Pancreaticoduodenectomy for pancreatoblastoma: a case report and literature review

Abstract

Pancreatoblastoma (PB) is extremely rare. A 11-year-old boy, who had excision of dilated common bile duct with hepaticoduodenostomy when 9 years old was referred to our institute for further management of abdominal pain and steatorrhea. Imaging studies showed a solid 4 cm tumor in the head of the pancreas and two lesions in the liver. Needle biopsies diagnosed PB with liver metastases. After five courses of chemotherapy, the primary tumor was completely resected with pancreaticoduodenectomy (PD) and Child's pancreaticobiliary tract reconstruction. The liver metastases were resected. Postoperative recovery was uneventful. Here, we present this case and review the literature focusing on management of PB.

Pancreaticoduodenectomy for pediatric and adolescent pancreatic malignancy: A single-center retrospective analysis

“…Pancreaticoduodenectomy is an exceedingly rare procedure performed in the pediatric population[3, 8, 14]. We present our series of 12 patients who underwent PD at our institution over the last 21 years.…”

Systemic Chemotherapy for Advanced Rare Pancreatic Histotype Tumors

“…Pancreatoblastoma typically mainly occurs in the pediatric population, with approximately only 40 cases described in adults. 96,97 Prognosis in this small group of patients is dismal, with an mOS of 5 months in the advanced stages. 98 In children, PLADO regimen represents the criterion standard in the firstline setting.…”

Surgical Treatment of Pancreatic Tumors in Childhood and Adolescence: Uncommon Neoplasms with Favorable Outcome

“…This neoplasm is most typically found in females (80–90% of cases in the literature, 83% in our experience) and behaves as a low-grade tumor [7,9]. Distant metastases are uncommon, and the long-term prognosis is favorable, even in the setting of a metastatic disease [10,11]. Surgical resection with curative intent must therefore be performed, when possible, in all cases.…”