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[胶质髓母细胞瘤。对肌源性脑肿瘤形态学和生物学行为的贡献(作者译)]

[Gliomedullomyoblastoma. A contribution to the morphology and biological behavior of myogenic brain tumors (author's transl)].

作者信息

Warzok R, Jänisch W, Schreiber D

出版信息

Zentralbl Allg Pathol. 1982;126(1-2):5-15.

PMID:6283766
Abstract

On the basis of an own observation and 38 cases found in the literature problems of histogenesis, classification and biological behavior of myogenic brain tumors and mixed neoplasms with myogenic elements are discussed. In a 2-year-old boy, 2 months after first clinical symptoms, a tumor of the walls of the 4th ventricle had been removed. The growth showed a large variety of histological structures. Irregularly arranged bundles of long muscle fibers with centrally located nuclei and cross striation were the most striking feature. Large cells with prominent nucleoli resembling ganglion cells were identified electronmicroscopically as rhabdomyblasts. Other areas were composed of astrocytic elements or of honeycomb-like structures usually observed in oligodendrogliomas. In serial sections small cells with dark nuclei typical for medulloblastomas were found in small areas. The body died 15 months after the onset of symptoms. The autopsy revealed a relapse in midline structures of the cerebellum. The histological picture differed substantially from that of the primary neoplasm. It was composed only of small cells, showed a large number of collagenous fibers and corresponded to a demoblastic medulloblastoma. The histological variety is regarded as an expression of the pluripotency of neoplastic elements, which are evidently capable to form neuroectodermal as well as mesenchymal structures. It is emphasized that an overgrowth more anaplastic cells led to the described morphological changes. Gliomedullomyoblastoma is regarded as a special variant of medulloblastoma with an unfavourable prognosis.

摘要

基于自身观察以及文献中报道的38个病例,对肌源性脑肿瘤以及含有肌源性成分的混合性肿瘤的组织发生、分类及生物学行为问题进行了讨论。一名2岁男孩,出现首次临床症状2个月后,切除了第四脑室壁的一个肿瘤。该肿瘤呈现出多种组织学结构。最显著的特征是有排列不规则的长肌纤维束,细胞核位于中央,有横纹。电镜下鉴定出具有明显核仁、类似神经节细胞的大细胞为横纹肌母细胞。其他区域由星形细胞成分或通常在少突胶质细胞瘤中观察到的蜂窝状结构组成。在连续切片中,在小区域发现了具有成神经管细胞瘤典型的深染细胞核的小细胞。患者在症状出现15个月后死亡。尸检显示小脑中线结构复发。组织学表现与原发肿瘤有很大不同。它仅由小细胞组成,有大量胶原纤维,符合促结缔组织增生性成神经管细胞瘤。组织学多样性被认为是肿瘤细胞多能性的一种表现,这些肿瘤细胞显然能够形成神经外胚层和间充质结构。强调更多间变细胞的过度生长导致了上述形态学变化。胶质成神经管细胞瘤被视为成神经管细胞瘤的一种特殊变体,预后不良。

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