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  • 1
    ISSN: 1432-1203
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology , Medicine
    Notes: Abstract Yolk sac tumors are the most frequent kind of malignant pediatric germ cell tumor and may have a fundamentally different pathogenesis than adult germ cell tumors. Since few cytogenetic studies have been performed so far, in situ hybridization was applied to interphase cell nuclei of seven gonadal yolk sac tumors of childhood in routine paraffin-embedded tissue sections. The panel of chromosome-specific DNA probes was selected on the basis of their relevance in adult germ cell tumors and consisted of five DNA probes specific for the (peri)centromeric regions of chromosomes 1, 8, 12, 17 and/or X and/ or one DNA probe specific for the subtelomeric region of chromosome 1 (p36.3). As in adult germ cell tumors, all pediatric gonadal yolk sac tumors had an increased incidence of numerical chromosome aberrations. All tumors showed an overrepresentation of at least three chromosomes. Gains of chromosome 12, which is highly specific in adult germ cell tumors, were diagnosed in six pediatric gonadal yolk sac tumors. The DNA indices determined in the paraffin-embedded tumor material correlated well with the in situ hybridization findings. A chromosome was either over- or underrepresented, compared with the corresponding DNA indices, in only a few cases. The short arm of chromosome 1 in adult germ cell tumors is often involved in structural aberrations. In pediatric germ cell tumors, the short arm of chromosome 1 is also a nonrandom site of structural aberrations. Moreover, the presence of a deletion at 1p36.3 in four out of five tumors suggests that the loss of gene(s) in this region is an important event in the pathogenesis of gonadal yolk sac tumors of childhood.
    Type of Medium: Electronic Resource
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  • 2
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 118 (1974), S. 97-105 
    ISSN: 1432-1076
    Keywords: Cerebrospinal fluid proteins ; Childhood acute leukemia ; Blood-CSF barrier ; Electroimmunodiffusion
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung Bei kindern mit akuter lymphatischer Leukämie wurden parallel zur Bestimmung von Zellzahl und Gesamteiweiß Präalbumin, Albumin, α1-Antitrypsin, α2-Makroglobulin, Transferrin, β1C/β1A-Globulin, IgG und IgA im Liquor quantitativ erfaßt. IgM wurde qualitativ nachgewiesen. Die Untersuchung erstreckte sich auf Kinder, die noch keine Zeichen einer Meningosis leucaemica geboten hatten, an einer Meningosis erkrankt waren oder sich in einem erscheinungsfreien Intervall befanden. Eine signifikante Korrelation zwischen der Zellzahl und dem Gesamteiweiß ergab sich ebensowenig wie zwischen der Zellzahl und der Vermehrung einzelner Proteine. Eine meßbare Vermehrung α2-Makroglobulin und der qualitative Nachweis von IgM im Liquor deuteten auf eine erhöhte Permeabilität der Blut-Liquor-Schranke hin. Auf die Problematik der sogenannten normalen Liquorzellzahl wird an Hand der häufig gleichzeitig vorhandenen Erhöhung einzelner oder aller Eiweißkomponenten hingewiesen.
    Notes: Abstract In cerebrospinal fluid from 26 children suffering from acute lymphocytic leukemia or lymphosarcoma, prealbumin, albumin, α1-antitrypsin, α2-macroglobulin, transferrin, β1C/β1A-globulin, IgG, and IgA were estimated in correlation with cell count and total protein determinations. IgM was demonstrated by means of double diffusion. The cerebrospinal fluid (CSF) from 9 children with a first complete remission of bone marrow were compared with 10 patients suffering from central nervous system leukemia, and 10 more children who had achieved a further hematological as well as central nervous system remission. The results showed no significant correlation between cell count and protein content, nor between cell count and the increase of individual proteins. The increase of α2-macroglobulin, as well as the qualitative demonstration of IgM in the CSF, reflected an enhanced permeability of the blood-CSF barrier. We discuss the problems in determining a so-called normal CSF cell count due to a simultaneous increase in a single or all protein components, which was frequently the case.
