A Case of Primary Malignant Solitary Fibrous Tumor of the Pancreas

高広, 山梨; 史樹, 鳥海; 雅史, 矢作; 史明, 白鳥; 一晴, 五十嵐; 知彦, 西; 寛, 半田; 和彦, 関根; 清, 向井; 豊, 下山

doi:10.5833/jjgs.45.961

Cited by 6 publications

(6 citation statements)

References 27 publications

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“…Staining is also negative for S-100, which is positive for neurogenic tumors, and negative for c-kit, which is positive for GIST. These features are useful for distinguishing SFT from other mesenchymal tumors [ 6 , 19 ]. Recently, it was revealed that NAB2–STAT6 fusion was the driver mutation in SFT, and the transcriptional repressor of the cell division pathway is converted to the transcriptional activator [ 34 ].…”

Section: Discussionmentioning

confidence: 99%

Malignant solitary fibrous tumor of the pancreas: a case report

et al. 2020

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Background Solitary fibrous tumors (SFTs) are rare tumors, mostly derived from connective tissue mesenchymal cells that arise from the pleura. There are very few reports of primary pancreatic SFT. Preoperative diagnosis is difficult owing to the lack of distinctive radiological findings. We report a case of pancreatic SFT with particularly rare malignant findings. Case presentation A 60-year-old man was referred to the hospital because of a right upper quadrant mass and abnormal liver function test results. Contrast-enhanced computed tomography (CT) showed a well-defined enhanced tumor measuring approximately 8 cm in the pancreatic head. Magnetic resonance imaging (MRI) showed T1WI hypointensity, T2WI hyperintensity, and DWI hyperintensity. The main pancreatic duct and common bile duct were dilated owing to obstruction by the tumor. The following tumor markers were mildly elevated: carcinoembryonic antigen (CEA), carbohydrate antigen 19-9 (CA19-9), SPan-1, and DUPAN-2. The histological diagnosis obtained by endoscopic ultrasound-guided fine needle aspiration (EUS-FNA) was negative for pancreatic ductal carcinoma, malignant lymphoma and neuroendocrine tumor, suggesting the possibility of mesenchymal tumor, but the diagnosis was not confirmed. The patient was judged suitable for surgery and underwent subtotal stomach-preserving pancreatoduodenectomy with D2 lymph node dissection. On histopathological examination of the resected specimen, infiltrating spindle-shaped cells had proliferated, containing numerous mitotic figures, with necrotic findings inside the tumor. Immunostaining was positive for cluster of differentiation-34 (CD34), B cell CLL/lymphoma-2 (Bcl-2), and signal transducer and activator of transcription (STAT6). On the basis of these findings, a diagnosis of malignant pancreatic SFT was made. The patient remains free of recurrent disease after 12 months of follow-up without adjuvant therapy and he is being carefully followed up as an outpatient. Conclusions We experienced a case of malignant pancreatic head SFT. Immunohistochemical staining of the extracted specimens was useful for diagnosis.

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Section: Discussionmentioning

confidence: 99%

Malignant solitary fibrous tumor of the pancreas: a case report

et al. 2020

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“…However, experience with SFT is very limited. Our current understanding of this tumor is mainly based on individual case reports 12–44 . In this study, we analyzed 9 cases.…”

Section: Discussionmentioning

confidence: 99%

“…The patient with intermediate-risk SFT is alive with no evidence of disease at 62 months’ follow-up. However, among previously published primary pancreatic SFTs, there are 7 cases that were reported as a malignant SFT 25,31,34,36,41–43 . These tumors were characterized with marked cellularity (reported in 5 of 7 cases), nuclear pleomorphism and/or atypia (reported in 4 of 7 cases), necrosis (reported in 5 of 7 cases) and frequent (up to 25/10 HPF) mitoses (reported in all 7 cases).…”

Section: Discussionmentioning

confidence: 99%

“…Therefore, the diagnosis appears to be questionable. One patient presented with multiple liver and bone metastases at diagnosis and is alive with disease at 6 months’ follow-up 41 ; another one with intrapancreatic metastases at the time of the diagnosis developed local recurrence after postoperative 21 months is alive at 32 months’ follow-up 25 . The other 4 patients, including one with sarcomatous component in the tumor, are alive with no evidence of disease at 1, 4, 12, and 40 months’ follow-up 31,36,42,43 …”

Section: Discussionmentioning

confidence: 99%

“…Pancreas is an exceedingly rare localization for SFT. The first primary pancreatic SFT case was described in 1999 by Lüttges et al 12 Since then, only single case reports have been described in the literature 12–44 . In this study, we present the first series of primary pancreatic SFTs, with the aim of further defining their clinicopathologic features and challenging differential diagnoses.…”

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confidence: 93%

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Solitary Fibrous Tumor of the Pancreas

Yavas

Tan

Özkan

et al. 2023

American Journal of Surgical Pathology

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Solitary fibrous tumor (SFT) has been increasingly reported in various anatomic sites. However, it is still extremely rare in the pancreas. Herein, we present the first series of primary pancreatic SFTs. Nine cases of primary pancreatic SFTs were analyzed. The mean age was 60 years (36 to 76 y) with no sex predilection. Six tumors were in the head, 3 were in the tail. On imaging studies, tumors were described as a hypervascular mass, 2 revealed cystic areas, and 3 were favored to be neuroendocrine tumors. On biopsy, 2 cases were diagnosed as atypical spindle cell tumor; one was misdiagnosed as suspicious for sarcoma, and another case as metastatic renal cell carcinoma. Two were diagnosed as low-grade sarcoma and low-grade stromal tumor on frozen sections. Grossly, tumors were well-demarcated with a median size of 4 cm (0.9 to 15 cm). Microscopically, they were composed of ovoid to spindle tumor cells with no significant mitotic activity and were arranged in alternating hypercellular and hypocellular areas. Staghorn-like vessels and entrapped pancreatic parenchyma were also detected within all tumors. Tumor cells revealed diffuse/strong nuclear STAT6 expression in 7 of 8, CD34 in 7 of 9, and bcl-2 in 4 of 4 tested cases. One tested tumor harbored NAB2-STAT6 fusion. Eight patients with available follow-up data were free of disease at a mean follow-up of 76 months (3 to 189 mo). SFT should be considered in the differential diagnoses of mesenchymal neoplasms of the pancreas. Immunohistochemical nuclear STAT6 expression is a characteristic feature of SFT. Primary pancreatic SFTs seem to have favorable biological behavior in our series.

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