Primary mesenchymal tumors of the pancreas in a single center over 15 years

Abstract

Abstract. In total, 95% of primary tumors in the pancreas are epithelial tumors; primary mesenchymal tumors at this site are extremely rare. At present, only one comprehensive study about these rare tumors has been performed. Another retrospective analysis of these rare tumors is performed in the present study, which, to the best of our knowledge, is the first to be performed in China. In the present study, 10 patients that underwent resection for primary mesenchymal tumors of the pancreas were identified in a 15-year period at the Chinese Academy of Medical Sciences and Peking Union Medical College, which accounted for 0.51% of the total surgically resected primary tumors of the pancreas at this hospital. Among the 10 patients, 7 patients (70%) were diagnosed with benign/borderline tumors, and the remaining 3 patients (30%) were diagnosed with malignant tumors. It was a unique finding of the present study that the preoperative diagnosis was frequently a misdiagnosis, in terms of the specific pathological diagnosis. Therefore, although primary mesenchymal tumors of the pancreas are extremely rare, they should be considered in order to make the correct preoperative diagnosis. Contrarily to a previous study, in the present study, the most common benign tumor was not desmoid tumor, but solitary fibrous tumors; the most frequent primary sarcoma was not undifferentiated/unclassified sarcoma either. In conclusion, the present study aids the understanding of these rare tumors; however, primary mesenchymal tumors of the pancreas require additional exploration in the future. IntroductionThe predominant primary tumor that occurs in the pancreas is ductal adenocarcinoma (1). Stroma is scant in the normal pancreas, and therefore primary mesenchymal tumors of the pancreas are extremely rare (2).Although numerous primary mesenchymal tumors of the pancreas have been reported, the majority of cases were reported as case reports (3-22). To the best of our knowledge, there has been only one comprehensive study of these tumors in a single institution (3). A lack of comprehensive studies from other large institutes to provide a comprehensive understanding of this type of tumor remains. In the present study, a thorough retrospective analysis of primary mesenchymal tumors of the pancreas in Peking Union Medical College Hospital (Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China) was performed and the relevant studies published in the English literature were reviewed.According to the published studies, at present there have been 221 cases of primary mesenchymal tumors of the pancreas reported in the English literature (3). Among these, the most commonly reported primary benign/borderline mesenchymal tumors of the pancreas were schwannomas, followed by inflammatory myofibroblastic tumors, solid and cystic hamartomas, and solitary fibrous tumors. In addition, the most frequently reported primary malignant mesenchymal tumors of the pancreas were leiomyosarcomas, followed by Ewing sarcomas/primitive neur...

Desmoid fibromatosis of the pancreas––A case report with radiologic-pathologic correlation

“…and Kim et al. it was found that desmoid tumor was the most common benign mesenchymal pancreatic tumor, followed by solitary fibrous tumor [3] . The mean age of presentation is 36 years old and there is slight female to male predilection for abdominal DTs [4] .…”

Invasive giant pancreatic desmoid-type fibromatosis with curative resection: A case report

“…The pancreatic origin DT remains an extremely rare subset of DTs. The English literature from the 1980s report 27 pancreatic DTs, and therefore our case is case number 28th [1] , [2] , [4] , [5] , [11] , [14] , [15] , [16] , [17] , [18] , [19] .…”

Primary Pancreatic Leiomyosarcoma: A Rare Malignant Transformation of Primary Pancreatic Head Leiomyoma

“…Presenting symptoms of primary pancreatic leiomyosarcomas may include abdominal pain, abdominal mass, weight loss, and jaundice, although the diagnosis is usually incidental [ 1 , 5 , 6 ]. Pancreatic leiomyosarcomas may be located in the pancreatic head, body, or tail [ 1 , 5 ]. These tumors are usually large in size at presentation [ 6 ].…”

“…Primary mesenchymal tumors of the pancreas are rare, with only 221 cases reported to date [ 1 ]. Primary pancreatic leiomyosarcomas are an even rarer subtype, with only 69 reported cases to date [ 2 ].…”