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颅骨骨肉瘤:一种罕见肿瘤的长期预后

Osteogenic sarcoma of the skull: long-term outcome of a rare tumor.

作者信息

Tyndall Ronette Goodluck, Popescu Oana-Eugenia, Derek Prevost, Steinbok Paul

机构信息

Division of Pediatric Neurosurgery, Department of Surgery, University of British Columbia & British Columbia Children's Hospital, 4480 Oak St., Room K3-154, Vancouver, BC, Canada.

Department of Pathology and Laboratory Medicine, Children's & Women's Hospital, Vancouver, BC, Canada.

出版信息

Childs Nerv Syst. 2018 Nov;34(11):2149-2153. doi: 10.1007/s00381-018-3937-0. Epub 2018 Aug 17.

DOI:10.1007/s00381-018-3937-0
PMID:30120533
Abstract

BACKGROUND

Osteogenic sarcoma of the skull is uncommon and long-term outcome is not well defined. We review the literature and present a pediatric case of calvarial osteogenic sarcoma with good long-term oncological and cosmetic outcome and excellent quality of life. This case presented major surgical challenges, which are detailed.

CASE DESCRIPTION

A 6-year-old boy presented with a painless 5 cm × 5 cm lump over the vertex region. He was neurologically normal. Imaging showed an extensive bony lesion with intradural extension. After incisional biopsy showed probable low grade osteosarcoma, a complete en bloc resection with margins was attempted via a concentric craniotomy around the lesion after embolization to reduce blood loss. Invasion of the brain by the tumor precluded the complete en bloc resection, but gross total resection was achieved. The final pathology was consistent with a low-grade osteosarcoma and adjuvant chemotherapy was provided. Follow-up for 8 years has shown no recurrence with good cosmetic and functional outcome.

摘要

背景

颅骨骨肉瘤并不常见,其长期预后尚不明确。我们回顾了相关文献,并呈现了一例儿童颅骨骨肉瘤病例,该病例具有良好的长期肿瘤学和美容效果以及出色的生活质量。此病例带来了重大的手术挑战,将详细阐述。

病例描述

一名6岁男孩头顶区域出现一个无痛性5厘米×5厘米的肿块。他神经系统正常。影像学检查显示有广泛的骨病变并向硬膜内扩展。切开活检显示可能为低级别骨肉瘤后,在栓塞以减少失血后,尝试通过围绕病变进行同心开颅术进行完整的整块切除并确保切缘阴性。肿瘤侵犯大脑使得无法进行完整的整块切除,但实现了大体全切。最终病理结果与低级别骨肉瘤一致,并给予了辅助化疗。8年的随访显示无复发,美容和功能效果良好。

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本文引用的文献

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Primary osteosarcoma of the cranial vault.颅骨原发性骨肉瘤。
Radiol Bras. 2017 Jul-Aug;50(4):263-265. doi: 10.1590/0100-3984.1914-2014.
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Pediatric head and neck bone sarcomas: An analysis of 204 cases.小儿头颈部骨肉瘤:204例分析
Int J Pediatr Otorhinolaryngol. 2017 Sep;100:71-76. doi: 10.1016/j.ijporl.2017.06.003. Epub 2017 Jun 15.
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Osteosarcoma of the skull base: An analysis of 19 cases and literature review.颅底骨肉瘤:19例病例分析及文献综述
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Osteosarcoma of the cranial vault and skull base in pediatric patients.小儿患者颅顶和颅底骨肉瘤
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Primary osteosarcoma of clivus: a short report.斜坡原发性骨肉瘤:简短报告
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Risk factors for metastatic disease at presentation with osteosarcoma: an analysis of the SEER database.骨肉瘤初诊时发生转移的风险因素:SEER 数据库分析。
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Childhood osteosarcoma of greater wing of sphenoid: case report and review of literature.蝶骨大翼儿童骨肉瘤:病例报告及文献复习
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