    Type of Medium: Electronic Resource
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  • 3
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 118 (1974), S. 107-115 
    ISSN: 1432-1076
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung Es wurden die Liquores von 28 Kindern mit akuter lymphatischer Leukämie nach Bestimmung von Zellzahl und Gesamteiweiß auf Cellulose-Acetat-Folien elektrophoretisch aufgetrennt. Die Untersuchung umfaßte Kinder mit einer Meningosis, solche im symptomenfreien Intervall zwischen Meningosisrezidiven und Patienten, die nach der ersten hämatologischen Remission einer Meningosisprophylaxe unterzogen wurden. Als verwertbare kriterien für eine Schädigung der Blut-Liquor-Schranke ergaben sich eine Verminderung der Präalbuminfraktion sowie die gleichzeitige Zunahme des Albuminanteils. Als schwerste Form der Schrankenstörung zeigte sich ein serumgleiches Elektropherogramm des Liquors. Dieses Mischelektropherogramm beobachteten wir vorübergehend während der Meningosis leucaemica. Bleibt es bestehen, so gilt es als Hinweis auf eine schlechte Prognose. 4 der 28 Kinder behielten ein serumähnliches Elektropherogramm. Sie starben 2 bis 7 Monate nach Auftreten der ersten Veränderungen. Der Aussagewert der Liquorelektrophorese reicht prognostisch weiter als die Bestimmung von Zellzahl, Gesamteiweiß und einzelnen Proteinen.
    Notes: Abstract The cerebrospinal fluid of 28 children with acute lymphocytic leukemia were investigated by means of electrophoresis on cellulose-acetate membranes. The study included children with meningeal leukemia who were in the first or a further central nervous system remission; those who received “prophylactic” central nervous system therapy were also studied. In cases of acute leukemia, decreases of the prealbumin fraction and coincident increases of albumin as well as α2 globulins were found to be clinically important signs of a disturbed blood-CSF barrier, reversible or not. Changes in protein composition, which led to a pattern similar to that of serum, indicated very severe disorder of the barrier function, as was observed during meningeal leukemia. Prolongation of this pathological pattern points to an infaust prognosis. 4 patients who maintained CSF patterns resembling that of serum died 2 to 7 months after the first abnormalities were evident. CSF electrophoresis is of much greater diagnostic value than determination of the cell count, total protein content, and individual proteins.
    Type of Medium: Electronic Resource
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  • 4
    Electronic Resource
    Electronic Resource
    Springer
    Virchows Archiv 409 (1986), S. 223-239 
    ISSN: 1432-2307
    Keywords: Germ cell tumours of childhood ; Yolk-sac tumour ; Endodermal sinus tumour ; Teratomas ; Mixed germ cell tumours ; Immunohistochemistry
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary 170 germ cell tumours of childhood and adolescence were studied by light microscopy and immunohistochemistry. The male-to-female ratio was 1:1.3. 52 (30.6%) tumours were benign (mature teratoma), 30 (17.6%) potentially malignant (immature teratoma), and 88 (51.8%) unequivocally malignant. The main locations were ovary, testis and sacrococcygeal region. 92 tumours were located in a gonad, 78 tumours in extragonadal sites (ratio: 1.2:1). Of the frankly malignant tumours 40 were yolk-sac tumours (YST) and an additional 19 tumours of more than one histological type contained a YST component. Therefore, 67% of the malignant tumours had a YST component. Children with immature teratoma and pure YST showed the lowest median age (5 and 24 months, respectively), while children with germinomas of various locations had the highest median age (153 months). A festoon pattern was the predominant histological feature in all YST and in the YST component of mixed germ cell tumours. Hyaline globules were found in 33/37 YST and in 16/17 YST components. Immunohistochemically, alpha1-fetoprotein (AFP) was demonstrated in 18/22 YST and in 6/7 YST components of mixed germ cell tumours. Hyaline globules were mostly AFP-negative (only 5 cases with AFP-positive globules in addition to many AFP-negative globules). In 3 cases beta-HCG-positive giant cells were seen. In most YST prekeratin intermediate filaments could be demonstrated in the epithelial cells. Follow-up data, available from 51 cases of YST and tumours with YST components showed disease-free survival in 37 cases (72.5 %). 10 patients (19.6 %) died of disease, and 4 patients (7.8%) are living with disease. The comparably high rate of survivors reflects the effectiveness of modern therapy, particularly polychemotherapy, in addition to surgery.
    Type of Medium: Electronic Resource
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  • 5
    Electronic Resource
    Electronic Resource
    Springer
    Virchows Archiv 406 (1985), S. 351-365 
    ISSN: 1432-2307
    Keywords: Neuroepithelioma ; Histology ; Immunohistochemistry ; Neuron-specific enolase ; Ultrastructure
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Seventeen cases of malignant peripheral neuroectodermal tumour (MPNT) were studied by means of light microscopy, immunohistochemistry and electron microscopy. There were nine males and eight females. The mean age of the 17 patients was 10 years with a range of seven months to 20 years. The vast majority of tumours was located in the trunk. Histologically, they closely resembled Ewing's sarcoma, although minor differences were obvious. Special findings included ganglion cells and Flexner rosettes. In 10/11 cases positive staining for neuron-specific enolase (NSE) was obtained. Five of 10 tumours were positive for protein S-100. Three contained vimentin, two neurofilaments and one vimentin, neurofilaments and GFAP. Neurosecretory granules were noted in the three cases studied. Five patients died, three are alive with disease and five patients are alive without evidence of disease. It is concluded that these tumours form a homogeneous group, although the grade of differentiation varies. The prognosis in most cases is poor. Distinction from Ewing's sarcoma is possible by staining for NSE and by electron microscopy.
