Atypical/malignant solitary fibrous tumor of the pancreas with spleen vein invasion

Abstract

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.

Pancreatic hamartoma: detection of harbouring NAB2::STAT6 fusion gene

“…Although most tumour lesions in the pancreas are epithelial tumours, non‐epithelial, mesenchymal tumours develop in rare cases and there are reports of solitary fibrous tumours (SFTs) 19–34 . An SFT is a fibroblastic tumour characterised by the prominent, branching, thin‐walled, dilated (staghorn) vasculature and NAB2::STAT6 gene rearrangement 35,36 .…”

“…1 Although most tumour lesions in the pancreas are epithelial tumours, non-epithelial, mesenchymal tumours develop in rare cases and there are reports of solitary fibrous tumours (SFTs). [19][20][21][22][23][24][25][26][27][28][29][30][31][32][33][34] An SFT is a fibroblastic tumour characterised by the prominent, branching, thin-walled, dilated (staghorn) vasculature and NAB2::STAT6 gene rearrangement. 35,36 Although SFTs are known to develop in the pleura and soft tissues, SFTs with a morphology resembling pancreatic hamartomas have been reported.…”

A rare sporadic pancreatic desmoid fibromatosis with splenic vein invasion diagnosed by CT scan-guided core needle biopsy: a case report with possible differential diagnosis from metastatic colorectal or renal cancer

“…DT involving the pancreas is very rare with only few cases previously reported in the English-language literature [ 6 ], and the splenic vein invasion is an uncommon finding [ 7 ].…”

Two case reports of rare diseases occurring in rare parts: Splenic vein solitary fibrous tumor and Liver solitary fibrous tumor

“…Most SFTs are benign lesions, and the prognosis is good after surgical resection. However, there are still malignant lesions, and the surrounding tissue in ltration, local recurrence, and distant metastasis can be seen (5).…”

“…Complete surgical resection is still the basis of its treatment. The reports about SFT were mainly concentrated in the pleura, a few reports suggested that it could also appear in any location, including the skin(6), meninges, and thyroid (7), round ligament of the liver(8), pancreas (5), and the liver SFT was rst reported in 1959 (9). But there have been no reports of splenic veins SFT, so far, and also the no report of liver recurrence SFT cured by orthotopic liver transplantation (OLT).…”