Pancreatoblastoma in children:9 case reports and literature review / 中华实用儿科临床杂志

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 manifestations6 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 markselevated 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 prognosisin 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.