小儿骶骨肿瘤的临床特点及外科治疗
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摘要
目的发生于儿童的骶骨肿瘤非常罕见,笔者旨在对其病理类型、诊疗过程、疾病转归等进行归纳总结。方法 2000年1月至2013年12月,北京大学人民医院骨与软组织肿瘤中心共收治14岁(含)以下儿童骶骨肿瘤患者20例,通过回顾其住院病历、影像学检查及病理学结果,总结其临床特征及诊断要点。20例中,尤文肉瘤/原始神经外胚层肿瘤(PNET) 7例,骨肉瘤3例,骨母细胞瘤、血管瘤各2例,畸胎瘤、软骨母细胞瘤、神经鞘瘤、动脉瘤样骨囊肿、嗜酸性肉芽肿、血管内皮细胞瘤各1例。7例以排便/排尿功能障碍为主要症状,13例以骶尾部疼痛为首发症状,11例入院查体可触及骶前包块。20例均接受手术治疗。统计学采用Kaplan-Meier法测算生存率。结果 13例以疼痛为主要症状的患儿出院时疼痛症状明显减轻。7例以骶神经功能障碍为主要症状的患儿中,3例明显改善,3例无明显变化,1例较术前恶化,并出现行走功能障碍。Kaplan-Meier生存曲线显示总体2年生存率70.6%,5年生存率64.7%。结论儿童骶骨肿瘤极其罕见,以尤文肉瘤/PNET及骨肉瘤等原发肿瘤为主。由于此类肿瘤高度恶性,因而一旦患儿诊断考虑骶骨肿瘤,应尽快完善相关影像学检查并获得病理学证据,制订治疗方案,以挽救患儿生命,并最大程度上保留骶神经功能。
Objective Sacral tumors in children are very rare. The purpose of this research is to investigate and discuss the clinical characteristics, pathological types, therapeutic process and prognosis of such lesions. MethodsFrom Jan. 2000 to Dec. 2013, the study retrospected 20 patients( ≤ 14 years) who were diagnosed as sacral tumors. Each patient's hospital records, radiographic reports and pathological results were reviewed. The pathological types of these lesions were Ewing sarcoma/primitive neuroectodermal tumor( PNET, n = 7), osteosarcoma( n = 3), osteoblastoma( n = 2), hemangioma( n = 2), teratoma( n = 1), chondroblastoma( n = 1), schwannoma( n = 1), aneurysmal bone cyst( n = 1), eosinophilic granuloma( n = 1) and hemangioendothelioma( n = 1). Seven patients had sacral nerve impairment as initial symptoms and the rest 13 complained with sacrococcygeal pain. Eleven patients presented with presacral mass during rectal examination. All 20 patients received surgical treatment. The survival rate was described with Kaplan-Meier survival curve. Results Thirteen patients with sacrococcygeal pain had their symptoms all relieved. Three of the 7 patients with sacral nerve impairment experienced improvement of the symptoms while 3 patients found no much change. One patient found the symptom deteriorated and suffered ambulation dysfunction postoperatively. The mean 2-year survival rate was 70.6%, 5-year survival rate 64.7%. Conclusions Sacral tumors in children are very rare, and most of them are primary tumors like Ewing sarcoma/PNET or osteosarcoma, which are usually highly malignant. Once a child is suspected with sacral tumors, prompt radiological and pathological examinations should be performed and therapeutic strategy should be made in order to improve the survival and preserve the sacral nerve function as much as possible.
Objective Sacral tumors in children are very rare. The purpose of this research is to investigate and discuss the clinical characteristics, pathological types, therapeutic process and prognosis of such lesions. MethodsFrom Jan. 2000 to Dec. 2013, the study retrospected 20 patients( ≤ 14 years) who were diagnosed as sacral tumors. Each patient's hospital records, radiographic reports and pathological results were reviewed. The pathological types of these lesions were Ewing sarcoma/primitive neuroectodermal tumor( PNET, n = 7), osteosarcoma( n = 3), osteoblastoma( n = 2), hemangioma( n = 2), teratoma( n = 1), chondroblastoma( n = 1), schwannoma( n = 1), aneurysmal bone cyst( n = 1), eosinophilic granuloma( n = 1) and hemangioendothelioma( n = 1). Seven patients had sacral nerve impairment as initial symptoms and the rest 13 complained with sacrococcygeal pain. Eleven patients presented with presacral mass during rectal examination. All 20 patients received surgical treatment. The survival rate was described with Kaplan-Meier survival curve. Results Thirteen patients with sacrococcygeal pain had their symptoms all relieved. Three of the 7 patients with sacral nerve impairment experienced improvement of the symptoms while 3 patients found no much change. One patient found the symptom deteriorated and suffered ambulation dysfunction postoperatively. The mean 2-year survival rate was 70.6%, 5-year survival rate 64.7%. Conclusions Sacral tumors in children are very rare, and most of them are primary tumors like Ewing sarcoma/PNET or osteosarcoma, which are usually highly malignant. Once a child is suspected with sacral tumors, prompt radiological and pathological examinations should be performed and therapeutic strategy should be made in order to improve the survival and preserve the sacral nerve function as much as possible.
