A case report of carcinoma of the papilla of Vater associated with a hyperplasia-dysplasia-carcinoma sequence by pancreaticobiliary maljunction.

BACKGROUND: Pancreaticobiliary maljunction (PBM) is a known risk factor for biliary tract cancer. However, its association with carcinoma of the papilla of Vater (PVca) remains unknown. We report a case with PVca that was thought to be caused by the hyperplasia-dysplasia-carcinoma sequence, which is considered a mechanism underlying PBM-induced biliary tract cancer. CASE PRESENTATION: A 70-year-old woman presented with white stool and had a history of cholecystectomy for the diagnosis of a non-dilated biliary tract with PBM. Esophagogastroduodenoscopy revealed a tumor in the papilla of Vater, and PVca was histologically proven by biopsy. We finally diagnosed her with PVca concurrent with non-biliary dilated PBM (cT1aN0M0, cStage IA, according to the Union for International Cancer Control, 8th edition), and subsequently performed subtotal stomach-preserving pancreaticoduodenectomy. Pathological findings of the resected specimen revealed no adenomas and dysplastic and hyperplastic mucosae in the common channel slightly upstream of the main tumor, suggesting a PBM related carcinogenic pathway with hyperplasia-dysplasia-carcinoma sequence. Immunostaining revealed positivity for CEA. CK7 positivity, CK20 negativity, and MUC2 negativity indicated that this PVca was of the pancreatobiliary type. Genetic mutations were exclusively detected in tumors and not in normal tissues, and bile ducts from formalin-fixed paraffin-embedded samples included mutated-ERBB2 (Mutant allele frequency, 81.95%). Moreover, of the cell-free deoxyribonucleic acid (cfDNA) extracted from liquid biopsy mutated-ERBB2 was considered the circulating-tumor deoxyribonucleic acid (ctDNA) of this tumor. CONCLUSIONS: Herein, we report the first case of PVca with PBM potentially caused by a "hyperplasia-dysplasia-carcinoma sequence" detected using immunostaining and next-generation sequencing. Careful follow-up is required if pancreaticobiliary reflux persists, considering the possible development of PVca.

Current status of and future issues related to endoscopic papillectomy.

Endoscopic papillectomy is widely performed to treat duodenal papillary tumors, particularly at high-volume centers. It is indicated for adenomas without intraductal extension of the bile or pancreatic ducts. However, despite numerous reports of carcinomas that expand the indications to include well-differentiated adenocarcinomas that do not invade the sphincter of Oddi, the low agreement between biopsy and final pathological diagnosis, as well as the current inability of imaging modalities to diagnose sphincter of Oddi invasion, makes it difficult to consider expanding indications. Although complications can be prevented by certain methods, such as pancreatic duct stenting, and the frequency of severe complications has decreased, the safety of the procedure remains unconfirmed. In the future, this technology is expected to progress and enable wider applications, including those in tumors with extensive horizontal spread and those with intraductal extension of the bile and pancreatic ducts. Such technology may also improve the safety and accuracy of diagnosis.

Papilla of Vater morphology as an influencing factor in successful cannulation during resident training in advanced endoscopy. A prospective clinical study.

INTRODUCTION AND AIM: Endoscopic retrograde cholangiopancreatography (ERCP) is a complex procedure. Clinical guidelines assess competence in ERCP through a defined number of procedures, but multiple factors are involved. Our aim was to analyze the morphology of the papilla of Vater as an independent factor in selective common bile duct cannulation during resident training. MATERIAL AND METHODS: Patients that underwent ERCP were studied consecutively. All ERCPs were begun by a resident in training. The type of papilla was classified according to Haraldsson, including those with previous sphincterotomy. Cannulation difficulty and success and their relation to the type of papilla were documented. The analysis was divided into three 4-month periods. RESULTS: Of the 429 patients, cannulation was difficult in 101 (23.5%). The residents achieved selective cannulation of the common bile duct in 276 (64.3%) and the cannulation success rate at the end of their training was 81.7%. Cannulation was performed with the least difficulty in papillae with previous sphincterotomy (2.8%), unlike the type 4 papilla, which was difficult to cannulate in 50% of the cases. The lowest overall cannulation success was in the type 2 papilla (81.8%). CONCLUSION: Papilla type can influence cannulation success, but it is not the only related factor. Patients that underwent previous sphincterotomy appear to be the cases in whom ERCP training can be started.

Correlation of ectopic distal location of papilla of Vater and clinical characteristics in pediatric choledochal cysts.

