CAJ | 학술논문

目的总结儿童肾上腺皮质癌的临床特征及诊治效果,提高对儿童肾上腺皮质癌的诊治水平.方法对我院2002年1月至2012年12月收治的16例儿童肾上腺皮质癌的临床资料进行回顾性总结,综合分析临床症状、内分泌功能测定、影像学特点、病理学诊断、治疗方法和随访结果.女10例,男6例,年龄10个月～11岁.肿瘤均为单侧,左8例、右8例,肿瘤直径3～20 mm.结果 16例患儿术后病理确诊为肾上腺皮质癌,功能性肿瘤10例,无功能性肿瘤6例;达到完整肉眼切除瘤体的12例,与周围组织、血管粘连重,有肉眼残留的4例;本组病例术后均安全度过围手术期,无手术死亡病例;均经门诊或电话随访,术后随访6～12年.行肿瘤全切者4例存活至今,发育正常;1例与肾脏粘连严重而行肾脏切除术,术后生存6个月;3例形成瘤栓的生存期分别为3个月、5个月、12个月;1例术后规律化疗4周出现肺转移死亡;术后半年因化疗白细胞低下继发感染死亡2例;术后规律化疗2年,第4年突发脑转移死亡1例;死于车祸1例,失访3例,平均生存期为35个月.结论儿童肾上腺皮质癌恶性程度高、预后差,早期诊断、及时治疗对改善预后起决定性作用.手术切除为首选治疗方式,围手术期使用皮质激素很有必要.手术方式以开腹为宜,不建议腹腔镜微创手术.肾上腺皮质癌的术前、术后化疗并未有效改善此病预后.
목적 총결인동신상선피질암적림상특정급진치효과,제고대인동신상선피질암적진치수평.방법 대아원2002년1월지2012년12월수치적16례인동신상선피질암적림상자료진행회고성총결,종합분석림상증상、내분비공능측정、영상학특점、병이학진단、치료방법화수방결과.녀10례,남6례,년령10개월～11세.종류균위단측,좌8례、우8례,종류직경3～20 mm.결과 16례환인술후병리학진위신상선피질암,공능성종류10례,무공능성종류6례;체도완정육안절제류체적12례,여주위조직、혈관점련중,유육안잔류적4례;본조병례술후균안전도과위수술기,무수술사망병례;균경문진혹전화수방,술후수방6～12년.행종류전절자4례존활지금,발육정상;1례여신장점련엄중이행신장절제술,술후생존6개월;3례형성류전적생존기분별위3개월、5개월、12개월;1례술후규률화료4주출현폐전이사망;술후반년인화료백세포저하계발감염사망2례;술후규률화료2년,제4년돌발뇌전이사망1례;사우차화1례,실방3례,평균생존기위35개월.결론 인동신상선피질암악성정도고、예후차,조기진단、급시치료대개선예후기결정성작용.수술절제위수선치료방식,위수술기사용피질격소흔유필요.수술방식이개복위의,불건의복강경미창수술.신상선피질암적술전、술후화료병미유효개선차병예후.
Objective To analyze the clinical features and prognosis of adrenalcortical carcinoma in children and improve its diagnosis and treatment.Methods Medical records were retrospectively analyzed for 16 patients hospitalized from January 2002 to March 2012.And their clinical symptoms,endocrine function,imaging findings,pathological diagnosis and treatment were discussed.And follow-ups were conducted.Results All cases were pathologically diagnosed as adrenal cortical carcinoma.There were functional (n =10) and non-functional (n =6) tumors.Complete macroscopic resection of tumor was performed for 12 cases while another,4 cases had gross residue.The postoperative period was uneventful.Outpatient or telephone follow-ups were conducted for 6-12 years..All 4 cases of total resection survived with a normal development.One case with severe kidney adhesions underwent nephrectomy with a postoperative survival of 6 months.Three cases with tumor thrombus survived 1,5 and 12 months respectively.One case on a 4-week chemotherapy died from pulmonary metastasis while another 2 cases died 6 months later from bone marrow suppression and infection due to chemotherapy.One case received a 2-year chemotherapy and died from sudden brain metastases.One case died in a car accident While another 3 cases were lost.The average survival time was 35 months.Conclusions Adrenal cortical carcinoma is a rare tumor with a high degree of malignancy and a poor prognosis in children.Surgical excision remains main treatment for this disease.The perioperative application of corticosteroids is absolutely necessary.Laparotomy is preferred over laparoscopic operation.In fact,preoperative and postoperative chemotherapies fail to improve the prognosis.