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"Pancreatoblastoma"

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"Pancreatoblastoma"

Original Article

[English]
Pediatric Pancreatic Tumors-Clinical Experience
Hyung Woo Park, Dae Yeon Kim, Min Jeong Cho, Tae Hun Kim, Seong Cheol Kim, In Ku Kim
J Korean Assoc Pediatr Surg 2010;16(1):11-17.   Published online June 30, 2010
DOI: https://doi.org/10.13029/jkaps.2010.16.1.11

Pancreatic tumors in children are relatively rare, and their prognosis differs from that in adults. The purpose of this study is to examine the clinical characteristics, treatment, and prognosis for children with pancreatic tumors. We retrospectively reviewed the medical records of children under 15 years of age with pancreatic tumors who were treated surgically at Asan Medical Center between January 1992 and November 2009. There were 16 patients, fourteen of whom were pathologically diagnosed with solid pseudopapillary tumor. The other two patients were diagnosed with pancreatoblastoma and acinar cell carcinoma, respectively. Six patients of the 16 patients (38%) were male, and there was a male-to-female ratio of 1:1.6. The initial presentations were upper abdominal pain in eight patients (50%), palpable abdominal mass in three, and vomiting in one. Four patients were diagnosed incidentally. Six patients' tumors were located in the pancreatic head, six in the pancreatic body, and four in the pancreatic tail, respectively. The surgical procedures performed included distal pancreatectomy (n=7, 44%), median segmentectomy (n=3), enucleation (n=3), pancreaticoduodenectomy (n=2), and pylorus-preserving pancreaticoduodenectomy (n=1). Three patients underwent laparoscopic surgery. The median tumor size was 6.5cm (1.8~20 cm). Early surgical complications included pancreatic fistula (n=4), bile leakage (n=1), and delayed gastric emptying (n=1). A late complication in one patient was diabetes. The median follow-up period was five years and four months, and all patients survived without recurrence. While pancreatic tumors in adults have a poor prognosis, pancreatic tumors of childhood are usually curative with complete resection and thus have a favorable prognosis.

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Case Report

[English]
Two Cases of Pancreatoblastoma
Young Soo Huh, Jeong Ok hah, Seon Mo Jang, Jung Hoon Lee
J Korean Assoc Pediatr Surg 2000;6(2):139-142.   Published online December 31, 2000
DOI: https://doi.org/10.13029/jkaps.2000.6.2.139

Pancreatoblastoma is uncommon in children and is exceedingly rare in adults. Prognosis is known to be good if the tumor is discovered prior to metastasis. Complete tumor resection is the most important factor for long survival. We report two cases of pancreatoblastoma in a 4 year and 7 year-old females. Palpable abdominal mass was accidentally discovered by their parents. Abdominal CT scan showed huge retroperitoneal mass of unknown origin in one case and of the pancres in the other case. At laparotomy, well encapsulated tumor mass (10×10 cm in diameter) was noted in the body of pancreas in one case, and well encapsulated tumor mass (8×7 cm in diameter) in the tail of pancreas in the other case. We resected the tumor and saved spleen in both ases. Histologic examination revealed that the tumors were pancreatoblastoma. Patients received postoperative chemotherapy with 6 cycles of cisplatinum, adriamycin, ifosfamide and etoposide and are alive in good condition 19 months and 17 months after operation.

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