Michael E. DeBakey Department of Surgery, Division of Pediatric Surgery, Texas Children's Surgical Oncology Program.
Texas Children's Department of Surgery, Dan L. Duncan Cancer Center, Baylor College of Medicine.
J Pediatr Hematol Oncol. 2022 Apr 1;44(3):e751-e755. doi: 10.1097/MPH.0000000000002248.
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.
胰腺血管肉瘤是一种极其罕见的恶性肿瘤，占胰腺肿瘤的<1%。文献中仅报道了极少数胰腺血管肉瘤，且没有儿童病例描述。我们报告了一例 17 岁女性的病例，该患者因胰腺肿块行胰十二指肠切除术，最初被误诊为胰腺实性假乳头状瘤，术后诊断为胰腺血管肉瘤。该血管肉瘤在 KDR 基因（KDR 内部串联重复）中发现了一种新的激活内部串联重复。我们讨论了该疾病过程的现有文献。这是首例报道的儿科患者胰腺血管肉瘤病例，也是首例具有激活 KDR 内部串联重复的病例。
