Geng Hao, Ye Yu, Jin Yun, Li Bai-Zhou, Yu Yuan-Quan, Feng Yang-Yang, Li Jiang-Tao
Department of General Surgery, The Second Affiliated Hospital, College of Medicine, Zhejiang University, Hangzhou 310009, Zhejiang Province, China.
Department of Pathology, The Second Affiliated Hospital, College of Medicine, Zhejiang University, Hangzhou 310009, Zhejiang Province, China.
World J Clin Cases. 2020 Jan 26;8(2):343-352. doi: 10.12998/wjcc.v8.i2.343.
Pancreatic solitary fibrous tumor (SFT) is a rare neoplasm of intermediate biological potential. So far, only 22 cases have been reported since 1999. All the cases, except one, exhibited benign features. Here, we report the first case of malignant pancreatic SFT with typical Doege-Potter syndrome, along with the clinical and pathologic evidence of its systemic metastasis.
The patient was a 48-year-old man with a 1-year history of pancreatic and liver masses and refractory hypoglycemia. Increased uptake of the tracer fluorodeoxyglucose (FDG) was found in the liver and bones by fluorine-18 FDG positron emission tomography/computed tomography. After multidisciplinary discussion, a distal pancreatectomy procedure was performed, and histological examination showed a lesion composed of abundant heterogeneous spindle cells with localized necrosis. On immunohistochemistry evaluation, STAT6 was found to be diffusely expressed in the tumor. Based on the overall evidence, the patient was diagnosed with malignant pancreatic SFT with liver and bone metastases.
The diagnosis of malignant SFT requires comprehensive evidence including clinical, immunohistochemistry, and histological features. This case may be presented as a reference for diagnoses and management of malignant pancreatic SFTs with systemic metastasis.
胰腺孤立性纤维瘤（SFT）是一种具有中等生物学潜能的罕见肿瘤。自1999年以来，迄今仅报道了22例。除1例之外，所有病例均表现为良性特征。在此，我们报告首例伴有典型杜格-波特综合征的恶性胰腺SFT病例，并提供其发生全身转移的临床及病理证据。
该患者为一名48岁男性，有胰腺和肝脏肿块病史1年，伴有难治性低血糖。氟-18氟代脱氧葡萄糖（FDG）正电子发射断层扫描/计算机断层扫描显示肝脏和骨骼中示踪剂FDG摄取增加。经过多学科讨论后，实施了胰体尾切除术，组织学检查显示病变由大量异质性梭形细胞组成，并伴有局部坏死。免疫组化评估发现，肿瘤中STAT6呈弥漫性表达。基于所有证据，该患者被诊断为伴有肝转移和骨转移的恶性胰腺SFT。
恶性SFT的诊断需要包括临床、免疫组化和组织学特征在内的综合证据。该病例可为伴有全身转移的恶性胰腺SFT的诊断和治疗提供参考。
