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一名16岁儿童患有具有肿瘤侵袭性细胞遗传学特征的鼻筛窦脑膜瘤。

Nasoethmoidal meningioma with cytogenetic features of tumor aggressiveness in a 16-year-old child.

作者信息

Ketter Ralf, Henn Wolfram, Feiden Wolfgang, Prowald Alexandra, Sittel Christian, Steudel Wolf-Ingo, Strowitzki Martin

机构信息

Department of Neurosurgery, Saarland University, Homburg/Saar, Germany.

出版信息

Pediatr Neurosurg. 2003 Oct;39(4):190-4. doi: 10.1159/000072470.

Abstract

The case of a 16-year-old girl is presented who was admitted to our hospital because of severe frontal headache. Magnetic resonance scans revealed a nasoethmoidal tumor on the right side, with erosion of the skull base and invasion of the right orbit. A needle biopsy specimen revealed the histological diagnosis of a fibroblastic meningioma (WHO grade II), with histochemically determined partial activity loss of alkaline phosphatase. The tumor was completely resected by a combined intracranial-transbasal and transnasal approach. In this case, we found a meningioma with deletion of the short arm of chromosome 1 through a translocation between chromosomes 1 and 11 [t(1;11)] as well as additional chromosomal aberrations, including partial or complete monosomy of chromosomes 2, 6, 7, 11, 13 and 22, a cytogenetic pattern known to be associated with elevated tumor aggressiveness in meningiomas of adult patients. Accordingly, this juvenile meningioma may biologically correspond to a WHO grade II adult-type meningioma with an increased risk of recurrence. Therefore, we incorporated this patient into an intensified schedule of postoperative care.

摘要

本文报告了一名16岁女孩的病例,她因严重的前额头痛入院。磁共振扫描显示右侧鼻筛窦肿瘤,伴有颅底骨质侵蚀和右侧眼眶侵犯。针吸活检标本的组织学诊断为纤维母细胞型脑膜瘤(WHO二级),组织化学检测显示碱性磷酸酶部分活性丧失。通过颅内-经基底和经鼻联合入路将肿瘤完全切除。在该病例中,我们通过1号和11号染色体之间的易位[t(1;11)]发现了1号染色体短臂缺失的脑膜瘤,以及其他染色体异常,包括2、6、7、11、13和22号染色体部分或完全单体性,这种细胞遗传学模式已知与成年患者脑膜瘤的肿瘤侵袭性增加有关。因此,这种青少年脑膜瘤在生物学上可能相当于具有较高复发风险的WHO二级成人型脑膜瘤。因此,我们将该患者纳入强化的术后护理计划。

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