Background: 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.
Case summary: 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.
Conclusion: 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.
Keywords: Case report; Doege-Potter syndrome; Malignant; Pancreas; Solitary fibrous tumor.
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