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脊髓内神经母细胞瘤:脊髓压迫的治疗选择及神经学转归

Intraspinal neuroblastoma: Treatment options and neurological outcome of spinal cord compression.

作者信息

Fawzy Mohamed, El-Beltagy Mohamed, Shafei Maged El, Zaghloul Mohamed Saad, Kinaai Naglaa Al, Refaat Amal, Azmy Sarah

机构信息

Pediatric Oncology Department, National Cancer Institute, Cairo University, Cairo, Egypt ; Department of Pediatric Oncology, Children's Cancer Hospital of Egypt (CCHE/57357), Cairo, Egypt.

Department of Neurosurgery, Kasr El-Aini Faculty of Medicine, Cairo, Egypt ; Department of Neurosurgery, Children's Cancer Hospital of Egypt (CCHE/57357), Cairo, Egypt.

出版信息

Oncol Lett. 2015 Feb;9(2):907-911. doi: 10.3892/ol.2014.2795. Epub 2014 Dec 12.

Abstract

Malignant spinal cord compression (MSCC) is a common complication of cancer. Paraspinal neuroblastoma (NB) in the thoracic, abdominal and pelvic regions may extend into the neural foramina causing compression of nerve roots and even the spinal cord. The prompt initiation of specific treatment can improve the neurological outcome. The aim of the present study was to review the clinical features, the management received and the factors that may affect the outcome of patients with MSCC caused by paraspinal NB. During a period between July 2007 and December 2012, a total of 576 NB patients were treated at the Children's Cancer Hospital (Cairo, Egypt). Intraspinal disease extension was present in 51 patients (9%). The children with intraspinal disease extension were reviewed for disease pattern, neurological manifestations and treatment outcome. Children with intraspinal disease extension had an equal male to female ratio (1:1), and approximately two-thirds of patients (34/51) had a clinically manifested cord compression. The duration of neurological manifestations was >4 weeks in 58.8% (20/34) of symptomatic patients and ≤4 weeks in 41.2% (14/34). Subsequent to starting treatment, neurological manifestations showed a complete recovery in 16 patients (47.1%), partial in 11 (32.4%), and stationary course was found in 7 (20.6%). Manifestations of ≤4 weeks in duration carried an improved outcome compared with longer time compression, with a complete recovery in 78.6%, versus 25% for patients with a longer symptom duration (P=0.008). The upfront treatment, patient age and site of the primary tumor did not significantly affect the neurological outcome. Spinal cord compression in NB can be effectively managed with upfront chemotherapy. Initial surgical decompression should be reserved for benign variants only, including ganglioneuroma. Neurological manifestations of <4 weeks duration upon presentation are usually reversible.

摘要

恶性脊髓压迫(MSCC)是癌症的常见并发症。胸、腹和盆腔区域的椎旁神经母细胞瘤(NB)可延伸至神经孔,导致神经根甚至脊髓受压。及时开始特异性治疗可改善神经功能结局。本研究的目的是回顾由椎旁NB引起的MSCC患者的临床特征、接受的治疗以及可能影响结局的因素。在2007年7月至2012年12月期间,共有576例NB患者在儿童癌症医院(埃及开罗)接受治疗。51例患者(9%)存在脊髓内疾病扩展。对有脊髓内疾病扩展的儿童进行了疾病模式、神经表现和治疗结局的评估。有脊髓内疾病扩展的儿童男女比例相等(1:1),约三分之二的患者(34/51)有临床表现的脊髓压迫。58.8%(20/34)有症状的患者神经表现持续时间>4周,41.2%(14/34)≤4周。开始治疗后,16例患者(47.1%)神经表现完全恢复,11例(32.4%)部分恢复,7例(20.6%)病情稳定。与压迫时间较长的患者相比,持续时间≤4周的表现结局更好,78.6%完全恢复,而症状持续时间较长的患者为25%(P=0.008)。前期治疗、患者年龄和原发肿瘤部位对神经功能结局无显著影响。NB引起的脊髓压迫可通过前期化疗有效治疗。初始手术减压仅应保留用于良性病变,包括神经节瘤。就诊时神经表现持续时间<4周通常是可逆的。

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