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  • Glial fibrillary acidic protein  (1)
  • Key words Von Hippel-Lindau disease (vHL)  (1)
  • Low grade gliomas  (1)
  • 1
    ISSN: 1437-2320
    Keywords: Key words Von Hippel-Lindau disease (vHL) ; Multiple spinal hemangioblastomas ; vHL protein ; Immunohistochemistry
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We report on a 57-year-old male presenting with radicular pain in the nerve roots of L5 and S1 on the right side and dysuria. Magnetic resonance imaging (MRI) of the lumbar spine showed multiple (up to 20) small, intradural enhancing nodules attached to the cauda equina down to the sacrum, the largest 1 cm in diameter at the level Th12/ L1 compressing the conus. Additionally, small nodules in the cervico-thoracal region adjacent to the cord, but no cerebellar or cerebral abnormalities, were detected in a consecutive MRI of the remaining neuroaxis. The histology of a resected lesion at Th12/L1 revealed hemangioblastoma of the reticular type. Together with a history of left eye enucleation performed 17 years ago for angiomatosis of the retina and the immunohistochemical detection of von Hippel-Lindau (vHL) protein within the removed spinal hemangioblastoma, a diagnosis of vHL disease was established. Family history and screening for visceral manifestations of vHL disease were negative. In contrast to cerebellar or solitary spinal hemangioblastomas, multiple spinal hemangioblastomas without cerebellar involvement in vHL represent unusual manifestations. Unlike the case for solitary lesions in non-syndromic patients, a surgical cure does not seem feasible in this case. The role of treatment modalities is discussed.
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1432-0533
    Keywords: Brain tumour ; Suprasellar tumour ; Meningioma ; Glial fibrillary acidic protein ; Neuropathology
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract A 24-year-old female presented with a 3-year history of a suprasellar and intraventricular solid midline process measuring about 3×4 cm. At surgery, this tumour was sharply delineated and of stone-like firmness and was removed completely. Histology suggested meningioma, featuring nests and cords of epithelium-like cells with prominent cytoplasm amidst abundant fibrous stroma with prominent lymphoplasmocellular infiltration. Immunocytochemically, the tumour cells expressed vimentin, S-100 protein, epithelial membrane antigen, cytokeratins, and most surprisingly, glial fibrillary acidic protein (GFAP). Ultrastructural investigation revealed abundant intermediate filaments and occasionally dense secretory granules in tumour cells with short, finger-like cytoplasmic processes joined by very rare small, but well-developed desmosomes. This tumour most likely represents a peculiar variant of meningioma with prominent production of GFAP, as previously described [Budka H (1986) Acta Neuropathol (Berl) 72: 43–54].
    Type of Medium: Electronic Resource
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  • 3
    ISSN: 0942-0940
    Keywords: Low grade gliomas ; Xenon CT ; stereotactic biopsy ; histological grading
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Xenon-enhanced computerized tomography (XeCT) was performed on 14 consecutive adult patients presenting with seizures showing supratentorial non-enhancing radiologically uniform appearing low grade gliomas on CT/MR images. Pre-operative XeCT patterns were compared with postoperative histological diagnosis, grading and Ki67 proliferation indices (PI). After gross-total, subtotal resection or biopsy, 11 astrocytomas, 2 oligodendrogliomas and 1 oligo-astrocytoma were diagnosed and graded: Grade I: 2 patients (Ki67-PI=0.5–0.8), Grade I–II: 4 patients (Ki67-PI=0.3–1.5), Grade II: 3 patients (Ki67-PI=0.5–3.5), Grade II–III: 4 patients (Ki67-PI=3.8–6.8) and Grade III: 1 patient (Ki67-PI=5.2), (Kernohan Classification). Xenon CT studies revealed different flow patterns, correlating with the postoperative histological diagnosis, grading and proliferation indices: A tumour group with well defined, delayed, only minimally enhancing tumour area (5 patients, Grade I, I–II or II), a second group with less well defined low-flow-area borders and inhomogenous, strong enhancement within the tumour (4 patients, Grade II–III, III) and a third group with fast enhancing tumours was identified. The third pattern was exclusively shown in the 2 oligodendrogliomas (Grade I and II–III) and 1 oligo-astrocytoma (Grade II). The preliminary report identifies the Xenon enhanced CT as a beneficial pre-operative investigation for patients with radiologically uniform appearing suspected adult supratentorial low-grade gliomas, which may give information about the presence of anaplastic foci or oligodendroglial components.
    Type of Medium: Electronic Resource
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