小儿原发性先天性颅内脂肪瘤伴颅外延伸1例报告及文献复习。

Surgical neurology international Pub Date : 2025-03-07 eCollection Date: 2025-01-01 DOI:10.25259/SNI_965_2024
Vikas Chandra Jha, Rahul Jain, Achintya Ajaya, Sangam Jha, Gaurav Verma, Nikhil Dhage, Anant Parashar, Vivek Sharan Sinha, Nitish Kumar
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引用次数: 0

摘要

背景:先天性颅内脂肪瘤是罕见的,仅占脑肿瘤的0.1-0.5%,通常无症状。颅外病变极为罕见,通常见于生长缓慢的成人病例。本报告提出了一个独特的情况下,迅速增长的先天性颅内脂肪瘤与颅外延伸的儿童患者。病例描述:一名3岁女孩,自出生以来,前额肿块逐渐增大,被发现有一个大的半球间脂肪瘤(12 × 10 × 9 cm),延伸到左侧脑室,并通过额骨缺损与颅外肿块(17 × 10 × 10 cm)相连。其他发现包括胼胝体发育不全和从头皮病变进入上矢状窦的静脉引流。显微外科手术包括颅外脂肪瘤的切除和颅内部分的次全切除,以保留关键的神经血管结构。结论:这个病例的快速脂肪瘤生长在儿科患者是不典型的,不同于缓慢生长,无症状的表现在成人。高倍显微手术和多普勒引导有助于安全切除,预后良好。该病例强调需要进一步研究具有颅外成分的儿童颅内脂肪瘤的发育因素和快速生长机制,可能代表一种独特的临床实体。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Primary congenital intracranial lipoma with extracranial extension in a pediatric patient: A case report and literature review.

Background: Congenital intracranial lipomas are rare, representing only 0.1-0.5% of brain tumors, and are typically asymptomatic. An extracranial component is exceedingly rare, usually reported in slow-growing adult cases. This report presents a unique case of a rapidly growing congenital intracranial lipoma with extracranial extension in a pediatric patient.

Case description: A 3-year-old girl with a progressively enlarging forehead mass, present since birth, was found to have a large interhemispheric lipoma (12 × 10 × 9 cm) extending into the left lateral ventricle and connected to an extracranial mass (17 × 10 × 10 cm) through a frontal bone defect. Additional findings included corpus callosal agenesis and venous drainage from the scalp lesion into the superior sagittal sinus. The microsurgical intervention involved the excision of the extracranial lipoma and subtotal resection of the intracranial component, preserving critical neurovascular structures.

Conclusion: This case of rapid lipoma growth in a pediatric patient is atypical, differing from slow-growing, asymptomatic presentations in adults. High-magnification microsurgery and Doppler guidance facilitated safe resection with a positive outcome. This case highlights the need for further research into developmental factors and rapid growth mechanisms in pediatric intracranial lipomas with extracranial components, potentially representing a distinct clinical entity.

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