小儿腹膜后成熟畸胎瘤合并扩张型心肌病1例并文献回顾

Retroperitoneal mature teratoma complicating dilated cardiomyopathy in an infant:A case report and literature review

目的探讨小儿腹膜后成熟畸胎瘤合并扩张型心肌病(dilated cardiomyopathy,DCM)的诊治经验。方法回顾性分析2013年上海交通大学医学院附属上海儿童医学中心普外科收治的1例小儿腹膜后成熟畸胎瘤合并DCM的临床资料、病理特征、主要治疗方案及预后。结果患儿女,4个月24 d,足月顺产,发现腹腔占位4个月余入院。腹部CT提示左侧腹腔巨大混合型密度肿块,内见脂肪、结节状钙化及液体密度影,伴少量腹水,考虑成熟型畸胎瘤。术前血清甲胎蛋白(alpha-fetoprotein,AFP)7.85μg/L。心脏彩超(外院)示左心室球样扩张,左心收缩功能下降,二尖瓣轻-中度反流,左心室射血分数(left ventricle ejection fraction,LVEF)约29.1%。心肌肌钙蛋白T(cardiac troponin T,cTn-T):0.89μg/L,β人绒毛膜促性腺激素(β-human chorionic gonadotropin,β-HCG):0.02 TU/ml,肌酸激酶同工酶(creatine kinase isoenzyme,CKMB):2.1 U/L,N端前脑钠肽(N terminal pro B type natriuretic peptide,NT-proBNP):3076 pg/ml。排除贫血、高血压、高血容量和儿茶酚胺分泌过多。病毒学和代谢等检查正常。术中探查见肿瘤来源于腹膜后,包膜完整,囊实性混合,肿瘤大小约20 cm×15 cm×11 cm,表面不规则。病理诊断:(腹膜后)畸胎瘤,组织分化成熟,见神经胶质组织等,另见肾上腺组织。手术切除肿瘤后,扩张型心肌病逐渐好转,心功能逐渐恢复,LVEF由术前29.1%上升到术后3个月49.2%、术后6个月68.1%、至今68.3%,AFP由术前7.85 ng/ml下降到术后3个月6.65 ng/ml、术后6个月3.75 ng/ml、至今3.70 ng/ml,NT-proBNP由术前3076 pg/ml下降到术后3个月1580 pg/ml、术后6个月157 pg/ml、至今150 pg/ml。截至2019年5月患儿病情平稳。结论小儿腹膜后成熟畸胎瘤合并DCM是一种儿童罕见病症,根据畸胎瘤的性质、分期及分级等采取手术治疗,手术切除巨大腹膜后畸胎瘤体后,扩张型心肌病可以得到缓解。

Objective To explore the experience of diagnosis and treatment of retroperitoneal benign teratoma complicated with dilated cardiomyopathy(DCM)in an infant.Methods Clinical data,pathological characteristics,therapeutic schedule and prognosis of a case of retroperitoneal mature teratoma complicating DCM admitted to Shanghai Pediatric Medical Center Affiliated to Shanghai Jiaotong University Medical College in 2013 were retrospectively analyzed,and related literatures at home and abroad were also reviewed.Results The case was a baby girl who was 4 months and 24 days old.She was born at full term and was hospitalized for detecting abdominal tumor for 4 months.Abdominal CT showed a large hypodense heterogeneous mass in the left abdominal cavity containing nodular calcification,fat,and liquid,and a small amount of peritoneal effusion.Mature teratoma was considered.Preoperative serum AFP was 7.85μg/L.Cardiac ultrasound showed globular dilatation of left ventricle,decreased left ventricular systolic function and mild to moderate mitral regurgitation,with LVEF of 29.1%.cTn-T of 0.89μg/L,B-HCG of 0.02 TU/ml,CKMB of 2.1 U/L,and NT-proBNP of 3076 pg/ml.Mia and excessive catecholamine secretion were excluded.The results of virological and metabolic tests were normal.Intraoperative exploration revealed that the tumor originated from the posterior peritoneum.The tumor capsule was intact and mixed with cysts and solid.The size of the tumor was about 20 cm×15 cm×11 cm,and the surface of the tumor was irregular.Pathological diagnosis was(retroperitoneal)teratoma(mature),with glial tissue and adrenal tissue.After resection of the tumor,dilated cardiomyopathy was gradually improved and cardiac function gradually recovered.LVEF increased from 29.1%(before operation)to 49.2%(after 3 months),68.1%(after 6 months)and 68.3%(up to now).AFP decreased from 7.85 ng/ml(before operation)to 6.65 ng/ml(after 3 months),3.75 ng/ml(after 6 months)and 3.70 ng/ml(up to now).NT-proBNP decreased from 3076 pg/ml(before operation)to 1580 pg/ml(after 3 months),157 pg/ml(after 6 months)and 150 pg/ml(up to now).Till May 2019,the patient’s condition was stable.Conclusion Retroperitoneal mature teratoma complicated with DCM is a rare disease in infants.According to the nature,stage and grade of teratoma,appropriate surgery or surgery plus chemotherapy should be applied.Associated DCM can be alleviated after resection of the tumor.