PURPOSE: Ectopic distal location of papilla of Vater (EDLPV) is an obvious pathological feature of choledochal cyst (CDC). This study aimed to investigate the correlation between EDLPV and clinical characteristics of CDCs. METHODS: Three groups were studied: Group 1 (G1), papilla in the middle third of second part of duodenum (n = 38); Group 2 (G2), papilla from the distal third of second part to the beginning of third part of duodenum (n = 168); Group 3 (G3), papilla from the middle of third part to fourth part of duodenum (n = 121). Relative variables among three groups were compared. RESULTS: Compared with G1 and G2, G3 patients had the largest cysts (relative diameter: 1.18 vs. 1.60 vs. 2.62, p < 0.001), the youngest age (20.52 vs. 19.47 vs. -3.40 months, p < 0.001), the highest rate of prenatal diagnosis (26.32% vs. 36.31% vs. 62.81%, p < 0.001), the lowest occurrence of protein plugs in common channel (44.74% vs. 38.69% vs. 16.53%, p < 0.001), and the most elevated total bilirubin level (7.35 vs. 9.95 vs. 28.70 µmol/L, p < 0.001). Prenatally diagnosed G3 patients had heavier liver fibrosis than G2 (13.16% vs. 1.67%, p = 0.015). CONCLUSION: The more distal papilla location, the more severe clinical characteristics of CDCs, suggesting a crucial role in its pathogenesis.

Impact of cancer-associated fibroblasts on survival of patients with ampullary carcinoma.

Background: Cancer-associated fibroblasts (CAFs) reportedly enhance the progression of gastrointestinal surgery; however, the role of CAFs in ampullary carcinomas remains poorly examined. This study aimed to investigate the effect of CAFs on the survival of patients with ampullary carcinoma. Materials and methods: A retrospective analysis of 67 patients who underwent pancreatoduodenectomy between January 2000 and December 2021 was performed. CAFs were defined as spindle-shaped cells that expressed α-smooth muscle actin (α-SMA) and fibroblast activation protein (FAP). The impact of CAFs on survival, including recurrence-free (RFS) and disease-specific survival (DSS), as well as prognostic factors associated with survival, was analyzed. Results: The high-α-SMA group had significantly worse 5-year RFS (47.6% vs. 82.2%, p = 0.003) and 5-year DSS (67.5% vs. 93.3%, p = 0.01) than the low-α-SMA group. RFS (p = 0.04) and DSS (p = 0.02) in the high-FAP group were significantly worse than those in the low-FAP group. Multivariable analyses found that high α-SMA expression was an independent predictor of RFS [hazard ratio (HR): 3.68; 95% confidence intervals (CI): 1.21-12.4; p = 0.02] and DSS (HR: 8.54; 95% CI: 1.21-170; p = 0.03). Conclusions: CAFs, particularly α-SMA, can be useful predictors of survival in patients undergoing radical resection for ampullary carcinomas.

Molecular genetic positioning of small intestine and papilla of Vater carcinomas including clinicopathological classification.

BACKGROUND: Small intestine carcinoma (SIC) cases in Japan have recently been treated with chemotherapy according to colorectal carcinoma classification, while papilla of Vater carcinoma (PVC) cases according to cholangiocarcinoma (CHC) classification. However, few research reports support the molecular genetic validity of these therapeutic choices. PATIENTS AND METHODS: Here, we investigated the clinicopathological and molecular genetic factors of SIC and PVC. We used the data from the Japanese version of The Cancer Genome Atlas. Additionally, molecular genetic data on gastric adenocarcinoma (GAD), colorectal adenocarcinoma (CRAD), pancreatic ductal adenocarcinoma (PDAC), and CHC were also referred to. RESULTS: This study consisted of tumor samples from 12 patients of SIC and three patients of PVC treated from January 2014 to March 2019. Among them, six patients had pancreatic invasion. t-Distributed stochastic neighbor embedding analysis showed that the gene expression pattern of SIC was similar not only to those of GAD and CRAD, but also to that of PDAC in the pancreatic invasion patients. In addition, PVC resembled the GAD, CRAD, and PDAC, rather than the CHC. The molecular genetic characteristics of the six patients with pancreatic invasion were: one had high microsatellite instability, two had a TP53 driver mutation, and three had tumor mutation burden values <1 mutation/Mb with no driver mutation. CONCLUSIONS: In this study, the extensive gene expression profiling of organ carcinomas newly suggests that SIC or PVC may resemble GAD, CRAD, and PDAC. In addition, the data demonstrate that pancreatic invasive patients may be classified into several subtypes using molecular genetic factors.

