儿童纵隔脂肪母细胞瘤的外科治疗

Surgical treatment of mediastinal lipoblastoma and lipoblastomatosis in children

目的:探讨儿童纵隔脂肪母细胞瘤的临床特点及手术疗效,以期为临床工作提供指导和帮助。方法:收集2007年3月至2019年7月间于首都医科大学附属北京儿童医院胸外科就诊的30例纵隔脂肪母细胞瘤患儿的临床资料,分析其临床表现、影像学特点、治疗方案、病理特征及预后情况。其中,男19例,女11例;中位年龄2.9岁。按手术方法不同,将患儿分为腔镜手术组(14例)和开放手术组(16例),对比分析两组手术时间、术中出血量、胸腔引流管放置时间及住院时间方面的差异,并用秩和检验进行统计学分析。结果:本组患儿表现为咳嗽15例,偶然发现纵隔占位9例,呼吸困难4例、颈部包块4例、霍纳综合征2例。按纵隔四分法,肿瘤位于上纵隔11例,后上纵隔6例,后纵隔5例,前纵隔3例,前上纵隔3例,其他位置2例。术前CT、MRI检查表现为含有脂肪和软组织混合密度的不均匀肿块。所有患儿均行手术治疗,中位手术时间为72.5(25.0~230.0)min,中位术中出血量为4(2~400)ml,中位胸腔引流管放置时间为4(2~17)d,中位住院时间为8(4~30)d。术后病理检查提示局限型脂肪母细胞瘤24例,弥漫型脂肪母细胞瘤6例。腔镜手术组14例患儿术中出血量、手术时间及术后住院时间分别为2(2~5)ml、65(25~115)min和5.5(3~11)d,开放手术组16例患儿上述指标分别为10(2~400)ml、77.5(60~230)min和7.0(5~23)d,前者均较后者少和短,且差异有统计学意义(P均<0.05)。本研究有26例(86.7%)患儿获得随访,中位随访时间为4.7年(2个月~12年),25例术后恢复良好,未见恶性变或转移。1例术后7个月复发,因肿瘤无压迫症状,密切随访中,未再次手术。结论:发生于纵隔内的脂肪母细胞瘤临床较为罕见,影像学检查是重要的辅助手段,病理检查有助于明确诊断,手术切除是有效的治疗方案,术后通常无需联合化疗或放疗。

Objective To review retrospectively clinical data of children with mediastinal lipoblastoma and lipoblastomatosis so as to provide guidance for clinical practices.Methods From March 2007 to July 2019,the relevant clinical data of 30 children with mediastinal lipoblastoma and lipoblastomatosis were collected and their clinical manifestations,imaging features,treatment protocols,pathological features and prognosis analyzed.There were 19 boys and 11 girls with a median age of 2.9(0.5-15.8)years.According to different surgical approaches,they were divided into two groups of thoracoscopy(n=14)and open surgery(n=16).The inter-group differences of operative duration,volume of intraoperative blood loss,indwelling duration of thoracic drainage tube and hospital stay were compared and rank sum test was utilized for statistical analysis.Results There were cough(n=15),mediastinal neoplasm detected during routine physical examination(n=9),dyspnea(n=4),cervical mass(n=4)and Horner's syndrome(n=2).Tumors were located in upper mediastinum(n=11),posterior-upper mediastinum(n=6),posterior mediastinum(n=5),anterior mediastinum(n=3),anterior-upper mediastinum(n=3)and other locations(n=2).Preoperative examinations of computed tomography(CT)and magnetic resonance imaging(MRI)revealed an inhomogeneous mass with mixed density of fat and soft tissue.Surgery was performed for all children.The median operative duration was 72.5(25.0-230.0)min,the median volume of intraoperative blood loss 4(2-400)ml,the median duration of thoracic drainage tube 4(2-17)days and the median hospital stay 8(4-30)days.Postoperative pathology hinted at lipoblastoma(n=24)and lipoblastomatosis(n=6).The median volume of intraoperative blood loss,median operative duration and median postoperative hospital stay of two groups were 2(2-5)ml and 10(2-400)ml,65(25-115)min and 77.5(60-230)min,5.5(3-11)days and 7.0(5-23)days respectively.The former was less and shorter than the latter and the differences were statistically significant(P<0.05).And 26 cases(86.7%)were followed up for a median period of 56.4(2-144)months.One recurrent case at Month 7 postoperatively was not re-operated for a lack of oppressive symptoms.The remainders recovered well and there was no malignant change or metastasis.Conclusions Lipoblastoma and lipoblastomatosis in mediastinum are clinically rare.Imaging examination is an important auxiliary tool while pathological examination aids a definite diagnosis.Surgical resection is efficacious.Usually there is no need for combined postoperative chemotherapy or radiotherapy.