Department of Gastroenterology and Hepatology, Kyoto University Graduate School of Medicine, 54 Kawahara-cho, Shogoin, Sakyo-ku, Kyoto, 606-8507, Japan.
Department of Gastroenterology, Hyogo Prefectural Amagasaki General Medical Center, 2-17-77, Higashinamba-cho, Amagasaki, Hyogo, 660-8550, Japan.
Clin J Gastroenterol. 2022 Feb;15(1):221-227. doi: 10.1007/s12328-021-01533-0. Epub 2021 Oct 15.
A 70-year-old woman was referred to our hospital for asymptomatic pancreatic tumors. She had a history of hemagiopericytoma (HPC) about 20 years ago, and no apparent recurrence has been observed. Contrast-enhanced computed tomography revealed two hypervascular tumors in the head and uncinate process of the pancreas, and no obvious neoplastic lesions were found in other organs. Endoscopic ultrasound guided fine-needle aspiration cytology was performed and histopathology showed that spindle-shaped tumor cells were arranged in a hemangiopericytoma-like pattern and positive for STAT6, which was a characteristic feature of solitary fibrous tumors (SFTs). Immunohistochemical staining for surgical pathology specimens from past HPC showed positive expression of STAT6, which was Grade 2 central nervous system solitary fibrous tumor/hemagiopericytoma (CNS SFT/HPC) according to the current WHO classification. From these findings, the pancreatic tumors were preoperatively diagnosed as pancreatic metastases of CNS SFT/HPC. She underwent pancreaticoduodenectomy. Histopathological examination of the surgically resected specimen proved that the both pancreatic tumors were SFT/HPC. Thus, pancreatic tumors were finally diagnosed as asynchronous pancreatic metastases from CNS SFT/HPC. Although extremely rare, metastatic pancreatic tumors derived from SFT/HPC should be considered as a differential diagnosis for hypervascular pancreatic tumors, especially when having a past history of brain tumors.
一位 70 岁女性因无症状性胰腺肿瘤被转诊至我院。她约 20 年前患有血管外皮细胞瘤(HPC),目前未见明显复发。增强 CT 显示胰头部和钩突部有两个富血管性肿瘤,其他器官未见明显肿瘤性病变。行超声内镜引导下细针穿刺细胞学检查,组织病理学显示梭形肿瘤细胞呈血管外皮细胞瘤样排列,STAT6 阳性,这是孤立性纤维瘤(SFT)的特征表现。对过去 HPC 的手术病理标本进行免疫组织化学染色显示 STAT6 阳性,根据目前的 WHO 分类,为 2 级中枢神经系统孤立性纤维瘤/血管外皮细胞瘤(CNS SFT/HPC)。根据这些发现,术前诊断胰腺肿瘤为 CNS SFT/HPC 的胰腺转移。她接受了胰十二指肠切除术。手术切除标本的组织病理学检查证实两个胰腺肿瘤均为 SFT/HPC。因此,胰腺肿瘤最终诊断为 CNS SFT/HPC 的异时性胰腺转移。尽管极为罕见,但来源于 SFT/HPC 的转移性胰腺肿瘤应被视为富血管性胰腺肿瘤的鉴别诊断之一,尤其是当有脑肿瘤病史时。