| In recent years,the incidence of pancreatic neuroendocrine tumors(pNETs)has increased significantly.The development of diagnostic techniques and the increasing awareness of such rare diseases may be the cause of the increase in incidence.Functional pNETs often present typical clinical manifestations caused by excessive hormone secretion.On the other hand,majority of non-functional pNETs present with distant metastases at initial time because of delayed diagnosis due to atypical symptoms such as abdominal pain,bloating and abdominal masses.In addition,well differentiated pNETs have heterogeneous and indolent nature,while poorly differentiated tumors often show aggressive behavior.Previous studies have found that a variety of factors such as histopathology,primary site,staging and grading of the tumor might predict the biological behavior of the tumor and the prognosis of the patient.However,because pNETs are rare,many studied were limited by their poor data in clinical pathological features and prognostic survival,which caused different results.ObjectiveThis study aimed to summarize the clinical experiences in pNETs diagnosis and treatment.We tried to find predictive factors which affected the prognosis of patients by analyzing the clinicopathological characteristics,grading and staging of tumors.Furthermore,our study might provide robust evidence for further research and clinical management.Methods157 patients with pNETs who had positive diagnosis of histology were identified from the First Affiliated Hospital of Zhengzhou University between 2011 and 2018.1756 patients were extracted from the Surveillance,Epidemiology,and End Results(SEER)database from 2004 to 2015.Firstly,we summarized the experience of diagnosis and treatment in pNETs according to the results of laboratory,imaging,pathological examination and treatment methods which were obtained from the First Affiliated Hospital of Zhengzhou University.After that,Kaplan-Meier method was performed to analyze overall survival(OS)depending on survival data which derived from two databases.Multivariate analyses were carried out using Cox proportional hazards model to identify independent prognostic risk factors.ResultsIn total,1913 patients with pNETs were selected,including 1010 males and 903females.Insulinoma(N=157)was the most common F-pNETs.Most of patients diagnosed as insulinoma presenting with Whipple triad.On the other hand,NF-pNETs frequently presented with non-specific symptoms such as abdominal pain and bloating.In our study,more than 30%of patients were accidentally diagnosed after physical examination.CT and MRI had a high detection rates in the diagnosis of pNETs,with 92.9%and 95.8%,respectively.However,abdominal ultrasound had a limited role in diagnosis.Immunohistochemistry results showed that the positive rates of CgA,Syn,CD56 and NSE were 65.9%,99.4%,72.7%and 93.6%,respectively.The OS of 1,3 and 5 years for all patients in this study were 92.1%,83.0%and75.6%,respectively.Univariate analysis demonstrated age,gender,tumor location,tumor function,tumor size,AJCC stage and surgery were associated with prognosis.However,there was a crossover between the Kaplan-Meier curves of patients with stageⅠandⅡ.On multivariate analysis,we found that age,tumor location,AJCC staging,and surgery were independent factors affecting the prognosis of patients.The older patients(≥55),tumor located in head of pancreas,ⅢorⅣstaging and the patients who were not underwent surgery showed a poorer survival.Furthermore,Ⅳstaging patients were categorized into surgery and non-surgery subgroups.The results showed that the survival of the surgery group was significantly greater than that of the non-surgery group(median survival time:69 months vs.24months,χ~2=39.495,P<0.001).In the multivariate analysis,surgery was still an independent protective factor of the prolonged prognosis(HR:0.397,95%CI:0.291-0.541,P<0.001).Conclusions1.pNETs are rare neoplasms with an increasing incidence in recent years.F-pNETs excrete active hormones,which cause specific endocrine symptoms,whereas most pNETs are NF-pNET.Insulinoma is the most common functional neuroendocrine tumor.2.The specific hormones secreted by functional neuroendocrine tumors can be used to assist diagnosis,while the diagnostic value of tumor markers for non-functional tumors was limited.The combination of CgA and Syn can improve the accuracy of diagnosis.CT/MRI imaging are reliable methods.3.pNETs are a group of clinically rare and heterogeneous diseases of the pancreas.Older patients(≥55),tumors located in head of pancreas,higher AJCC staging and without surgical resection were independent predictors of worse overall survival in patients with pNETs.Surgical resection was the preferred method for curing pancreatic neuroendocrine tumors,which could prolong survival of patients.Furthermore,even patients who diagnosed asⅣstaging could benefit from palliative surgery.4.AJCC staging was more accurate in predicting the prognosis of patients withⅢandⅣstaging,but the prognostic value in predicting patients withⅠandⅡstaging was limited. |
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