Introduction: Solitary fibrous tumor (SFT) is an uncommon mesenchymal tumor that is most common in the pleura. However, according to previous studies, the SFT of the pancreas is extremely rare; only 20 cases have been reported so far. Here, we conduct a literature review and report the first case of atypical/malignant SFT of the pancreas with spleen vein invasion. Patient concerns: The patient is a 61-year-old Chinese male who presented with 1 week of upper abdominal pain. Abdominal magnetic resonance imaging showed a huge mass (>10 cm) at the distal end of the pancreas, and the mass obstructing the splenic vein. Diagnosis: Atypical/malignant SFT of the pancreas with splenic vein tumor thrombus. Interventions: The patient underwent laparoscopic distal pancreatectomy with splenectomy procedure to achieve a radical resection, and did not undergo chemotherapy or radiotherapy. Outcomes: Abdominal computed tomography scans were performed at 1 and 4 months after resection, and no signs of recurrence or metastasis were found (Fig. 1 JOURNAL/medi/04.03/00005792-202004240-00032/figure1/v/2023-09-15T184447Z/r/image-tiff . B). Conclusion: The clinical symptoms of atypical/malignant SFT of the pancreas with spleen vein invasion are not atypical, and imaging feature is lack of specificity. Preoperative diagnosis is difficult, and there is a potential for malignancy. However, due to the paucity of randomized control trials, there is no established, globally accepted treatment strategy, radiation therapy and chemotherapy regimens have not demonstrated global effectiveness, and no standardized treatments have been identified. Therefore, we recommend complete surgical resection and close clinical follow-up.
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