Pancreatoblastoma in children:9 case reports and literature review
10.3760/cma.j.issn.2095-428X.2017.15.011
- VernacularTitle:儿童胰母细胞瘤9例病例报告并文献复习
- Author:
Jing LI
;
Weiling ZHANG
;
Dongsheng HUANG
;
Huimin HU
;
Yizhuo WANG
;
Yuan WEN
;
Tao HAN
;
Fan LI
;
Liping CHEN
- Keywords:
Pancreatoblastoma;
Treatment;
Prognosis
- From:
Chinese Journal of Applied Clinical Pediatrics
2017;32(15):1162-1165
- CountryChina
- Language:Chinese
-
Abstract:
Objective To analyze the clinical features,treatment and prognosis of the pancreatoblastoma (PB).Methods A total of 9 patients including 4 boys and 5 girls diagnosed as pancreatoblastoma in Beijing Tongren Hospital Affiliated to Capital Medical University between July 2008 and February 2016 were enrolled.The clinical features,therapy and prognosis were analyzed.Results (1) Clinical manifestations:6 cases had abdominal mass,1 case had jaundice,2 cases had abdominal pain at the time of diagnosis;5 cases were found in caput pancreatis,2 cases were found in corpora pancreatis and 2 cases were found in cauda pancreatis;4 cases had local tumor,3 cases had local invasion,and 2 cases had liver metastasis at diagnosis.(2) Enzymology and tumor marks:elevated alpha-fetoprotein (AFP) was found in 5 cases (48.5-52 971.0 μg/L),elevated neuron-specific enolase(NSE) was found in 5 cases(22.4-53.4 pg/L),elevated carbohydrate antigen 125 (CA-125) was found in 5 cases [(40.3-122.8) × 103 U/ L],elevated carbohydrate antigen 19-9 (CA19-9) was found in 2 cases [(65.4-362.5) × 103 U/ L],elevated lactate dehydrogenase was found in 2 cases(380 ～ 838 U/L) and elevated hemodiastase was found in 2 cases (105.5-122.8 U/L).(3) Treatment and prognosis:in 9 patients,the tumors were resected at first in 6 patients,and 5 of them remitted until now,1 of them relapsed after the comprehensive treatment for 9 months.Two of the patients underwent operation after the chemotherapy,in which 1 case received complete resection but without chemotherapy after the surgery,whom relapsed 1 year later;and the other one was resected partly.But after stopping the comprehensive treatment for 6 months,the disease relapsed.One patient could not receive operate because of huge tumor size,and the chemotherapy failed to control tumor progression;the patient died without operation.Three relapsed children were still in chemotherapy.The median follow-up period was 21 months (8-98 months).Conclusions AFP may be used as monitoring indicator for PB.Complete tumor resection is an important factor affecting the outcome.Adjuvant chemotherapy may reduce recurrence and prolong survival.
