title: Neuroendocrine Neoplasms of the Major Duodenal Papilla With Focus on Histopathological Features and Prognosis.
abstract: Neuroendocrine neoplasms (NENs) are generally indolent and progress slowly. However, NENs of major duodenal papilla are uncommon. We retrospectively assessed relevant clinicopathological findings in 9 consecutive patients treated for major duodenal papilla NENs by pancreaticoduodenectomy in our hospital from 2009 to 2013. Eight of the 9 patients (89%) presented with painless obstructive jaundice and one with intermittent fever, attributable to pancreatitis, without jaundice. The diagnostic accuracy was 75% (6/8) for biopsies obtained under duodenoscope guidance. Enhanced multidetector computed tomography detected 89% (8/9) of tumors. Patients with uncertain preoperative diagnoses all underwent intraoperative frozen section pathological diagnosis. Tumor cells extended to at least the muscularis propria in all patients. There were 5 neuroendocrine tumors, 2 neuroendocrine carcinomas, and 2 mixed adenoneuroendocrine carcinomas. Two, 4, and 3 cases were grades 1, 2, and 3, respectively. Grade 3 tumor patients had poor prognoses with tumor recurrence or metastasis within 2 months and all died within 1 year. The overall survival rate (1 year) of grade 3 was lower than in grades 1 and 2 (P < .05). Patients with grade 1 tumors had a similar prognosis to grade 2 (P > .05). To date, only 4 cases of this tumor have been reported in the Chinese literature. The prognosis can be predicted accurately by histopathological features accordingly to the World Health Organization 2010 classification. Multiple imaging techniques and pathological examination should be carried out appropriately to diagnose the disease early.
authors.affiliation: Third Central Hospital of Tianjin Medical University, Tianjin, China.
authors.forename: Qin
authors.initials: Q
authors.lastname: Zhang
authors.affiliation: Third Central Hospital of Tianjin Medical University, Tianjin, China.
authors.forename: Cheng-Jun
authors.initials: CJ
authors.lastname: Lu
authors.affiliation: Third Central Hospital of Tianjin Medical University, Tianjin, China.
authors.forename: Xiang
authors.initials: X
authors.lastname: Zhang
authors.affiliation: Third Central Hospital of Tianjin Medical University, Tianjin, China.
authors.forename: Zhe
authors.initials: Z
authors.lastname: Ma
authors.affiliation: Third Central Hospital of Tianjin Medical University, Tianjin, China.
authors.forename: Qi
authors.initials: Q
authors.lastname: Xin
authors.affiliation: Third Central Hospital of Tianjin Medical University, Tianjin, China chuanshanzh@126.com.
authors.forename: Chuan-Shan
authors.initials: CS
authors.lastname: Zhang
mesh_terms: D000328:Adult; D000368:Aged; D014670:Ampulla of Vater / Q000473:pathology*; D003138:Common Bile Duct Neoplasms / Q000401:mortality / Q000473:pathology* / Q000601:surgery; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D008875:Middle Aged; D060787:Neoplasm Grading; D009362:Neoplasm Metastasis; D009364:Neoplasm Recurrence, Local; D018358:Neuroendocrine Tumors / Q000401:mortality / Q000473:pathology* / Q000601:surgery; D016577:Pancreaticoduodenectomy; D011379:Prognosis; D012189:Retrospective Studies; D016019:Survival Analysis
keywords: histopathological features; major duodenal papilla; neuroendocrine neoplasm; prognosis