Angiosarcoma of the Pancreas in a Pediatric Patient With an Activating KDR-Internal Tandem Duplication: A Case Report and Review of the Literature

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 KDRinternal tandem duplication.

Comments and illustrations of the European Federation of Societies for Ultrasound in Medicine guidelines: Rare pancreatic tumors, ultrasound and contrast-enhanced ultrasound features—Malignant mesenchymal tumors

“…By 2022, a total of 10 cases had been published in the English-language literature. [ 67 – 71 ] Nguyen et al [ 70 ] described the simultaneous diagnosis of ductal adenocarcinoma with AS of the pancreas in surgical histology. Angiosarcoma can be localized in all areas of the pancreas.…”