引文
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[8]Wang Y,Guo W,Shen D,et al.Surgical treatment of primary osteosarcoma of the sacrum:a case series of 26 patients[J].Spine,2017,42(16):1207-1213.
[9]Hesla AC,Tsagozis P,Jebsen N,et al.Improved prognosis for patients with ewing sarcoma in the sacrum compared with the innominate bones:the scandinavian sarcoma group experience[J].J Bone Joint Surg Am,2016,98(3):199-210.
[10]李晓,郭卫,杨荣利,等.骶骨原发尤文肉瘤15例分析[J].中国骨与关节杂志,2014,3(2):100-104.
[11]Ruggieri P,Huch K,Mavrogenis AF,et al.Osteoblastoma of the sacrum:report of 18 cases and analysis of the literature[J].Spine,2014,39(2):E97-103.
[12]郭卫,王臻,郭征,等.尤文肉瘤肿瘤家族(ESFT)临床循证诊疗指南[J].中华骨与关节外科杂志,2018(4).
[13]Ng VY,Jones R,Bompadre V,et al.The effect of surgery with radiation on pelvic Ewing sarcoma survival[J].J Surg Oncol,2015,112(8):861-865.
[14]Li D,Guo W,Qu H,et al.Experience with wound complications after surgery for sacral tumors[J].Eur Spine J,2013,22(9):2069-2076.
[2]郭卫,李大森,蔚然,等.单中心原发骶骨肿瘤790例的流行病学分析[J].中国脊柱脊髓杂志,2014,24(11):971-978.
[3]Ozdemir MH,Gürkan I,Yildiz Y,et al.Surgical treatment of malignant tumours of the sacrum[J].Eur J Surg Oncol,1999,25(1):44-49.
[4]Huang L,Guo W,Yang R,et al.Proposed scoring system for evaluating neurologic deficit after sacral resection:functional outcomes of 170 consecutive patients[J].Spine,2016,41(7):628-637.
[5]Tomita K,Tsuchiya H.Total sacrectomy and reconstruction for huge sacral tumors[J].Spine,1990,15(11):1223-1227.
[6]Tang X,Guo W,Yang R,et al.Use of aortic balloon occlusion to decrease blood loss during sacral tumor resection[J].J Bone Joint Surg Am,2010,92(8):1747-1753.
[7]郭卫,杨荣利.骶骨肿瘤的手术治疗[J].中华外科杂志,2003,(11):30-34.
[8]Wang Y,Guo W,Shen D,et al.Surgical treatment of primary osteosarcoma of the sacrum:a case series of 26 patients[J].Spine,2017,42(16):1207-1213.
[9]Hesla AC,Tsagozis P,Jebsen N,et al.Improved prognosis for patients with ewing sarcoma in the sacrum compared with the innominate bones:the scandinavian sarcoma group experience[J].J Bone Joint Surg Am,2016,98(3):199-210.
[10]李晓,郭卫,杨荣利,等.骶骨原发尤文肉瘤15例分析[J].中国骨与关节杂志,2014,3(2):100-104.
[11]Ruggieri P,Huch K,Mavrogenis AF,et al.Osteoblastoma of the sacrum:report of 18 cases and analysis of the literature[J].Spine,2014,39(2):E97-103.
[12]郭卫,王臻,郭征,等.尤文肉瘤肿瘤家族(ESFT)临床循证诊疗指南[J].中华骨与关节外科杂志,2018(4).
[13]Ng VY,Jones R,Bompadre V,et al.The effect of surgery with radiation on pelvic Ewing sarcoma survival[J].J Surg Oncol,2015,112(8):861-865.
[14]Li D,Guo W,Qu H,et al.Experience with wound complications after surgery for sacral tumors[J].Eur Spine J,2013,22(9):2069-2076.