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

Richard S. Whitlock, Kingsley Ebare, Lily S. Cheng, Douglas S. Fishman, Joseph L. Mills, Hai Thuy N. Nguyen, Jed G. Nuchtern, Wenly Ruan, Valeria E. Smith, Kalyani A. Patel, Kevin E. Fisher, Sanjeev A. Vasudevan

Research output: Contribution to journalReview articlepeer-review

1 Scopus citations

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.

Original languageEnglish (US)
Pages (from-to)751-755
Number of pages5
JournalJournal of Pediatric Hematology/Oncology
Volume44
Issue number3
DOIs
StatePublished - Apr 1 2022
Externally publishedYes

Keywords

  • Angiosarcoma
  • KDR
  • Pancreas
  • Pediatric
  • Resection
  • VEGFR2

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Hematology
  • Oncology

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