Clinicopathological study of pediatric gliomas with extensive myxoid change

从群马大学医院诊断的脑肿瘤档案中选取10例有广泛粘液样改变的脑胶质瘤患者和送作者会诊的病例进行检查。9例患者年龄在1~16岁之间，增加了24岁患者。除1例小脑肿瘤外，其余肿瘤均发生于幕上区。3例有急性淋巴细胞性白血病(ALL)病史，肿瘤组织呈微囊型、血管中心型、稀疏弥漫型伴广泛粘液样改变。肿瘤细胞有向血管周围聚集的趋势。根据肿瘤的分化程度和增殖能力分为低度恶性组和高度恶性组。7例高级别肿瘤中有5例含有小原始细胞成分，其中1例仅由突触素阳性的小原始细胞组成，具有较高的增殖能力。在组织学上与Pri…一致更具侵袭性的神经外胚层肿瘤(PNET)。除原始细胞外，2例由分化的星形胶质细胞组成，无间变，另2例为弥漫性增生性胶质瘤成分，表现为间变。2例无原始成分的肿瘤与高级别星形细胞瘤相似，但肿瘤细胞几乎不表达胶质纤维酸性蛋白(GFAP)，而表达Vimentin。其他3例为低级别胶质瘤，包括毛粘液样星形细胞瘤、GFAP阴性的星形细胞瘤和少突胶质瘤样瘤，多数为高级别。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种。小原始细胞多表达Orig2或5-100蛋白。这一发现与传统的PNET不同。APNET样瘤不表达EGFR和p53。3例ALL经化疗和/或放疗缓解后发生粘液性胶质瘤。粘液样胶质瘤有可能发生为继发性肿瘤或放射诱导肿瘤。较少

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