    Type of Medium: Electronic Resource
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  • 6
    ISSN: 1432-1173
    Keywords: Schlüsselwörter Riesenzellfibroblastom ; Dermatofibrosarcoma protuberans ; Weichteiltumoren ; Kindesalter ; Key words Giant cell fibroblastoma ; Dermatofibrosarcoma protuberans ; Soft tissue tumours ; Childhood
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Summary We report on a 9-year-old girl with a giant cell fibroblastoma of the right anterior chest wall. This rare soft tissue tumour occurs predominantly in the first two decades of life. The typical clinical presentation is a solitary, usually 2–6 cm large, non-tender mass of blue-greyish colour which is mostly located on the back, anterior chest wall, thigh or groin. The histology shows a loose infiltrate of predominantly bland spindle cells in dermis and subcutis. Characteristic elements of the tumour are large angiectoid branching spaces lacking any endo- or epithelium, and relatively small multinucleated cells (floret cells). The recurrence rate is high if the tumour is not excised with adequate safety margins. Metastases are not report. It is of pre-eminent importance to differentiate this rare benign tumor from sarcomas, in order to avoid an inappropriately aggressive therapy.
    Notes: Zusammenfassung Berichtet wird über eine 9jährige Patientin mit einem Riesenzellfibroblastom der Brustwand. Dieser seltene, semimaligne Weichteiltumor des Kindes- und frühen Erwachsenenalters imponiert klinisch als solitärer, langsam wachsender, meist 2–6 cm großer, bläulich-grauer, weicher Tumor. Bevorzugte Lokalisationen sind Rücken, vordere Brustwand, Oberschenkel und Leiste. Die Histologie zeigt einen dermal und subkutan gelegenen, lockeren Verband von z.T. mäßig pleomorphen Spindelzellen in einer myxoiden Matrix. Charakteristisch sind pseudovaskuläre oder sinusoide Hohlräume ohne epi- oder endotheliale Auskleidung und relativ kleine mehrkernige Riesenzellen (floret cells). Die Rezidivrate bei unvollständiger Exzision wird mit 47% angegeben; eine Metastasierung wurde bislang nicht beschrieben. Die histologische Abgrenzung von malignen Weichteiltumoren wie dem myxoiden Liposarkom und dem malignen fibrösen Histiozytom ist im Hinblick auf eine angemessene Therapie von außerordentlicher Bedeutung.
    Type of Medium: Electronic Resource
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  • 7
    ISSN: 1432-1963
    Keywords: Schlüsselwörter Rhadomyosarkom ; Klassifizierung ; Immunhistochemie ; Genetik ; Prognose ; Key words Rhabdomyosarcoma ; Classification ; Immunohistochemistry ; Genetics ; Prognosis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Summary Rhabdomyosarcoma (RMS) is the most important and a very heterogeneous group of malignant soft tissue tumors of childhood and adolescence.The two major subtypes (embryonal and alveolar) share a common myogenic differentiation, but seem to be histogenetically not related. The so-called ’International Classification of Rhabdomyosarcoma’ includes, besides the two major subtypes, the botryoid and leiomyomatous subtypes of embryonal RMS which are associated with a better prognosis and are treated less aggressively according to current protocols. In addition, the solid variant of alveolar RMS is included in the alveolar group of RMS. The identification of the various subtypes is necessary and important because the treatment with the current protocols is also related to histology. Using conventional stains and immunohistochemistry, these subtypes are distinguishable. Genetic analysis can be helpful in the demonstration of t(2;13) or t(1;13) translocations in alveolar RMS. The identification of alveolar RMS with t(1;13) translocation might become important in the future, because this type of translocation seems to be related to a better prognosis as compared to tumors with a t(2;13) translocation.
