婴幼儿原发性腹膜后畸胎瘤36例的诊断与治疗

Diagnosis and management of primary retroperitoneal teratoma in 36 infants

目的探讨婴幼儿原发性腹膜后畸胎瘤的临床特点和治疗效果。方法回顾性分析2015年8月至2017年8月在广州市妇女儿童医疗中心肿瘤外科住院诊断并治疗，且病理证实为腹膜后畸胎瘤的患儿共36例。收集患儿性别、年龄、手术时间、出血量、肿瘤质量等资料，患儿术前均完善超声和计算机断层扫描（CT）检查，围术期实验室检查包括血甲胎蛋白（AFP）及人绒毛膜促性腺激素（HCG）。肿瘤按照Norris病理分型分为成熟性畸胎瘤组（27例）和未成熟组（Ⅰ级1例，Ⅱ级4例，Ⅲ级4例）。结果本研究中男9例，女27例；手术时平均年龄11.4个月（7 d～7岁）；肿瘤质量736.47 g（7.90～2 355.00 g）；平均手术时间2.88 h（1.08～7.06 h）；出血量29.11 mL（2～150 mL）；影像学检查发现25例（69.4%）肿瘤位于左腹部，11例（30.6%）位于右腹部，且83.3%（30/36例）的肿瘤跨越中线；患儿均获得随访，平均随访时间6.56个月（9 d～23个月）。术前AFP水平为7 593.1 μg/L（1.7～80 000.0 μg/L），术后AFP水平为891.9 μg/L（1.4～16 519.6 μg/L），手术前后比较差异有统计学意义（P＝0.001）。其中，成熟组术前AFP水平为5 439.6 μg/L（1.7～80 000.0 μg/L），术后AFP水平为1 130.6 μg/L（1.4～16 519.6 μg/L），手术前后比较差异有统计学意义（P＝0.001）；未成熟组术前AFP水平为11 182.3 μg/L（17.4～80 000.0 μg/L），明显高于成熟组，2组比较差异有统计学意义（P＝0.006）；末次随访时AFP水平为38.3 μg/L（1.4～325.4 μg/L），其中成熟组14.3 μg/L（1.4～43.3 μg/L），未成熟组78.4 μg/L（1.7～325.4 μg/L），2组比较差异有统计学意义（P=0.028）；未成熟组中化疗患儿随访时AFP水平平均为54.9 μg/L（2.6～116.6 μg/L），较其术后AFP水平[265.2 μg/L（206.8～384.1 μg/L）]明显降低，差异有统计学意义（P＝0.042）。患儿均行手术治疗，腹腔镜下手术切除3例（8.33%），且未成熟组9例中4例（44.4%）患儿术后接受化疗；手术时间与肿瘤质量、部位及Norris病理分型均无明显相关性（均P〉0.05）。本研究中手术并发症的发生率为42.1%，其中，肿瘤破裂6例，血管损伤5例，消化道损伤4例，未完整切除肿瘤导致肿瘤复发1例。无死亡病例。结论婴幼儿原发性腹膜后畸胎瘤具有典型的临床特点，且多数可手术完整切除，但手术并发症并不少见；AFP作为一种肿瘤复发相关的预测性指标，化疗可明显降低术后AFP水平。

ObjectiveTo investigate the clinical manifestations and surgical effectiveness of the primary retroperitoneal teratoma in infants.MethodsThe records of 36 patients were retrospectively reviewed who were diagnosed as primary retroperitoneal teratoma and treated at the Department of Pediatric Oncology, Guangzhou Women and Children′s Medical Center between August 2015 and August 2017.The related data were collected, including gender, age, operation time, operational bleeding and tumor weight.All patients took the ultrasound, computed tomography（CT） before surgery, alpha-fetal protein（AFP） and human chorionic gonadotrophin（HCG） were detected during peri-operation period.All cases were classified into 0 to Ⅲ grades according to the Norris classification （27 patients of 0 grade, 1 patient of Ⅰ grade, 4 patients of Ⅱ grade, 4 patients of Ⅲ grade）.ResultsThere were 9 males and 27 females in the study.All patients were treated surgically at the age of 11.4 months （7 days-7 years） on the average; the mean weight of incised tumor was 736.47 g （7.90-2 355.00 g）; operation time was 2.88 hours （1.08-7.06 hours）; the amount of bleeding during operation was 29.11 mL （2-150 mL）. All patients received follow-ups for 6.56 months（9 days-23 months） on the average.Radiographs found that tumors in 25 patients （69.4%） were located in the left side of the abdominal cavity, and the rest of them were located in the right side.In addition, tumors in 30 patients （83.3%） were big enough to pass across the mid-line of the body.Average pre-operation AFP was 7 593.1 μg/L（1.7-80 000.0 μg/L）, post-operation AFP was 1 339.5 μg/L（1.4-16 519.6 μg/L）, and the difference was statistically significant （P=0.001）; pre-operation AFP of the mature group was 5 439.6 μg/L（1.7-80 000.0 μg/L）, post-operation AFP of the mature group was 1 130.6 μg/L（1.4-16 519.6 μg/L）, and the difference was statistically significant （P=0.001）; pre-operation AFP of the immature group [11 182.3 μg/L（17.4-80 000.0 μg/L）] was higher than that of the mature group, and the difference was statistically significant（P=0.006）. On the final follow-up, AFP was mean 38.3 μg/L（1.4-352.4 μg/L）; among them, AFP of the mature group was mean 14.3 μg/L（1.4-43.3 μg/L）, the immature group was 78.4 μg/L（1.7-325.4 μg/L）, and the difference was statistically significant（P=0.028）; AFP of 4 patients in the immature group who underwent chemotherapy was 54.9 μg/L （2.6-116.6 μg/L） on the average, lower than those of post-operation AFP of 265.2 μg/L （206.8-384.1 μg/L）, and the difference was statistically significant（P=0.042）. All patients were treated surgically, and 3 cases of them received laparoscope technique; no significant relationship was proved between time of surgery and the tumor weight, location, and Norris classification（all P〉0.05）. The incidence rate of the surgical complications was 42.1%, such as tumor rupture （6 patients）, vascular injury （5 patients）, digestive tract damage （4 patients） and incomplete incision （1 patient）. There was no death case in the serials.ConclusionsPrimary retroperitoneal teratoma can be completely incised.However, the complications of surgery could appear in many cases.As a predictive index for the recurrence retroperitoneal teratoma, AFP can be diminished by chemotherapy following the surgery.