399例儿童畸胎瘤的单中心临床诊疗分析

摘要

目的：回顾性分析小儿畸胎瘤的治疗及预后特点。方法收集重庆医科大学附属儿童医院1994年10月至2015年6月收治的畸胎瘤患儿，共399例，按照性别、年龄、部位、病理类型、血清AFP 水平、治疗及预后等因素进行回顾性分析。结果①成熟型畸胎瘤301例；未成熟畸胎瘤17例，其中组织学Ⅰ级9例，Ⅱ级5例，Ⅲ级3例；恶性畸胎瘤81例，按 COG／CCG 肿瘤分期标准，Ⅰ期5例，Ⅱ期14例，Ⅲ期44例，Ⅳ期18例。②成熟型畸胎瘤及未成熟型畸胎瘤共318例一期接受手术治疗，其中未成熟畸胎瘤Ⅲ级及部分Ⅱ级病例术后辅以化疗；81例恶性肿瘤患儿中，Ⅰ、Ⅱ期均手术完全或完整切除肿瘤，部分Ⅱ期病例术后辅以化疗；40例接受术前新辅助化疗、手术治疗、术后化疗的个体化综合治疗；另外22例中断治疗并放弃；③术后随访1～10年，3例成熟型畸胎瘤患儿术后良性复发，再次予以完整切除后，截止随访时间，301例均无瘤存活；1例未成熟畸胎瘤Ⅲ级术后恶性复发，放弃治疗后死亡；恶性畸胎瘤完成治疗的患儿仅1例恶性复发后死亡，未完成治疗患儿中10例死亡，其余患儿各有不同程度的病情进展。结论在儿童畸胎瘤治疗中，一期完整切除肿瘤，避免肿瘤破溃是预防复发及恶变的关键，未成熟畸胎瘤依据 AFP、影像学、肿瘤组织分级、病理免疫组化、术中情况等选择是否化疗；对于恶性畸胎瘤，彻底手术切除的同时结合化疗，术后密切随访，可获得较高的生存率。%Objetive To explore the characteristics and clinical experiences of diagnosing and treating pediatric teratomas.Methods From October 2004 to June 2015,a total of 150 cases of pediatric teratomas were reviewed retrospectively.Results The histopathologic types of teratoma were mature (n =301),imma-ture (n =17)(histological levelⅠ,n =9;Ⅱ,n =5;Ⅲ,n =3)and malignant (n =81 ).According to the tumor staging standard of COG/CCG,the periods were Ⅰ (n =5),Ⅱ (n =14),Ⅲ(n =44)and IV (n =18).All 318 cases of mature and immature teratomas underwent primary surgery while histological level Ⅲ and several cases of level Ⅱreceived postoperative chemotherapy.Forty cases received comprehensive treatments including preoperative neoadjuvant chemotherapy,surgery and postoperative chemotherapy while another 22 ca-ses failed to finish treatment.During a follow-up period of 1 ~10 years,3 benign cases of mature teratoma were recurrent and had a complete remission after secondary complete resection.And all 301 cases survived tumor-free until the latest follow-up deadline.One case of immature teratoma of histological level Ⅲ died from malig-nant recurrence after surgery.All malignant cases completing treatment survived except for one dying from ma-lignant recurrence.Those cases with interrupted treatment had 10 deaths.The remaining cases had progres-sion. Conclusions Complete removal of tumors and prevention of tumor rupture are essential for preventing re-currence of malignancy.Whether offering chemotherapy or not for immature teratoma depends on if tumor tissue is completely removed and if tumor has infiltration relationship with surrounding tissues,imaging expression and index of alpha feto-protein.For completely removed malignant teratomas,postoperative chemotherapy may a-chieve a higher survival rate.