    Notes: Zusammenfassung Rhabdomyosarkome stellen eine heterogene Gruppe von ganz verschiedenartigen, histogenetisch wohl nicht zusammengehörenden Tumoren dar. Nach der heute verwendeten „Internationalen Klassifikation” der Rhabdomyosarkome werden neben der Unterteilung in embryonalen und alveoläre Rhabdomyossarkome auch Subtypen des embryonalen RMS identifiziert (botryoider und leiomyomatöser Subtyp), die durch eine günstigere Prognose und durch die Notwendigkeit einer weniger aggressive Therapie gekennzeichnet sind. Durch Einsatz von verschiedenen histologischen und immunhistochemischen Färbungen ist die Identifizierung der verschiedenen Typen der RMS heute möglich und auch zwingend notwendig, da die einzelnen Entitäten nach ganz unterschiedlichen Therapieprotokollen behandelt werden. Der Nachweis typischer molekulargenetischer Veränderungen kann in der Unterscheidung insbesondere von embryonalen und alveolären RMS hilfreich sein. In der Regel ist die Abgrenzung zwischen diesen beiden Entitäten auch an konventionell gefärbten Schnittpräparaten möglich. Die Identifizierung von alveolären RMS mit einer t(1;13)-Translokation könnte in Zukunft eine große Bedeutung haben, da diese genetische Veränderung möglicherweise mit einer günstigeren Prognose assoziert sein könnte als die t(2;13)-Translokation.
    Type of Medium: Electronic Resource
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  • 8
    ISSN: 1432-1998
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Congenital hydrocephalus is caused by a broad spectrum of underlying disorders. In the majority of cases it is due to aqueductal stenosis and other distinct congenital anomalies, like Arnold-Chiari malformation. Nevertheless, in the differential diagnosis rare conditions such as cerebral malignancies must also be considered. We present two cases of congenital intracerebral teratoma as a differential diagnosis in congenital obstructive hydrocephalus. A teratoma is suggested when a rapidly growing hydrocephalus with a central calcified and vascularized mass is found sonographically. Regular cerebral structures usually cannot be detected. Early diagnosis in such cases is of clinical importance as the prognosis of congenital intracerebral teratoma is generally very poor.
    Type of Medium: Electronic Resource
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  • 9
    Electronic Resource
    Electronic Resource
    Springer
    Virchows Archiv 436 (2000), S. 473-480 
    ISSN: 1432-2307
    Keywords: Key words Solid ; pseudopapillary tumor ; Immunoprofile ; Pancreatic origin ; Genital ridge/ovarian origin
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract  Solid–pseudopapillary tumor of the pancreas (SPT) has distinctive morphologic and biologic features but an unclear origin. It is classified among the pancreatic epithelial tumors, though many are reported to be negative for cytokeratin. Also unclear are its neuroendocrine differentiation, its capability to express alpha-1-antitrypsin (AAT) and, in view of the tumor’s striking prevalence in women, its relationship with the female genital tract. To clarify these issues, the immunoprofiles of 59 SPTs were defined by applying a battery of antibodies against cytokeratin, vimentin, neuron-specific enolase (NSE), synaptophysin, chromogranin A, tyrosine hydroxylase (TH), AAT, LeuM1, Ki-M1P, smooth-muscle actin, CD34, alpha-inhibin, calretinin, placental alkaline phosphatase (PLAP), and progesterone and estrogen receptors. The most consistent markers with the strongest immunoreactivity were vimentin, AAT, NSE, and the progesterone receptor, which were each found in more than 90% of the tumors. Using immunocytochemical methods involving antigen retrieval, cytokeratin was demonstrated in almost 70% of the cases. Synaptophysin was found in 22% of the tumors, while chromogranin was absent and tyrosine hydroxylase was only present in a few tumors. None of the other markers tested were expressed by SPTs. This staining pattern fails to reveal a clear phenotypic relationship with any of the defined cell lineages of the pancreas. In view of the striking female preponderance of SPTs and the known close approximation of the genital ridges to the pancreatic anlage during embryogenesis, it is, however, hypothesized that SPTs might derive from genital ridge/ovarian anlage-related cells, which were attached to the pancreatic tissue during early embryogenesis.
    Type of Medium: Electronic Resource
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  • 10
    ISSN: 1432-2307
    Keywords: Keywords Small round cell tumors ; Ewing’s sarcoma ; Translocation ; Immunohistochemistry ; Differential diagnosis ; RT-PCR
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract  It is now widely accepted that the EWS/FLI-1 fusion transcript is associated with tumors of the Ewing family. To test whether it is possible to detect the fusion transcript by means of combining polymerase chain reaction (PCR) methodology and immunohistochemistry, we investigated tumors of the Ewing family using in situ reverse transcriptase (RT)-PCR. We were able to demonstrate the t(11;22) fusion transcript in five of six cases of Ewing’s sarcoma and four of four peripheral primitive neuroectodermal tumors. These results were confirmed using fluorescence in situ hybridization in seven tumor samples. In situ RT-PCR-labeled fusion transcripts were found in virtually all tumor cells within a given sample, indicating that each cell possessed the t(11;22) transcript. We conclude from these results that in situ RT-PCR can be used for the rapid detection of EWS/FLI-1 fusion transcripts in biopsy material. The findings also suggest that all cells of the tumors of the Ewing family carry the EWS/FLI-1 fusion transcript.
    Type of Medium: Electronic Resource
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