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原始神经外胚层肿瘤,包括髓母细胞瘤:胶质细胞分化通过胶质纤维酸性蛋白(GFAP)免疫反应性来显示,仅限于纯促结缔组织增生性髓母细胞瘤(“小脑蛛网膜肉瘤”)。

Primitive neuroectodermal tumors including the medulloblastoma: glial differentiation signaled by immunoreactivity for GFAP is restricted to the pure desmoplastic medulloblastoma ("arachnoidal sarcoma of the cerebellum").

作者信息

Herpers M J, Budka H

出版信息

Clin Neuropathol. 1985 Jan-Feb;4(1):12-8.

PMID:3978900
Abstract

Immunoreactivity of tumor cells for glial fibrillary acidic protein (GFAP) is usually regarded as sign of astrocytic histogenesis and/or differentiation. The present study aimed at a systematic evaluation of the significance of GFAP-containing cells in primitive neuroectodermal tumors (PNETs) with special reference to the controversial entity of desmoplastic medulloblastoma (so-called "circumscribed arachnoidal sarcoma of the cerebellum"). Fifty-three PNETs, including 17 pure desmoplastic medulloblastomas were investigated, using GFAP antisera and the peroxidase-antiperoxidase (PAP) technique. Seventy percent of the pure desmoplastic medulloblastomas showed GFAP immunoreactive cells, in 47% indistinguishable from adjacent nonreacting tumor cells. Most immunoreacting cells were found in the reticulin free islands, showing in 6 cases a gradual transition of immunoreacting cells from tumor cells to larger cells shaped like astrocytes. The classical medulloblastomas showed only larger immunoreacting cells which were interpreted as reactive astrocytes. Therefore, the so-called circumscribed arachnoidal cerebellar sarcoma or pure desmoplastic medulloblastoma merits a separate place in the group of PNETs as a tumor with frequent signs of astroglial differentiation; this interpretation appears to be clinically correlated by a difference in age incidence and prognosis of that special tumor-type in comparison with classical medulloblastoma.

摘要

肿瘤细胞对胶质纤维酸性蛋白(GFAP)的免疫反应性通常被视为星形细胞组织发生和/或分化的标志。本研究旨在系统评估含GFAP细胞在原始神经外胚层肿瘤(PNETs)中的意义,特别参考促结缔组织增生性髓母细胞瘤(所谓的“小脑局限性蛛网膜肉瘤”)这一有争议的实体。使用GFAP抗血清和过氧化物酶-抗过氧化物酶(PAP)技术,对53例PNETs进行了研究,其中包括17例单纯促结缔组织增生性髓母细胞瘤。70%的单纯促结缔组织增生性髓母细胞瘤显示有GFAP免疫反应性细胞,其中47%与相邻的无反应性肿瘤细胞难以区分。大多数免疫反应性细胞见于无网状纤维的岛区,6例显示免疫反应性细胞从肿瘤细胞逐渐过渡为星形样大细胞。经典型髓母细胞瘤仅显示较大的免疫反应性细胞,被解释为反应性星形细胞。因此,所谓的小脑局限性蛛网膜肉瘤或单纯促结缔组织增生性髓母细胞瘤作为一种常有星形胶质细胞分化迹象的肿瘤,在PNETs组中值得单独分类;与经典型髓母细胞瘤相比,该特殊肿瘤类型在年龄发病率和预后方面的差异似乎与这一解释存在临床相关性。

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