing animals, 5 human cancer cell lines with this activity were studied for cytokine production. Tumor cell products with activity to inhibit lipoprotein lipase (LPL) were shown to play an important role in the development of the cancer cachexia syndrome. All culture media conditioned by the 5 cell lines possessed LPL-inhibitory activity. However, the activity differed with the cell line. In order to characterize the activity, we examined whether the cultured cells produced cytokines with activity to inhibit LPL. A melanoma cell line, SEKI, and a neuroepithelioma cell line, NAGAI, were found to express a large amount of leukemia inhibitory factor (LIF) mRNA. Furthermore, both of these cell lines were demonstrated to produce a large amount of LIF protein, and plasma levels of LIF were extremely elevated in SEKI- and NAGAI-bearing nude mice, indicating that LIF produced by the tumor cells induced cancer cachexia syndrome in the animals. Thus, LIF fulfills the requirements for a toxohormone, except for suppressive activity on liver catalase. In contrast, the mechanisms responsible for cachexia in the MKN-1-, LX-1- and LS180-bearing mice remain unknown. These findings suggest that various types of bioactive substances produced by cancer cells could be toxohormones.
Expression of pericyte, mesangium and muscle markers in malignant rhabdoid tumor cell lines: differentiation-induction using 5-azacytidine.
Malignant rhabdoid tumor (MRT) has been considered to have multiphenotypic diversity characteristics. Some MRTs exhibit a neural phenotype. However, it is still unclear whether MRT cells can display a skeletal muscle, smooth muscle or smooth muscle-like cell phenotype, like those of pericytes and mesangial cells. To determine if MRTs exhibit skeletal muscle cell or smooth muscle-like cell phenotypes, six MRT cell lines (TM87-16, STM91-01, TTC549, TTC642, YAM-RTK1 and TTC1240) were examined for markers of skeletal muscle (MyoD, myogenin, myf-5, myf-6, acetylcholine receptor-alpha, -beta and -gamma), smooth muscle (alpha-smooth muscle actin, SM-1 and SM22), and smooth muscle-like cells, such as pericytes (angiopoietin-1 and -2) and mesangial cells (megsin), using conventional RT-PCR, semi-quantitative PCR, western blotting and immunocytochemistry before and after differentiation-induction with 5-azacytidine. alpha-Smooth muscle actin and SM22 were detected in all six MRT cell lines, while MyoD and myf-5, crucial markers for skeletal myogenic determination, were not. The TM87-16 cell line expressed SM-1 and angiopoietin-1. TTC1240 also expressed angiopoietin-1. Interestingly, STM91-01 expressed megsin, a novel marker for mesangial cells, in addition to angiopoietin-1. Our results indicated that some MRTs exhibited smooth muscle and/or smooth muscle-like cell phenotypes and some renal MRTs might be of mesangial origin. Recently, smooth muscle and also smooth muscle-like cells have been considered to be of neuroectodermal origin. MRT can thus considered to belong to the category of primitive neuroectodermal tumors (PNETs) in the broad sense.
Olfactory neuroblastoma: imaging by magnetic resonance, CT and conventional techniques.
Twenty-four patients with the histological diagnosis of olfactory neuroblastoma have been treated at the Royal National Throat, Nose and Ear Hospital since 1975. The tumour showed a bimodal age distribution and 30% of the patients were under 30 years of age. The imaging characteristics on plain film, computed tomography and magnetic resonance combined with intravenous gadolinium DTPA are detailed. None of the changes described is wholly specific. However, a tumour in the ethmoids and upper part of the nasal cavity, which expands into the orbit and erodes the roof of the fronto-ethmoid complex or cribriform plate unilaterally in a young patient, is highly suggestive of olfactory neuroblastoma, particularly if this is combined with the magnetic resonance signal characteristics of a vascular tumour. The typical MR features are those of an intense signal on pre-contrast T2 weighted spin echo sequences and strong enhancement after gadolinium on T1 weighted sequences. A characteristic feature of the response to gadolinium is an enhancement of tumour higher than that of turbinate mucosa on inversion recovery and less than that of mucosa when T1 weighted spin echo sequences are employed. The extent of tumour in the paranasal sinuses and anterior cranial fossa is best demonstrated after magnetic resonance with intravenous gadolinium and this is now regarded as the most accurate method of preoperative assessment of these patients prior to craniofacial surgery.
Primitive neuroectodermal (neuroepithelial) tumour of soft tissue of the neck in a child: demonstration of neuronal and neuroglial differentiation.
A 4-year-old girl had a pathologically proven primitive neuroectodermal (neuroepithelial) tumour of soft tissue in the left posterolateral aspect of the neck. The neoplasm consisted of primitive neuroepithelial cells forming Homer Wright rosettes, mature ganglion cells and astrocytes. Astroglia were identified by localization of cytoplasmic glial fibrillary acidic protein (GFAP). Striking similarity is noted between the current tumour and those found in the central nervous system, including cerebellar medulloblastomas. The diverse cellular elements of the present primitive neuroectodermal neoplasm suggest an origin of the tumour from the neuroectodermal component of an ectomesenchymal remnant of the neural crest. Differentiation of the neuroectodermal component of the neural crest into primitive neuroepithelial cells could result in the occurrence of a primitive neuroectodermal neoplasm which may further differentiate into neurons and neuroglia.
Intracranial olfactory neuroblastoma mimicking carcinoma: report of two cases.
Two cases of olfactory neuroblastoma which presented clinically as intracranial lesions are described. Prominent features of epithelial differentiation were present, which led to initial diagnoses of poorly differentiated carcinoma. The true nature of the lesions was only established subsequently by careful histological examination, immunohistochemistry and electron microscopy. The potential towards epithelial differentiation in such tumours was emphasized and certain new histological features were described, including a biphasic epithelial and stromal pattern, papillae formation and positive staining for cytokeratin. These two cases underline the importance of exhaustive examination of poorly differentiated epithelial-like lesions of the frontal lobes by conventional histology, immunohistochemistry and electron microscopy.
Rhabdomyoblasts in olfactory neuroblastoma.
Explore the source record for details and available documents.