Efficacy of gel immersion endoscopic ultrasonography for delineating the duodenal papilla and pancreatobiliary ducts: A retrospective study with video.

Objectives: Endoscopic ultrasonography is an important examination for periampullary diseases. The duodenum is filled with water to ensure a clear image and distend the duodenal wall without burying the papilla within duodenal folds; however, peristalsis frequently makes it difficult to maintain water within the duodenum. The gel immersion method (intestine is filled with viscosity gel) has recently been attracting attention. We evaluated the usefulness of using this method for endoscopic ultrasonography to detect and delineate the major duodenal papilla. Methods: Fifty-nine consecutive patients who underwent gel immersion-endoscopic ultrasonography between February and March 2021 were included retrospectively. The papilla was observed by filling the duodenum with clear viscosity gel. Outcomes were the rate of duodenal distention, delineation rates of the papilla, the time required for delineation, volume of the gel used, and adverse events. Results: Duodenal distention was excellent, good, and poor in 58%, 34%, and 7% of cases, respectively. The delineation rates of the papilla in the axial and longitudinal views were 98% and 66%, respectively. The median time required to delineate the papilla in each view was 3.1 (range, 1.0-1.4) and 7.9 (1.9-28.6) min; the median volume of the gel used was 80 (30-150) ml and 100 (50-200) ml, respectively. No adverse events were noted. Conclusions: Gel immersion-endoscopic ultrasonography provided sufficient duodenal distention, leading to high rates of detection and delineation of the papilla using a small volume of gel within a short time. This method may be useful for the evaluation of the ampullary region.

Distinguishing the papilla of Vater during biliary cannulation using texture and color enhancement imaging: A pilot study.

Objectives: Understanding the exact morphology of the bile duct opening is important for determining the success of bile duct cannulation. Texture and color enhancement imaging (TXI) has been reported to enhance slight changes in color tone and structure that are difficult to see with white light imaging. This study investigated whether TXI mode1 could improve papillary recognition by trainees inexperienced in endoscopic retrograde cholangiopancreatography. Methods: We included 31 patients with naive papilla of Vater at a single institution in the study. Trainee endoscopists (n = 4) evaluated and identified the papilla according to the Inomata classification using white light imaging and TXI. The degree of agreement with the evaluation of supervising physicians (n = 4) was examined using the McNemar test. Results: In the trainee group, the kappa coefficient agreements were κ = 0.346 and κ = 0.754 for white light imaging and TXI, respectively. When further evaluated, the separate and septal types of papilla groups showed an increased concordance rate in one of the four trainees (76.67%-96.67%, p = 0.031, respectively). Moreover, comparison for two-group evaluation showed an increased kappa coefficient in two of four trainees (0.34-0.92, p = 0.010, 0.45-0.92, p = 0.024). Conclusions: Observation of the duodenal papilla using TXI improved papillary differentiation and suggested the potential of TXI as a clinical tool. Further study of this method is necessary; it is expected to help reduce cannulation time and the incidence of pancreatitis.

Rare cause of obstructive icterus in a young patient.

In this case report is discussed the diagnosis of papillary carcinoma in the case of a patient in whose age the occurrence of the disease is very rare. The patient underwent a series of non-invasive and invasive examinations, the results of which eventually led to a surgical solution of the suspected lesion (Wipples procedure). Histological examination of the resection confirmed mixed type ampular adenocarcinoma. In the context of this finding, the results of the examination, their information value, interpretation and mutual interaction are discussed. In the postoperative period, the patient suffered from gastrointestinal bleeding, subileum and poor postoperative healing. After hospitalization, the patient was transferred to oncology care and indicated for genetic testing.

Successful endoscopic resection by using gel immersion and the technique of endoscopic papillectomy for a tumor adjacent to the papilla of Vater.

Video 1Successful endoscopic resection using gel immersion for a tumor adjacent to the papilla of Vater.

Prognostic Value of the Regional Lymph Node Station in Pancreatoduodenectomy for Ampullary Carcinoma.

