Clinical analysis of 10 cases of pancreatoblastoma in children

Abstract

Objective To analyze the clinical characteristics, treatment and prognostic factors of children with pancreaticoblastoma (PBL). Methods The clinical characteristics, treatment and outcome of 10 children with PBL admitted to the First Affiliated Hospital of Zhengzhou University from January 2008 to March 2018 were retrospectively analyzed, and the related factors of prognosis were analyzed. Results Ten children were with PBL, aged 2-7 years, with the median age of 5 years, and the ratio of male to female was 1∶1. In the initial diagnosis, 9 cases had palpable abdominal mass, including 4 cases of abdominal pain, 2 cases of diarrhea, 1 case of jaundice, 1 case of anemia and 1 case of vomiting symptoms. There were 3 cases of pancreatic head tumor, 5 cases were in body and tail of pancreas, and 2 cases were in tail of pancreas. Postoperative immunohistochemical results showed 6 cases of Sny (+ ) or (leison+ ) , 10 cases of cytokeratin (+ ), 8 cases of α -antitryptase (+ ), 9 cases of ki-67 (> 30%+ ), 1 case of ki-67 (30%), 3 cases of CD56 (+ ) or (leison+ ) , 9 cases of α - fetoprotein (-), 1 case of α-fetoprotein (leison+ ) . One case did not receive chemotherapy and died after 5 months. The remained 9 cases received chemotherapy after operation. During 0.9 to 5.0 years follow-up, 4 cases of 9 patients survived without tumors, 1 case lost follow-up and 4 cases died. Conclusions PBL is a rare malignant tumor, which can occure in each part of the pancreas. The malignancy, recurrence and mortality of PBL are high. Complete resection and progression of tumors are important factors affecting prognosis. Chemotherapy can remove some of the tumors that cannot be surgically removed. Lomplete excision adjuvant with chemotherapy can bring some patients a long time of survival. Key words: Pancreatoblastoma; Children; Prognosis