| Objective:To investigate the incidence, clinical pathological features, diagnosis and treatment status and prognosis of gastroenteropancreatic neuroendocrine neoplasm (GEP-NEN) in Yunnan province, establish GEP-NEN open database of yunnan province, improving clinicians’ understanding of the disease and their treatment level, improve the prognosis.Methods:Totally 237 patients with GEP-NENs from 6 provincial general hospitals in Kunming were analyzed retrospectively.We analyzed the clinical pathological features, diagnosis,treatment method and prognosis.Results:1.General conditions:The incidence of GEP-NEN is increasing in Yunnan province year by year, the incidence of 2014 was 6.28 times that of 2005. the male-to-female ratio was 1.3:1, onset age was 14～90, the average onset age was 51.38±14.8, the median age was 52, high-risk age group was 50 to 59. Tumor position differs as onset age, appendix NEN onset age is the youngest(the median age was 25.5), esophagus, rectal NEN onset age is the oldest(the median age was 64). Average onset age of functional GEP-NEN (45.41±13.18) is younger than that of non-functional GEP-NEN (52.31±14.82) Among all the GEP-NEN, functional GEP-NEN accounted for 13.50%, non-functional GEP-NEN accounted for 86.49%.2.Clinical manifestation:The main Clinical manifestations of GEP-NEN were: abdominal pain (45.57%), gastrointestinal bleeding (22.36%), defecate habit change (18.14%), weight loss (12.24%), etc.As for functional GEP-NEN patients,their main Clinical manifestations were Wipple views (87.09%), recurrent spontaneous hypoglycemia (93.55%) and neuropsychological symptoms.Most patients (78.06%) showed no positive signs.3.Laboratory examination:The sensitivity of commonly used tumor markers AFP, CEA、CA125、CA199、CA242、ferritin is 2.38%、10.78%、13.11、11.03%. 7.43%、36.27% for diagnosis. The blood glucose<2.8mmol/L in all of the insulin tumor patients when attacking, all of their fasting insulin/fasting glucose>0.3, 61.29% of the insulin tumor patients’fasting insulin>25mU/L.4. Imaging examination:The sensitivity of B ultrasound、CT MRI、digestive tract radiography is 64.79%、85.61%、88.4%、92.86%. The sensitivity of gastroscopy and enteroscopy、EUS、PET-CT are 100%.5.1 Tumor position:Among the tumor positions of 237 patients with GEP-NEN,7 were esophagus (2.95%),19 were Stomach (8.02%),20 were duodenum (8.43%), 1 was jejunum (1.27%),1 wasileum (0.42%),15 were appendix (6.33%),9 were colon(3.79%),95 were rectum(40.08%),1 was anal (0.42%),2 were rectal(0.84%), 55 were pancreas (23.21%),6 were liver (2.53%),3 were retroperitoneal (1.27%) and 1 was mesenteric (0.42%)5.2 Tumor size:30 of 237 patients with GEP-NEN were not quite clear, the tumor diameters of the rest 207 patients were 0.1～15cm,2.65±2.68cm on average, the tumor diameters of 124 of the rest 207 patients (59.9%)≤2cm,83 of their diameters (40.09%)>2cm.5.3 Tumor metastasis:Among the tumor metastasis of 237 patients with GEP-NEN, 174(73.42%) were non-metastasis,11(4.64%) were local metastasis,35 were regional lymph node invaded,24(10.13%) were distant metastasis,2 were (0.84%) blood vessel invaded.5.4 Pathological stage:Among the tumor metastasis of 237 patients with GEP-NEN, the pathological stage of 134 were not quite clear,48 (20.25%) were on stage G1,35 (14.77%) were on stage G2,20 (8.44%) were on stage G3.5.5 Disease stage:Among the disease stage of 237 patients with GEP-NEN,8 were not quite clear,145 (61.18%) were on stage I,25 (10.55%) were on stage II,36 were on stage III,23 (9.7%) were on stage IV.5.6 Immunohistochemistry:The positive rate of Syn, CgA, NSE was 95.36%, 78.44%,91.45%, the positive rate of other neuroendocrine markers:CD34, CD56, CK7、CK20、CDX-2 was 62.5%、84.62%、62.96%、26.67%、33.33%。the positive rate of Commonly used hormone insulin、gastrin、glucagon was 33.33%、40%、50%.5.7 The way of tissue-obtaining:Among 237 patients with GEP-NEN.74 were resected under endoscope,15 were biopsied under endoscope.6. Misdiagnosis:Among 237 patients with GEP-NEN,23 were misdiagnosed, misdiagnosis rate was 9.7%, misdiagnosis rate of functional GEP-NEN was 25%(8/32), misdiagnosis rate of non-functional was7.32%(15/205)7.treatmentAmong 237 patients with GEP-NEN,138 (58.23%) conduct operation,85 (35.86%) conducted endoscopic resection,14 (5.9%) did not conduct operation or endoscopic resection.29 (12.24%) took Chemotherapy, chemotherapeutic agents were 5-Fu, platinum drugs, calcium folinate, itabines.8.follow-upAmong 237 patients with GEP-NEN,169 have been followed,the duration of follow-up was 2 days to 35 months, survival rate of 1、2、3、4、5、6、7、8、9、10 year was 78.69%、65.09%、49.70%、37.28%、26.63%、14.20%、9.47%、4.14%、1.18%、 0.59%.9. prognostic analysisThe single factor analysis show:age, tumor location, with or without metastasis, tumor size, pathologic stage, disease stage, treatment methods affected prognosis, multiple factor analysis show:age, tumor location, with or without metastasis, tumor size, pathologic stage, disease stage were independent risk factors of prognosis.Conclusion:The incidence of GEP-NEN is increasing in Yunnan province year by year. This disease can occur at any age, mainly occured in the middle aged and elderly people, tumor position varies with onset age, onset age of functional GEP-NEN is younger than that of non-functional GEP-NEN. Among the GEP-NEN, majority were non-functional GEP-NEN, minority were functional GEP-NEN. The main clinical manifestation of non-functional GEP-NEN were nonspecific symptoms of digestive tract or tumor local occupying effect. The main clinical manifestation of functional GEP-NEN were clinical symptoms casused by tumor excessive secretion hormones or vascular active peptide. Commonly used tumor markers AFP、CEA、CA125、CA199、 CA242、ferritin have slight significance for diagnosis. NSE-the specific marker of NEN also has a low Sensitivity. Different imaging examinations were chosed according different locations of the GEP-NEN, endoscopy was preferred imaging examination for gastrointestinal tract,B ultrasound, CT or MRI were preferred imaging examinations for pancreas and liver NEN. Among GEP-NEN, rectum, pancreas NEN were common.Most of their diameter was less than 2cm.The main therapy was operation, endoscopical therapy was the secondary therapy, there were a variety of chemotherapy regimens, Molecular targeted therapy limited, biological therapy was not conducted. the single factor analysis show:age, tumor location, with or without metastasis, tumor size, pathologic stage, disease stage, treatment methods affected prognosis. multiple factor analysis show:age, tumor location, with or without metastasis, tumor size, pathologic stage, disease stage were independent risk factors of prognosis. |
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