BACKGROUND/AIM: The optimal extent of lymph node dissection for ampullary carcinoma is controversial. The aim of this study was to investigate the efficacy of lymph node dissection for ampullary carcinoma. PATIENTS AND METHODS: Between 2000 and 2020, a total of 75 patients undergoing radical resection for ampullary carcinoma were included. The efficacy index (EI) was calculated by multiplication of the frequency of lymph node metastasis (LNM) at the station and the 5-year survival rate of patients with metastasis at the station. RESULTS: Out of 75 patients, 14 had LNM. The EI for the peri-pancreatic head (station 13 and 17) and superior mesenteric artery (station 14) lymph node were 4.4 and 3.5, respectively. Whereas the peri-gastric (station 5 and 6), common hepatic artery (station 8), and liver hilum (station 12) lymph node stations had zero EI. Although the number of patients with the station 16 dissected was small (9%), the para-aortic (station 16) lymph nodes had the highest EI of 14.3 despite being distant lymph nodes. CONCLUSION: We identified the distribution of LNM and survival benefit of lymph node dissection for ampullary carcinoma. Our results suggest that the optimal extent of lymph node dissection for ampullary carcinoma could be reconsidered.

Divertículos duodenales periampulares y el fallo técnico-terapéutico durante colangiopancreatografía retrógrada endoscópica (CPRE) / Periampullary duodenal diverticula and technical-therapeutic failure during endoscopic retrograde cholangiopancreatography (ERCP)

Resumen Introducción y objetivos: los divertículos duodenales periampulares (DDP) son infrecuentes y su hallazgo es incidental. Además, se clasifican en tres tipos según Boix por la cercanía a la ampolla mayor. El objetivo de este estudio fue realizar una descripción de nuestra experiencia con esta anomalía anatómica y demostrar la forma en que esto afecta la tasa de éxito técnica y terapéutica del procedimiento. Material y métodos: se realizó un estudio de corte transversal, con recolección de datos de forma retrospectiva en un período de 5 años de pacientes con divertículos periampulares duodenales. Se evaluó el fracaso terapéutico, complicaciones y dificultad de canulación de la vía biliar. Resultados: se evaluó un total de 214 pacientes, con una relación mujer-hombre de 2,15:1. La distribución de los sujetos por tipo de DDP fue: tipo 1 (29,9 %), tipo 2 (51,9 %) y tipo 3 (18,2 %). La indicación más frecuente de CPRE fue los cálculos del conducto biliar común en un 53,3 %. El DDP tipo 1 presentó mayor dificultad de canulación (11,6 %) y falla terapéutica (28,12 %). Conclusión: la presencia de DDP durante la CPRE se asocia con una mayor falla técnica (falla en la canulación) y falla terapéutica (persistencia de la obstrucción biliar). Además, estas fallas aumentan considerablemente cuando se trata de una papila intradiverticular tipo 1 de la clasificación según Boix. Por lo anterior, se sugiere que los procedimientos endoscópicos biliares en estas condiciones sean realizados por endoscopistas con gran experiencia con el fin de minimizar la probabilidad de falla técnica y terapéutica, y las complicaciones asociadas.

Abstract Introduction and objectives: Periampullary duodenal diverticula are infrequent, and their finding is incidental. They are classified into three types according to Boix due to their proximity to the larger blister. This study aims to describe the experience with this anatomical abnormality and to demonstrate how this affects the technical and therapeutic success rate of the procedure. Materials y Methods: A cross-sectional study was conducted, with retrospective data collection over a 5-year period of patients with periampullary duodenal diverticula. Therapeutic failure, complications, and difficulty of cannulation of the bile duct were evaluated. Results: A total of 214 patients were evaluated, with a female-male ratio of 2.15: 1. The distribution of the subjects by type of PDD was: type 1 (29.9%), type 2 (51.9%), and type 3 (18.2%). The most frequent indication for ERCP was common bile duct stones in 53.3%. Type 1 PDD presented greater difficulty in cannulation (11.6%) and therapeutic failure (28.12%). Conclusion: The presence of PDD during ERCP is associated with greater technical failure (failure in cannulation) and therapeutic failure (persistence of biliary obstruction). In addition, this failures increases considerably when it is a type 1 intradiverticular papilla of the Boix classification. Therefore, it is suggested that biliary endoscopic procedures in these conditions are performed by highly experienced endoscopists to minimize the probability of technical and therapeutic failure and associated complications.

Acute cholangitis detected ectopic ampulla of Vater in the antrum incidentally: A case report.

