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

10 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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