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  • 1
    ISSN: 1432-0533
    Keywords: Neurofibromatosis 2 ; Bilateral acoustic neurofibromatosis ; Ghal hamartomas ; Immunohistochemistry ; S-100 protein
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Bilateral acoustic neurofibromatosis (neurofibromatosis 2, NF2) accounts for less than 10% of all cases of neurofibromatosis and manifests itself with bilateral acoustic schwannomas, multiple schwannomas of spinal nerve roots, meningiomas, glial tumors and hamartomatous CNS lesions. We have observed dysplastic foci of immature neuroectodermal cells in the cerebral cortex and basal ganglia of six patients afflicted with neurofibromatosis 2, ranging from occasional clusters of immature, dysplastic cells to numerous, confluent lesions. These cells, although often polymorphic and multinuclear did not show mitotic acitivity or a tendency for neoplastic transformation. To determine the histogenesis of these foci, extensive immunocytochemical reactions were carried out with antibodies to a variety of glial, neuronal and nonneural cell lineages. With the exception of S-100 protein, no immunoreactivity was detectable. S-100 was consistently expressed in these foci, irrespective of their size, location, and degree of polymorphism. On the basis of cytological appearance, distribution and immunoreactivity we tentatively designate these foci as glial micro-hamartomas. Although we did not systematically analyze the CNS of patients with von Recklinghausen neurofibromatosis (neurofibromatosis 1, NF1), the present study strongly suggests that these micro-hamartomas constitute a morphological hallmark of bilateral acoustic neurofibromatosis (NF2).
    Type of Medium: Electronic Resource
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  • 2
    Electronic Resource
    Electronic Resource
    Springer
    Der Radiologe 38 (1998), S. 888-897 
    ISSN: 1432-2102
    Keywords: Schlüsselwörter Alkalische Phosphatase (ALPL) ; Chromosom 1p ; Gliom- Grading ; Ki-67 ; Meningeom ; Key words Alkaline phosphatase (ALPL) ; Chromosome 1p ; Glioma ; Grading ; Ki-67 ; Meningioma
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Summary Gliomas: As we demonstrated for supratentorial, diffuse gliomas in adults, a stratification into just two grades of malignancy, ’low’ and ’high grade’, proved reliable and prognostically relevant. The discriminating histomorphological criterion for high-grade astrocytoma (WHO glioblastoma) as well as anaplastic oligodendroglioma and anaplastic oligoastrocytoma is endothelial hyperplasia/proliferation, which is usually associated with uptake of contrast medium in computed tomography and magnetic resonance imaging. As neoangiogenesis indicates glioma progression, it is worthwhile considering these radiographic features to judge the representativeness of the tumor samples critically. Meningiomas: The revised edition of the WHO classification of brain tumors now includes the ’atypical’ meningioma (WHO ’grade’ II): Based on both its histomorphological features and prognosis, it should be placed between the common type and anaplastic meningioma. Nuclear area related Ki-67 proliferation indices, as determined by morphometry, were the prerequisite for outlining its histomorphological spectrum better. Cytogenetically, the most consistent progression-associated feature was loss of the distal part of the short arm of one chromosome 1 (1p-). Thus, a screening method using the tissue non-specific form of alkaline phosphatase (ALPL) as the respective marker enzyme was established. Diagnosing a meningioma of the intermediate type implies careful clinical and radiological patient follow-ups to detect tumor recurrences early.
    Notes: Zusammenfassung Gliome: Wir konnten zeigen, daß für übliche supratentorielle Gliome im Erwachsenenalter ein 2stufiger Grading-Ansatz mit alleiniger Unterscheidung eines niedrigen und hohen Malignitätsgrads zu reproduzierbaren und prognostisch relevanten Informationen führt. Das entscheidende diagnostische Kriterium sowohl für High-grade-Astrozytome (WHO Glioblastome) als auch für anaplastische Oligodendrogliome und Oligoastroazytome sind Endothelhyperplasien bzw. -proliferate als Zeichen einer in Gang gekommenen Neoangiogenese. Diese ist in der Regel mit einer Kontrastmittelaufnahme in den bildgebenden Verfahren korreliert. Daher hat es sich sehr bewährt, wenn zur histopathologischen Untersuchung eines Gliomresektats bzw. -biopsats die neuroradiologischen Bilder (CCT, KST nativ und nach Kontrastmittelgabe) vorliegen oder zumindest das neuroradiologische Untersuchungsergebnis bekannt ist. Meningeome: Jene rezidivgefährdeten Meningeome, die sowohl feingeweblich als auch prognostisch eine Zwischenstellung zwischen üblichen und anaplastischen Meningeomen einnehmen, sind als sog. atypische Meningeome (WHO-Grad II) in die revidierte Fassung der WHO Klassifikation aufgenommen worden. Mit morphometrisch ermittelten kernflächenbezogenen Ki-67 Indizes konnte dieser intermediäre Typ näher eingegrenzt und histologisch besser definiert werden. Da rezidivgefährdete Meningeome als häufigste progressionsassoziierte Veränderung einen distalen Stückverlust am kurzen Arm eines Chromosoms 1 (1p-) zeigen, ließ sich eine Screening-Methode mit der gewebeunspezifischen Form der Alkalischen Phosphatase als Markerenzym etablieren. Die Diagnose eines Meningeoms vom intermediären Typ bedeutet, daß engmaschigere postoperative Verlaufskontrollen zur frühzeitigen Erfassung eines „Rezidivs” erfolgen sollten.
    Type of Medium: Electronic Resource
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