中华小儿外科杂志
中華小兒外科雜誌
중화소인외과잡지
CHINESE JOURNAL OF PEDIATRIC SURGERY
2015年
4期
269-272
,共4页
柳龚堡%刘百慧%马阳阳%李凯%董岿然
柳龔堡%劉百慧%馬暘暘%李凱%董巋然
류공보%류백혜%마양양%리개%동규연
肝肿瘤%婴儿,新生%预后
肝腫瘤%嬰兒,新生%預後
간종류%영인,신생%예후
Liver neoplasms%Infant,newborn%Prognosis
目的 总结新生儿肝母细胞瘤的临床表现与治疗结果,提高对新生儿病例的认识,探讨更有效的治疗方案.方法 回顾性分析2007年至2013年在本院诊断及治疗的初诊年龄小于60 d的8例新生儿肝母细胞瘤病例,其中男6例,女2例,初诊年龄2～50d,平均(24.75±17.62)d,出生体重2.6～4.0kg,平均(3.42±0.47)kg.有7例肿瘤来源于右叶,1例来源于左叶.按照PRETEXT分期标准,Ⅰ期与Ⅱ期各1例,Ⅲ期6例.按照病理类型均为上皮型肝母细胞瘤,其中纯胎儿型1例,胚胎与胎儿混合型7例.Ⅰ期与Ⅱ期病例均行一期肿瘤根治性切除,6例Ⅲ期病例均经开腹肝脏肿瘤活检,明确病理类型后予辅助化疗,其中5例行延期肿瘤切除术.术前平均(4.17±1.16)个疗程,中位时间4疗程.肿瘤切除术后首次化疗时间平均为(22.00±9.42)d,中位时间23.5 d.术后化疗平均(5.00±2.52)个疗程,中位时间4.5疗程.结果 7例肿瘤切除术中有1例出现右肝管损伤,行肝管-空肠Roux-en-Y吻合术修复,其余6例手术无术后并发症.有2例(2/7,28.6％)术后复发,其余5例现均无瘤生存.中位随访时间29.5个月.Kaplan-Meier曲线计算后显示2年、5年的总体生存率分别为75％、60％.结论 正常足月新生儿肝母细胞瘤经过规范化治疗后仍具有良好的预后.由于新生儿肝母细胞瘤非常罕见,需要多中心、多学科联合研究.
目的 總結新生兒肝母細胞瘤的臨床錶現與治療結果,提高對新生兒病例的認識,探討更有效的治療方案.方法 迴顧性分析2007年至2013年在本院診斷及治療的初診年齡小于60 d的8例新生兒肝母細胞瘤病例,其中男6例,女2例,初診年齡2～50d,平均(24.75±17.62)d,齣生體重2.6～4.0kg,平均(3.42±0.47)kg.有7例腫瘤來源于右葉,1例來源于左葉.按照PRETEXT分期標準,Ⅰ期與Ⅱ期各1例,Ⅲ期6例.按照病理類型均為上皮型肝母細胞瘤,其中純胎兒型1例,胚胎與胎兒混閤型7例.Ⅰ期與Ⅱ期病例均行一期腫瘤根治性切除,6例Ⅲ期病例均經開腹肝髒腫瘤活檢,明確病理類型後予輔助化療,其中5例行延期腫瘤切除術.術前平均(4.17±1.16)箇療程,中位時間4療程.腫瘤切除術後首次化療時間平均為(22.00±9.42)d,中位時間23.5 d.術後化療平均(5.00±2.52)箇療程,中位時間4.5療程.結果 7例腫瘤切除術中有1例齣現右肝管損傷,行肝管-空腸Roux-en-Y吻閤術脩複,其餘6例手術無術後併髮癥.有2例(2/7,28.6％)術後複髮,其餘5例現均無瘤生存.中位隨訪時間29.5箇月.Kaplan-Meier麯線計算後顯示2年、5年的總體生存率分彆為75％、60％.結論 正常足月新生兒肝母細胞瘤經過規範化治療後仍具有良好的預後.由于新生兒肝母細胞瘤非常罕見,需要多中心、多學科聯閤研究.
목적 총결신생인간모세포류적림상표현여치료결과,제고대신생인병례적인식,탐토경유효적치료방안.방법 회고성분석2007년지2013년재본원진단급치료적초진년령소우60 d적8례신생인간모세포류병례,기중남6례,녀2례,초진년령2～50d,평균(24.75±17.62)d,출생체중2.6～4.0kg,평균(3.42±0.47)kg.유7례종류래원우우협,1례래원우좌협.안조PRETEXT분기표준,Ⅰ기여Ⅱ기각1례,Ⅲ기6례.안조병리류형균위상피형간모세포류,기중순태인형1례,배태여태인혼합형7례.Ⅰ기여Ⅱ기병례균행일기종류근치성절제,6례Ⅲ기병례균경개복간장종류활검,명학병리류형후여보조화료,기중5례행연기종류절제술.술전평균(4.17±1.16)개료정,중위시간4료정.종류절제술후수차화료시간평균위(22.00±9.42)d,중위시간23.5 d.술후화료평균(5.00±2.52)개료정,중위시간4.5료정.결과 7례종류절제술중유1례출현우간관손상,행간관-공장Roux-en-Y문합술수복,기여6례수술무술후병발증.유2례(2/7,28.6％)술후복발,기여5례현균무류생존.중위수방시간29.5개월.Kaplan-Meier곡선계산후현시2년、5년적총체생존솔분별위75％、60％.결론 정상족월신생인간모세포류경과규범화치료후잉구유량호적예후.유우신생인간모세포류비상한견,수요다중심、다학과연합연구.
Objective To summarize the clinical manifestations and outcomes of neonatal hepatoblastoma for its improved recognization and more effective treatment.Methods For this retrospective study,8 patients with neonatal hepotoblastoma were admitted from 2007 to 2014.Only neonates and infants aged under 2 months with adequate clinicopathologic data were accepted for review.There were 6 boys and 2 girls with a mean diagnostic age of 24.75 ± 17.62 (2-50) days.The mean birth weight at diagnosis was 3.42 ± 0.47 (2.6-4.0) kg.Seven tumors were derived from right hepatic lobe while another from left hepatic lobe.According to the criteria of PRETEXT,the stages were Ⅰ (n =1),Ⅱ (n =1) and Ⅲ (n =6).According to the histology classification,all tumors were of epithelial type.One patient was of pure fetal histology subtype while the remainder mixed fetal & embryonal subtype.Patients with stages Ⅰ and Ⅱ underwent primary operation while those with stage Ⅲ open biopsy and neoadjuvant chemotherapy.Five patients had delayed operation.The mean interval of preoperative chemotherapy was 4.17 ± 1.16 cycles,median 4 cycles.The mean time of initiating postoperative chemotherapy was 22.00 ± 9.42 days,median 23.5 days.The mean interval of postoperative chemotherapy was 5.00 ± 2.52 cycles,median 4.5cycles.Results Right hepatic duct was injured and subsequently repaired with cholangio-jejunostomy in 1 case.There were no other complications.Two tumors recurred postoperatively.Another 5 patients survived tumor-free.The median period of follow-up was 29.5 months.The 2,5-year overall survivals were 75％ and 60％ respectively.Conclusions Normal term newborns with hepatoblastoma have a good prognosis after standard treatments.Neonatal hepatoblastoma is rare so that it is necessary to conduct a multicenter and multidisciplinary study.
