OncoTargets and Therapy (Mar 2017)
Pancreatic solitary fibrous tumor in a toddler managed by pancreaticoduodenectomy: a case report and review of the literature
Abstract
Qingfeng Sheng,1 Weijue Xu,1 Jiangbin Liu,1 Baiyong Shen,2 Xiaxing Deng,2 Yibo Wu,1 Wei Wu,1 Shenghua Yu,1 Xueli Wang,3 Zhibao Lv1 1Department of General Surgery, Shanghai Children’s Hospital, 2Department of General Surgery, Rui Jin Hospital, 3Department of Pathology, Shanghai Children’s Hospital, Shanghai Jiao Tong University, Shanghai, People’s Republic of China Abstract: Solitary fibrous tumor (SFT) of the pancreas is rare, with 15 adult cases reported in the English literature. We described a 14-month-old boy who presented with obstructive jaundice. Dominantly elevated serum CA19-9 was detected. Imaging studies revealed a well-circumscribed, solid mass in the pancreatic head. A pancreaticoduodenectomy (child procedure) was performed using Shen’s anastomosis technique. After resection of the tumor, liver function and serum tumor markers normalized and clinical signs receded. The boy was disease free after a follow-up of 12 months. Histological examination showed the tumor consisted of “patternless pattern” arranged spindle tumor cells and keloid-like hyalinized collagen. Immunohistochemical staining was positive for CD34 and vimentin. Mutation analysis of CTNNB1 was negative. To the best of our knowledge, our patient was the first case of pancreatic SFT in a pediatric population. SFT should be considered in differential diagnosis when confronted with a pancreatic tumor in children. Complete resection should be meticulously pursued. Keywords: pancreas, rare tumors, children, rare pediatric tumors
