Clinicopathological study of pediatric gliomas with extensive myxoid change

从群马大学附属医院诊断的脑肿瘤病例和笔者会诊的病例中选择10例具有广泛粘液样改变的胶质瘤进行检查。9例病例的患者年龄范围为1 - 16岁，增加了24岁患者。除小脑肿瘤外，所有肿瘤均发生在幕上区域。3例既往有急性淋巴细胞白血病（ALL）病史，肿瘤组织呈微囊样改变，血管中心型，弥漫型，广泛粘液样改变。肿瘤细胞有向血管周围聚集的趋势。根据间变性和增殖能力将肿瘤分为低级别组和高级别组。7例高级别肿瘤中5例含有小原始细胞成分，其中1例仅由小原始细胞组成，呈突触素阳性，并表现出高增殖能力。组织学上与原发性肝癌一致 ...更多信息 缓解性神经外胚层肿瘤（PNET）。除原始细胞外，2例由分化的星形胶质细胞组成，无间变性，另2例有弥漫性增生的胶质瘤成分，显示间变性。2例无原始成分，与高级别星形细胞瘤相似，但肿瘤细胞几乎不表达胶质细胞酸性蛋白（GFAP），而表达波形蛋白。低级别胶质瘤3例，包括毛黏液样星形细胞瘤、GFAP阴性星形细胞瘤和少突胶质细胞瘤样瘤，黏液样胶质瘤多为高级别。此外，它们通常含有小的原始细胞成分，其中一些被认为是胶质母细胞瘤的变体。小的原始细胞经常表达Olig 2或5-100蛋白。这一发现与传统的PNET不同。APNET样肿瘤不表达EGFR和p53。3例ALL经化疗和/或放疗缓解后发展为粘液样胶质瘤。粘液样胶质瘤可能是继发性肿瘤或放射性肿瘤。少

Ten cases with gliomas with extensive myxoid change were selected from files of brain tumors diagnosed at Gunma University Hospital and cases sent to the author for consultation, and were examined. Patient age of nine cases ranged between 1 and 16 years and 24-old year patient was added. All tumors except a cerebellar tumor arose in supratentorial region. Three cases had developed acute lymphoblastic leukemia(ALL) in their past history.Tumor tissues showed microcystic change, angiocentric pattern, and sparsely diffuse pattern with extensive myxoid change. Tumor cells appeared to have a tendency of aggregating to perivascular space. The tumors were divided into low-grade and high-grade groups from the aspects of anaplasia and proliferation ability. Five of seven high-grade tumors included small primitive cell component One of them consisted of only small primitive cells that were positive for synaptophysin, and showed high proliferation ability. It was histologically consistent with pri … More mitive neuroectodermal tumor (PNET). In addition to primitive cells, two cases were composed of differentiated astrocytes without anaplasia, and other two cases had diffusely proliferating glioma component showing anaplasia. Two cases without primitive component were similar to high grade astrocytoma, but the tumor cells hardly expressed glial fibrillary acidic protein (GFAP), but did vimentin. Other three cases were classified as low-grade glioma, including pilomyxoid astrocytoma, astrocytic tumor negative for GFAP, and oligodendroglioma-like tumor.Most myxoid gliomas were high-grade. In addition, they often harbored small primitive cell component Some of them were considered to be a variant of glioblastoma. Small primitive cells frequently expressed Olig2 or 5-100 protein. The finding was different from that of conventional PNET. APNET-like tumor did not overexpress EGFR or p53.Three cases developed myxoid glioma after remission of ALL by chemotherapy and/or radiotherapy. It is possible that myxoid gliomas may occur as secondary tumor or radiation-induced tumor. Less