Key result
A 17-year-old female was diagnosed with the first reported pediatric case of pancreatic angiosarcoma, featuring a novel activating KDR-internal tandem duplication.
Why the study?
Pancreatic angiosarcoma is an exceedingly rare malignancy with very few reported cases and none previously described in children.
Case Report (n=1)
This is the first reported case of pancreatic angiosarcoma in a pediatric patient and the first with an activating KDR-internal tandem duplication.
Hypothesis-generating for KDR-targeted therapy in pediatric angiosarcoma; leaves open driver role and treatment response pending validation.
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.
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Whitlock et al. (2022) conducted a case report in Pancreatic angiosarcoma (n=1). KDR-internal tandem duplication was evaluated. A 17-year-old female was diagnosed with the first reported pediatric case of pancreatic angiosarcoma, featuring a novel activating KDR-internal tandem duplication.
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