Angiosarcoma of the Pancreas in a Pediatric Patient With an Activating KDR-Internal Tandem Duplication: A Case Report and Review of the Literature. Issue 3 (30th April 2022)

• Record Type: Journal Article
• Authors: Whitlock, Richard S.; Ebare, Kingsley; Cheng, Lily S.; Fishman, Douglas S.; Mills, Joseph L.; Nguyen, HaiThuy N.; Nuchtern, Jed G.; Ruan, Wenly; Smith, Valeria E.; Patel, Kalyani A.; Fisher, Kevin E.; Vasudevan, Sanjeev A.
• Is Part Of: Journal of pediatric hematology/oncology. Volume 44:Issue 3(2022)
• Page Start: e751
• Page End: e755
• Publication Date: 2022-04-30
• Subjects: pediatric -- pancreas -- angiosarcoma -- resection -- VEGFR2 -- KDR; Pediatric hematology -- Periodicals; Tumors in children -- Periodicals; 618.9215
• DOI: 10.1097/MPH.0000000000002248
• Languages: English
• ISSNs: 1077-4114

Abstract

Abstract : 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.