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儿童脊髓室管膜瘤的评估:一项25年的回顾性观察研究。

Evaluation of pediatric spinal ependymomas: A 25-year retrospective observational study.

作者信息

Narin Firat, Bahadir Sinan, Hanalioğlu Şahin, Karakaya Dicle, Yahya Diaa, Özer Hidir, Söylemezoğlu Figen, Bilginer Burçak

机构信息

Neurosurgery Clinic, Mersin City Education and Research Hospital, Mersin, Turkey.

Department of Neurosurgery, Başkent University Faculty of Medicine, Ankara, Turkey.

出版信息

Medicine (Baltimore). 2024 Dec 20;103(51):e40986. doi: 10.1097/MD.0000000000040986.

Abstract

This study aims to evaluate the clinical and radiological features, histopathological characteristics, treatment modalities, and their effectiveness, as well as long-term follow-up results of pediatric spinal ependymomas treated at a single institution. In this retrospective study, medical records of 14 pediatric patients (3 females and 11 males) who were surgically treated for spinal ependymoma in our institution between 1995 and 2020 were reviewed. Data regarding age, gender, presenting symptoms and signs, radiological findings, postoperative status, extent of resection, histopathological grading, recurrence, tumor growth, seeding, and adjuvant treatment were collected and analyzed. Six patients had myxopapillary ependymoma, 7 patients had grade II ependymoma, and 1 had grade III ependymoma. Median age at diagnosis was 10.5 (range, 1-15) years. The most common presenting symptoms and signs were axial and radicular pain, paresis, and first motor neuron involvement. In 10 patients, gross total tumor resection was achieved. Five patients underwent additional surgeries for disease progression (recurrence or residual tumor growth). Most of the patients who had no disease progression were treated with gross total resection. Two patients experienced seeding. One patient who had a grade III tumor and did not achieve gross total resection died during follow-up. Pediatric spinal ependymomas are uncommon tumors with relatively benign course. Gross total resection should be the primary goal of surgery since it may prevent disease progression, and in case it fails, progression occurs later than those that were treated with subtotal resection.

摘要

本研究旨在评估在单一机构接受治疗的小儿脊髓室管膜瘤的临床和放射学特征、组织病理学特征、治疗方式及其有效性,以及长期随访结果。在这项回顾性研究中,我们回顾了1995年至2020年间在本机构接受脊髓室管膜瘤手术治疗的14例小儿患者(3例女性和11例男性)的病历。收集并分析了有关年龄、性别、临床表现和体征、放射学检查结果、术后状态、切除范围、组织病理学分级、复发、肿瘤生长、播散及辅助治疗的数据。6例患者为黏液乳头型室管膜瘤,7例为Ⅱ级室管膜瘤,1例为Ⅲ级室管膜瘤。诊断时的中位年龄为10.5岁(范围1 - 15岁)。最常见的临床表现和体征为轴性和根性疼痛、轻瘫及第一运动神经元受累。10例患者实现了肿瘤全切。5例患者因疾病进展(复发或残留肿瘤生长)接受了二次手术。大多数无疾病进展的患者接受了肿瘤全切治疗。2例患者发生了播散。1例Ⅲ级肿瘤且未实现全切的患者在随访期间死亡。小儿脊髓室管膜瘤是相对少见的肿瘤,病程相对良性。肿瘤全切应是手术的首要目标,因为这可能预防疾病进展,若手术失败,疾病进展也会比接受次全切治疗的患者出现得更晚。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0b94/11666201/ad804c38b4ac/medi-103-e40986-g001.jpg

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