| Objectives:To investigate the histological origin,clinical characteristics,pathological and immunohistochemical features,treatment and prognosis of solid pseudopapillary tumor of the pancreas(SPTs),so as to improve the level of diagnosis and treatment of SPTs.Methods:A retrospective analysis was performed on the clinical data of 97 patients with post-operative pathologic confirmation of SPTs in the databases of four hospitals in JiangXi province during different periods from January 2007 to January 2020,and the data of 3927 patients with SPTs who met the inclusion criteria in the Chinese literature from January 2015 to January 2020.Results:1.A total of 4024 cases were enrolled the current study,including 97 cases in our group and 3927 cases in the literature.Among them,658 were male and 3366 were female.The ratio of male to female was 1:5.1.The average age was 30.9 years(7-83 years),which was 37.4 years in male,and 30.0 years in female.2.A total of 3219 patients were recorded their clinical manifestations,of which1845(57.3%)were symptomatic,and 1374(42.7%)were asymptomatic with the tumors found during routine physical examinations.The most common symptom was abdominal pain and distention(42.0%,1351/3219),following by nausea and vomiting(9.5%,307/3219).3.The laboratory examination results of patients with SPTs were mostly within normal ranges,with a total of 11.5%(244/2113)patients showed elevated CA19-9 of serum tumor marker.Among the 97 patients in our group,the positive rates of Hepatitis B surface antigen(HBsAg)and Hepatitis B core antibody(anti-HBc)were14.4%(14/97)and 49.5%(48/97),respectively.The diagnostic rate of preoperative imaging examination was 54.5%(414/759),and the diagnostic rate of endoscopicultrasound or CT-guided fine needle biopsy was 61.8%(42/68).4.A total of 1763 patients were recorded the type of surgical procedure,and the resection rate was 98.6%.The surgical procedures mainly included 412 distal pancreatectomy with splenectomy,394 splenic-preserving distal pancreatectomy,400 tumor enucleation,347 pancreatoduodenectomy,147 central pancreatectomy,33pyloro-preserving pancreaticoduodenectomy,2 total pancreatectomy,21 combined other organ or metastatic tumor resection and 7 palliative resection.The most common postoperative complication was pancreatic fistula(13.4%,175/1309).5.A total of 3167 patients had tumor growth sites recorded,of which only 11(0.3%)had multiple tumors,and the most common sites of tumor growth were the body and tail of pancreas(55.4%,1762/3178).Tumor size was recorded in 2516 patients with an average maximum diameter of 6.3 cm(0.5-24 cm).Pathologically,the mass is composed of a mixture of solid,cystic and junction region,and the typical tumor cells grow around the fiber vascular axis to form branched papillary structure or pseudophora structure.Immunohistochemistry examination showed that a variety of proteins were positively expressed in varying degrees,including ?-Catenin,CD10,CD56,Vimentin,NSE,CK,a1-AT,PR and so on.6.The biological behavior of SPTs is mostly benign,with the tumor's invasive rate accounting for 13.9%(205/1480).The invasion sites mainly occur in nerves and blood vessels,and organ involved mainly occurs in duodenum and spleen.Distant metastasis mainly occurs in liver,and lymph node metastasis was rare found.7.A total of 1859 patients were followed up for an average of 40.3 months(1-167 months).During the follow-up period,there were 18 cases of tumor recurrence,19 cases of liver metastasis,and 4 cases of metastasis to other sites.The recurrence/metastasis rate was 2.2%(41/1859),and the median recurrence/metastasis time was 35.1 months.Reoperation was performed in 6 patients with recurrence/metastasis.A total of 17 patients died during the follow-up period,of which 13(0.7%,13/1859)died of SPTs.Conclusions:1.SPTs are a rare low-grade malignant tumor,and its pathogenesis is related to the aberrant activation of Wnt/?-catenin signaling pathway.SPTs tumor tissue mightbe originated from pancreatic multipotent stem cells.However,whether SPTs are related to HBV infection or not is still need further research.2.SPTs are most commonly occur in young females.The clinical symptoms of SPTs were nonspecific,mainly presented as abdominal discomfort,and some patients were asymptomatic.The laboratory examination results of patients with SPTs are mostly within normal ranges,and the preoperative diagnosis is mainly based on imaging examination.3.Pseudopapillary structure is the typical pathological feature of SPTs.Immunohistochemical staining of SPTs is characterized by exocrine,endocrine and epithelial phenotypes,which supports the origin of pancreatic multipotent stem cells.4.Surgical resection is the first option for treatment of SPTs,even in the presence of local invasion or metastases.Reoperation could be considered for patients with postoperative recurrence or metastasis.SPTs demonstrate a excellent prognosis after surgical resection,and the vast majority of patients have long-term survival.It is suggested that all patients with SPTs should be closely long-term followed up after operation,because there is still a low recurrence or metastasis rate and disease mortality. |
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