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儿童第四脑室星形细胞瘤:21例临床病理特征

IV ventricle astrocytomas in childhood: clinicopathological features in 21 cases.

作者信息

Tomita T, Chou P, Reyes-Mugica M

机构信息

Division of Pediatric Neurosurgery, Children's Memorial Hospital, Chicago, IL 60614, USA.

出版信息

Childs Nerv Syst. 1998 Oct;14(10):537-46. doi: 10.1007/s003810050269.

Abstract

The authors conducted a study of 21 children with benign astrocytomas in the IV ventricle treated with radical tumor resection from 1982 through 1991. The purposes of this study were to identify the tumor origin and neural involvement, and to determine the natural history following surgical resection. Pathological studies showed that 18 were pilocytic astrocytomas (pure pilocytic in 12, mixed in 6), 2 fibrillary, and 1 gemistocytic. In the IV ventricle, 12 patients had a transependymal involvement of the floor (brain stem), 6 had an involvement of the wall (cerebellar peduncle), and 3 had involvement of both floor and wall. A gross total resection was performed in 9 patients, and the remaining 12 patients underwent a subtotal resection. All patients were followed without radiation therapy (RT) or chemotherapy. During a follow-up period of 6.5-15 years, all patients were alive. Eight patients suffered recurrence between five months and 66 months after diagnosis. Of these, five received RT for recurrence and had a complete response in all cases. The remaining 13 patients showed no evidence of disease and one had a stable residual tumor. The recurrence-free 5-year and 10-year survival rates were 62.5% and 57% respectively. Patients without brainstem involvement, with total resection, or with pure pilocytic astrocytoma had a better outcome than those with brain stem involvement, with subtotal resection, or with nonpilocytic or mixed histology. In summary, a great majority of benign IV ventricle astrocytomas involve the floor of the IV ventricle. It is often difficult to determine the origin of these tumors in most cases. Benign IV ventricle astrocytomas may not recur even after incomplete resection, and close observation without RT is recommended, although RT appears to be effective for these tumors when they recur.

摘要

作者对1982年至1991年间接受根治性肿瘤切除术治疗的21例第四脑室良性星形细胞瘤患儿进行了一项研究。本研究的目的是确定肿瘤起源和神经受累情况,并确定手术切除后的自然病程。病理研究显示,18例为毛细胞型星形细胞瘤(12例为纯毛细胞型,6例为混合型),2例为纤维型,1例为肥胖细胞型。在第四脑室,12例患者的第四脑室底部(脑干)有经室管膜受累,6例患者的侧壁(小脑脚)受累,3例患者的底部和侧壁均受累。9例患者进行了全切除,其余12例患者进行了次全切除。所有患者均未接受放疗或化疗。在6.5至15年的随访期内,所有患者均存活。8例患者在诊断后5个月至66个月之间复发。其中,5例复发患者接受了放疗,所有病例均完全缓解。其余13例患者无疾病证据,1例有稳定的残留肿瘤。无复发生存5年和10年的生存率分别为62.5%和57%。无脑干受累、全切除或纯毛细胞型星形细胞瘤的患者比有脑干受累、次全切除或非毛细胞型或混合型组织学的患者预后更好。总之,绝大多数第四脑室良性星形细胞瘤累及第四脑室底部。在大多数情况下,这些肿瘤的起源往往难以确定。第四脑室良性星形细胞瘤即使在不完全切除后也可能不复发,建议密切观察而不进行放疗,尽管放疗在这些肿瘤复发时似乎有效。

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