Pancreatic angiosarcoma is an exceedingly rare malignancy accounting for <1% of pancreatic neoplasms. A very limited number of pancreatic angiosarcomas have been reported in the literature without any cases described in children. We present the case of a 17-year-old female diagnosed with angiosarcoma of the pancreas following pancreaticoduodenectomy for a pancreatic mass, initially presumed to be a solid pseudopapillary neoplasm of the pancreas. The angiosarcoma was found to have a novel activating internal tandem duplication in the KDR gene (KDR-internal tandem duplication). We discuss the current literature on this disease process. This is the first reported case of pancreatic angiosarcoma in a pediatric patient and the first with an activating KDR-internal tandem duplication.

Download full-text PDF

Source
http://dx.doi.org/10.1097/MPH.0000000000002248DOI Listing

Publication Analysis

Top Keywords

angiosarcoma pancreas
4
pancreas pediatric
4
pediatric patient
4
patient activating
4
activating kdr-internal
4
kdr-internal tandem
4
tandem duplication
4
duplication case
4
case report
4
report review
4

Similar Publications

Want AI Summaries of new PubMed Abstracts delivered to your In-box?

Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!