Zhang Xu,Zeng Qi,Zhang Na,et al.Diagnosis and treatment of pediatric pleuropulmonary blastoma: a report of 38 cases[J].Journal of Clinical Pediatric Surgery,2020,19(01):63-68.[doi:10.3969/j.issn.1671-6353.2020.01.013]
儿童胸膜肺母细胞瘤38例诊治分析
- Title:
- Diagnosis and treatment of pediatric pleuropulmonary blastoma: a report of 38 cases
- Keywords:
- Pleuropulmonary Blastoma; Surgical Procedures; Operative; Treatment; Child
- 分类号:
- R726;R734.3
- 摘要:
- 目的 探讨儿童胸膜肺母细胞瘤的发病年龄、临床表现、病理分型及特点、治疗原则及预后情况。方法 回顾性研究自2002年3月至2018年3月期间首都医科大学附属北京儿童医院收治的38例胸膜肺母细胞瘤患儿临床资料，并进行统计描述。结果 38例中男18例，女20例；首诊诊断为非肿瘤性病变者占比47.4%（18/38），从出现症状到手术或化疗干预的时间范围为5~210 d；Ⅰ型4例（10.5%），中位诊断年龄22个月；Ⅱ型12例（31.6%），中位诊断年龄37个月；Ⅲ型22例（57.9%），中位诊断年龄39.5个月。治疗方式包括手术及化疗。术中见肿瘤最大直径为2~18 cm，手术时间25~210 min，术中出血量2~500 mL（1例出血500 mL病例于术中行胸膜剥脱术）；化疗方案多采用IVADo（异环磷酰胺+阿霉素+长春新碱+放线菌素D）和IVA（异环磷酰胺+长春新碱+放线菌素）方案，5年无瘤生存率为69.2%。结论 胸膜肺母细胞瘤是一种较为罕见的儿童恶性肿瘤，临床表现缺乏特异性，误诊率高，预后较差，需要手术与化疗联合进行治疗。儿科医生应该充分认识到PPB的严重性，对于出现气胸、多发部位肺囊性病变、肺囊肿家族史、PPB家族史、DICER1家族性综合征的胸腔占位性病变患儿应警惕本病的可能，并做到严密监测及随访。
- Abstract:
- Objective To explore the onset age,clinical manifestations,pathological classifications and characteristics,treatment principles and outcomes of pediatric pleuropulmonary blastoma (PPB).Methods Retrospective analysis was conducted for clinical data of 38 PPB children,including age of onset,clinical manifestations,familial disease characteristics,pathological types,treatment and prognosis from March 2002 to March 2018.Results There were 18 boys and 20 girls.The non-neoplastic lesion of initial diagnosis was 47.4%.The intervention time from symptom onset to surgery/chemotherapy was 5 to 210 days.For typeⅠ(n=4,10.5%),the median diagnostic age was 22 months; for type Ⅱ(n=12,31.6%),the median diagnostic age 37 months; for type Ⅲ (n=22,57.9%),the median diagnostic age 39.5 months.The treatments included surgery and chemotherapy.During operation,the maximal diameter of tumor was 2-18 cm,operative duration 25-210 min and intraoperative blood loss 2-500 ml.For one case of pleural exfoliation,the volume of intraoperative bleeding was 500 ml.The chemotherapeutic regimens of IVADo (ifosfamide+vincristine+actinomycin D+doxorubicin)and IVA (ifosfamide+vincristine+actinomycin D)were prescribed.The 5-year disease-free survival rate was 69.2%.Conclusion PPB is a rare form of malignant pediatric tumor.Due to non-specific clinical manifestations,it has a high misdiagnostic rate and a poor prognosis.A combination of surgery and chemotherapy is required for its treatment.Pediatricians should understand its severity and stay on a high alert for pneumothorax,multiple cystic lung lesions,a history of lung cysts,a family history of PPB and chest space-occupying lesions of DICER1 familial syndrome.Close follow-ups are recommended.
参考文献/References:
1 Manivel JC,Priest JR,Watterson J,et al.Pleuropulmonary blastoma:the so-called pulmonary blastoma of childhood[J].Cancer,1988,62(8):1516-1526.DOI:10.1002/1097-0142(19881015)62:8<1516:aid-cncr2820620812>3.0.co;2-3.
2 Feinberg A,Hall NJ,Williams GM,et al.Can congenital pulmonary airway malformation be distinguished from Type Ⅰ pleuropulmonary blastoma based on clinical and radiological features?[J].J Pediatr Surg,2016,51(1):33-37.DOI:10.1016/j.jpedsurg.2015.10.019.
3 Priest JR,Mcdermott MB,Bhatia S,et al.Pleuropulmonary blastoma:a clinicopathologic study of 50 cases[J].Cancer,1997,80(1):147-161.
4 Hill DA,Jarenzbowski JA,Priest JR,et al.Type Ⅰ pleuropulmonary blastoma:Pathology and biology study of 51 cases from the International Pleuropulmonary Blastoma Registry[J].Am J Surg Pathol,2008,32(2):282-295.DOI:10.1097/PAS.0b013e3181484165.
5 Wright JR.Pleuropulmonary blastoma:A case report documenting transition from type I (cystic) to type Ⅲ(solid)[J].Cancer,2000,88(12):2853-2858.DOI:10.1002/1097-0142(20000615)88:12<2853:AID-CNCR28>3.0.CO;2-D.
6 Khan AA,El-Borai AK,Alnoaiji M.Pleuropulmonary blastoma:a case report and review of the literature[J].Case Rep Pathol,2014:509086.DOI:10.1155/2014/509086.
