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

doi:10.1097/MPH.0000000000002248

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 journal › Review article › peer-review

7 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 language	English (US)
Pages (from-to)	751-755
Number of pages	5
Journal	Journal of Pediatric Hematology/Oncology
Volume	44
Issue number	3
DOIs	https://doi.org/10.1097/MPH.0000000000002248
State	Published - Apr 1 2022
Externally published	Yes

Keywords

Angiosarcoma
KDR
Pancreas
Pediatric
Resection
VEGFR2

ASJC Scopus subject areas

Pediatrics, Perinatology, and Child Health
Hematology
Oncology

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10.1097/MPH.0000000000002248

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Whitlock, R. S., Ebare, K., Cheng, L. S., Fishman, D. S., Mills, J. L., Nguyen, H. T. N., Nuchtern, J. G., Ruan, W., Smith, V. E., Patel, K. A., Fisher, K. E., & Vasudevan, S. A. (2022). Angiosarcoma of the Pancreas in a Pediatric Patient with an Activating KDR-Internal Tandem Duplication: A Case Report and Review of the Literature. Journal of Pediatric Hematology/Oncology, 44(3), 751-755. https://doi.org/10.1097/MPH.0000000000002248

Angiosarcoma of the Pancreas in a Pediatric Patient with an Activating KDR-Internal Tandem Duplication: A Case Report and Review of the Literature. / Whitlock, Richard S.; Ebare, Kingsley; Cheng, Lily S. et al.
In: Journal of Pediatric Hematology/Oncology, Vol. 44, No. 3, 01.04.2022, p. 751-755.

Research output: Contribution to journal › Review article › peer-review

Whitlock, RS, Ebare, K, Cheng, LS, Fishman, DS, Mills, JL, Nguyen, HTN, Nuchtern, JG, Ruan, W, Smith, VE, Patel, KA, Fisher, KE & Vasudevan, SA 2022, 'Angiosarcoma of the Pancreas in a Pediatric Patient with an Activating KDR-Internal Tandem Duplication: A Case Report and Review of the Literature', Journal of Pediatric Hematology/Oncology, vol. 44, no. 3, pp. 751-755. https://doi.org/10.1097/MPH.0000000000002248

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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.",

keywords = "Angiosarcoma, KDR, Pancreas, Pediatric, Resection, VEGFR2",

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AU - Fishman, Douglas S.

AU - Mills, Joseph L.

AU - Nguyen, Hai Thuy N.

AU - Nuchtern, Jed G.

AU - Ruan, Wenly

AU - Smith, Valeria E.

AU - Patel, Kalyani A.

AU - Fisher, Kevin E.

AU - Vasudevan, Sanjeev A.

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AB - 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.

KW - Angiosarcoma

KW - KDR

KW - Pancreas

KW - Pediatric

KW - Resection

KW - VEGFR2

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

Abstract

Keywords

ASJC Scopus subject areas

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