儿童和青少年脊柱原发肿瘤及瘤样病损的临床特点

Review of clinical features of primary spine tumors and tumor-like lesions in children and adolescents

中文关键词:  脊柱原发肿瘤  儿童  青少年  临床特征

英文关键词:Primary spine tumor  Children  Adolescent  Clinical features

中文摘要:

  【摘要】 目的：总结儿童和青少年脊柱原发肿瘤和瘤样病损的临床表现和治疗效果，探讨其临床特点。方法：收集我院骨科2004年2月~2012年5月收治的所有20岁以下的脊柱原发肿瘤及瘤样病损病例的临床资料，分析该年龄段患者的发病情况，比较不同肿瘤患者的症状、体征、影像学表现以及对各种治疗的反应和转归。结果：共收治113例儿童及青少年患者，占同期我院收治所有脊柱原发肿瘤和瘤样病损患者的25.8%。其中良性肿瘤和瘤样病损104例（嗜酸性肉芽肿51例，动脉瘤样骨囊肿12例，骨纤维异样增殖症3例，血管瘤3例，神经鞘瘤6例，神经纤维瘤2例，骨母细胞瘤7例，骨软骨瘤5例，骨样骨瘤7例，骨巨细胞瘤8例）；恶性肿瘤9例（脊索瘤4例，侵袭性骨母细胞瘤2例，原始神经外胚层肿瘤1例，恶性神经鞘瘤2例）。颈椎肿瘤82例（其中上颈椎41例），颈胸段10例，胸椎8例，腰椎8例，腰骶和骶尾椎5共例。单节段88例，多节段25例。除上颈椎外，肿瘤累及椎体32例，累及附件14例，累及椎体＋附件26例。98例（88.5%）以局部疼痛为主诉，其中74例不伴有其他不适，71例疼痛部位病变呈膨胀性生长，27例有病理性骨折。35例患者有神经损害。所有病例影像学检查均有骨质破坏，其中34例伴病理骨折，不同肿瘤病理骨折发生率无显著性差异。依据目前脊柱原发肿瘤的治疗原则，62例接受手术治疗，33例接受单纯放疗，18例未行治疗。70例患者获得8~48个月随访，其中44例为手术患者，术后局部疼痛明显缓解，32例术前有神经损害者22例改善，8例复发（良性肿瘤4例，恶性肿瘤4例），无肿瘤转移者；单纯放疗和观察者症状无进展。结论：儿童和青少年脊柱肿瘤患者多以局部疼痛为唯一症状，尽管影像学检查可见骨质破坏甚至病理骨折，但神经损害相对少见；良性肿瘤比例远高于恶性，依据目前脊柱原发肿瘤治疗原则治疗可取得良好效果。

英文摘要:

  【Abstract】 Objectives: To investigate clinical features of spine tumors by analyzing manifestations and treatment outcome of children and adolescents with primary spine tumors and tumor-like lesions. Methods: Medical records and follow-up data of all primary spine tumor and tumor-like lesion cases where patients were under 20 and treated in our center from Februrary 2004 to May 2012 were analyzed retrospectively. Features of interest include symptoms, physical examination, radiological findings and clinical outcome of various treatments in young patients of various ages and types of tumors. Results: The total number of patients was 113, accounting for 25.8% of all patients treated in our center with the same conditions. Among these cases, 104 were diagnosed of benign tumors and tumor-like lesions(51 cases of eosinophilic granuloma, 12 aneurysmal bone cyst, 3 fibrous dysplasia, 3 hemoangioma, 6 Schwannoma, 2 neurofibroma, 7 osteoblastoma, 5 osteochondroma, 7 osteoid osteoma and 8 giant cell tumor); 9 were diagnosed of malignant tumors(4 cases of chordoma, 2 aggressive osteoblastoma, 1 primary neuroectodermal tumor and 2 malignant peripheral nerve sheath tumor). In 82 cases, tumors were in the cervical spine(41 in the upper cervical spine), 10 in the cervico-thoracic spine, 8 in the thoracic spine and 5 in the lumbo-sacral region. Tumors involved in single spinal segment in 88 patients and multiple segments in 25 patients. In 32 cases, tumors were found in the vertebral bodies, 14 in the appendixes, and 26 in both, outside C1-2 region). Focal pain was the chief complain in 98(88.5%) of all 113 cases, and the only complain in 74 cases. Among whom, 71 was found with a tumor in expansive growth, 27 with pathological fractures. In total, 35 patients presented with neurological deficits. Bone destruction was radiologically visible in all patients, whereas only 34/113 cases were found with pathological fractures. There was no significant difference in the occurrence of pathological fracture amongst various tumors. Our cases were treated based on current principle for the treatment of primary spine tumors, including surgery in 62 cases, radiotherapy in 33 cases and simple observation in 18 cases. Follow-up for 8-48 months was obtained in 70 patients. At the last follow-up, 44 patients who underwent surgery had significant pain relief and 22 out of 32 patients who had preoperative neurological symptoms also saw improvement in neurological function. There were 8 cases of tumor reoccurrence(4 cases of malignant and 4 cases of benign tumor), none metastasis. No change was seen in patients who had radiotherapy alone or no treatment at all. Conclusions: Local pain is usually the only symptom in children and adolescents with primary spinal tumors and tumor-like lesions. Radiological features include destruction of bone and sometimes, pathological fractures, yet seldom associated with neurological deficits. Tumors in this age group are much more likely to be benign than malignant; hence a better treatment outcome is to be expected.