7 Messinger YH,Stewart DR,Priest JR,et al.Pleuropulmonary blastoma:a report on 350 central pathology-confirmed pleuropulmonary blastoma cases by the International Pleuropulmonary Blastoma Registry[J].Cancer,2015,121(2):276-285.DOI:10.1002/cncr.29032.
8 John R,Priest,Watterson J,et al.Pleuropulmonary blastoma:a marker for familial disease[J].J Pediatr,1996,128(2):220-224.DOI:10.1016/s0022-3476(96)70393-1.
9 Hill DA,Ivanovich J,Priest JR,et al.DICER1 mutations in familial pleuropulmonary blastoma[J].Science,2009,325(5943):965.DOI:10.1126/science.1174334.
10 Faure A,Atkinson J,Bouty A,et al.DICER1 pleuropulmonary blastoma familial tumour predisposition syndrome:What the paediatric urologist needs to know[J].J Pediatr Urol,2016,12(1):5-10.DOI:10.1016/j.jpurol.2015.08.012.
11 López-Andreu JA,Ferrís J,Esquembre C,et al.Familial cystic nephroma and pleuropulmonary blastoma[J].Cancer,1993,72(9):2792-2793.DOI:10.1002/1097-0142(19931101)72:9<2792:aid-cncr2820720943>3.0.co;2-a.
12 Boman F,Hill DA,Williams GM,et al.Familial association of pleuropulmonary blastoma with cystic nephroma and other renal tumors:A report from the International Pleuropulmonary Blastoma Registry[J].J Pediatr,2006,149(6):850-854.DOI:10.1016/j.jpeds.2006.08.068.
13 Priest J,Watterson J,Dehner L,et al.Childhood pleuropulmonary blastoma (PPB) as a unique marker for familial childhood neoplasms[C].Proceedings of the American Society of Clinical Oncology,1994:186.
14 Dehner L,Watterson J,Priest J,et al.Pleuropulmonary blastoma:unique intrathoracic pulmonary neoplasm of childhood[J].Perspect Pediatr Pathol,1995,18,214-226.
15 Cai S,Wang X,Zhao W,et al.DICER1 mutations in twelve Chinese patients with pleuropulmonary blastoma[J].Sci China Life Sci,2017,60(7):714-720.DOI:10.1007/s11427-017-9081-x.
16 Miniati DN,Chintagumpala M,Langston C,et al.Prenatal presentation and outcome of children with pleuropulmonary blastoma[J].J Pediatr Surg,2006,41(1):66-71.DOI:10.1016/j.jpedsurg.2005.10.074.
相似文献/References:
[1]杜朝峻,丁力,胡英超,等.手术治疗婴幼儿法洛四联症83例[J].临床小儿外科杂志,2010,9(06):413.
DU Chao jun,DING Li,HU Ying chao,et al.Surgical treatment of Tetralogy of Fallot in infancy: Experiences of 83 cases.[J].Journal of Clinical Pediatric Surgery,2010,9(01):413.
[2]李勇,尹强,周小渔,等.术中胆道造影在先天性胆总管囊肿手术中的应用[J].临床小儿外科杂志,2010,9(06):430.
LI Yong,YIN Qiang,ZHOU Xiao yu,et al.The clinic application experience on intraoperative cholangiography in pediatric surgery.[J].Journal of Clinical Pediatric Surgery,2010,9(01):430.
[3]许芝林,王强,李琰,等.乙状结肠冗长症的术式选择探讨[J].临床小儿外科杂志,2010,9(06):432.
XU Zhi lin,WANG Qiang,LI Yan,et al.Initiatory exploration of the standard of surgical treatment methods for Dolichosigmoid.[J].Journal of Clinical Pediatric Surgery,2010,9(01):432.
[4]常绘文,沈振亚.右腋下小切口在儿童心内手术中的应用[J].临床小儿外科杂志,2010,9(06):439.
[5]胡明,施诚仁,严志龙.脐环小切口治疗婴幼儿腹盆腔疾病的探讨[J].临床小儿外科杂志,2010,9(06):471.
[6]丁勇,魏华,孔燕,等.横裁包皮岛状皮瓣法在尿道下裂治疗中的应用[J].临床小儿外科杂志,2010,9(06):474.
[7]江峰赵阳孙莲萍金惠明马杰.婴幼儿阻塞性脑积水两种手术方式的疗效比较[J].临床小儿外科杂志,2011,10(01):0.
JIANG Feng,ZHAO Yang,SUN Liang ping,et al.Endoscopic third ventricul ostomy versus ventricul operitoneal shunt:clinical study of obstructive hydro cephalus in infants.[J].Journal of Clinical Pediatric Surgery,2011,10(01):0.
[8]王伟程少文彭磊林忠勤张伟陈庆玉寇冬权.弹性髓内钉在儿童肱骨骨折中的应用[J].临床小儿外科杂志,2011,10(01):0.
WANG Wei,CHENG Shaowen,PENG Lei,et al.Application of elastic intramedullary nailing in pediatric humeral fractures.[J].Journal of Clinical Pediatric Surgery,2011,10(01):0.
[9]景登攀张根领孙蔓丽.改良Snodgrass术治疗尿道下裂疗效观察[J].临床小儿外科杂志,2011,10(01):0.
[10]陈快 黄金狮 戴康临 陶俊峰 赖勇强 陶强. 腹腔镜与开腹Ladd手术治疗新生儿肠旋转不良效果比较[J].临床小儿外科杂志,2011,10(02):128.
[J].Journal of Clinical Pediatric Surgery,2011,10(01):128.
备注/Memo
收稿日期:2018-05-30。
通讯作者:曾骐,Email:zengqi-1@163.com