BACKGROUND: The ampulla of Vater is an opening at the confluence of the common bile duct and pancreatic duct. It is located in the second portion of the duodenum. An ectopic papilla of Vater is an anomalous termination. Few cases have been reported. We report the rare case of a man with an ectopic ampulla of Vater in the pylorus. CASE SUMMARY: An 82-year-old man had experienced abdominal pain and fever with chills 1 d before his presentation. A computed tomography scan of the abdomen demonstrated dilatation of the common bile duct approximately 2.2 cm in width. Gas retention was found in his intrahepatic ducts. Acute cholangitis with pneumobilia was identified, and he was hospitalized. Esophagogastroduo-denoscopy and endoscopic retrograde cholangiopancreatography disclosed no ampulla of Vater in the second portion of the duodenum. Moreover, a capsule-like foreign body (pharmaceutical desiccant) approximately 1 cm × 2 cm in size was found at the gastric antrum and peri-pyloric region. After the foreign body was removed, one orifice presented over the pyloric ring in the stomach, a suspected ectopic ampulla of Vater. Subsequently, sludge in the common bile duct was cleaned, and balloon dilatation was performed. The general condition improved daily. The patient was discharged in a stable condition and followed in our outpatient department. CONCLUSION: This case involved an ampulla of Vater in an unusual location. Endoscopic retrograde cholangiopancreatography with balloon dilatation is the main treatment recommended and performed.

Ampullary Carcinosarcoma with Enteroblastic Differentiation: A Case Report.

Adenocarcinoma with enteroblastic differentiation is an extremely rare tumor with poor prognosis and unique pathologic features. The tumor appears to be relatively more common in stomach, with rare cases reported in esophagus, colon, rectum and ampulla. Underrecognition by pathologists may be a contributing factor towards underreporting of this tumor. Combination of carcinosarcoma and enteroblastic differentiation has not been reported so far.We report a unique case of ampullary carcinosarcoma with enteroblastic differentiation in a 59-year-old female, diagnosed in the pancreatoduodenectomy specimen. The carcinomatous component showed features of enteroblastic differentiation characterized by tubular architecture with clear cytoplasm, solid component with trabecular architecture and immunohistochemical expression of SALL4 and AFP. The patient was treated with adjuvant Folfirinox chemotherapy and is disease free at 17 months follow up.

Unusual Biliary Complication following Christmas Eve Dinner.

We present a case of a fish bone impacted in the papilla of Vater resulting in dyspepsia and mild elevation in liver function tests, which was subsequently treated endoscopically. Fish bones are one of the most commonly encountered swallowed foreign bodies. However, involvement of the biliary tract, such as the one described by us, represents an extremely rare complication of fish bone ingestion. The diagnosis of a foreign body in the biliary tract can be difficult, and early endoscopic or surgical extraction may be required to avoid complications such as biliary stone formation, obstructive jaundice, cholangitis or cholecystitis, and/or biliary sepsis. Prompt endoscopic treatment can avoid severe biliary complications or surgical therapy.

Clinical outcomes of patients with duodenal adenocarcinoma and intestinal-type papilla of Vater adenocarcinoma.

BACKGROUND: Duodenal adenocarcinoma (DA) and intestinal-type papilla of Vater adenocarcinoma (it-PVA) are rare malignancies of the gastrointestinal tract. Current therapeutic options are translated nowadays from treatment strategies for patients with colorectal cancer due to histopathological similarities. AIM: To retrospectively investigate the clinical outcome of patients with DA and it-PVA. METHODS: All patients with DA and it-PVA diagnosed between 2000 and 2017 were included at two academic centers in the Netherlands. All patients with histopathologically-confirmed DA or it-PVA were eligible for inclusion. Clinical outcome was compared between DA and it-PVA per disease stage. In the subgroup of stage IV disease, survival after local treatment of oligometastases was compared with systemic therapy or supportive care. RESULTS: In total, 155 patients with DA and it-PVA were included. Patients with it-PVA more often presented with stage I disease, while DA was more often diagnosed at stage IV (P < 0.001). Of all patients, 79% were treated with curative intent. The median survival was 39 mo, and no difference in survival was found for patients with DA and it-PVA after stratification for disease stage. Seven (23%) of 31 patients with synchronous stage IV disease underwent resection of the primary tumor, combined with local treatment of oligometastases. Local treatment of metastases was associated with an overall survival of 37 mo, compared to 14 and 6 mo for systemic therapy and supportive care, respectively. CONCLUSION: Survival of patients with DA and it-PVA is comparable per disease stage. These results suggest a potential benefit for local treatment strategies in selected patients with oligometastases, although additional prospective studies are needed.

Choice of surgical procedures for patients with stage T1 carcinoma of the papilla of Vater: a retrospective study.

BACKGROUND: Given the high incidence of complications after pancreaticoduodenectomy (PD), local resection is being applied to cure stage T1 carcinoma of the papilla of Vater (CPV). In the present study, risk factors related to nodal involvement and prognosis were evaluated so as to enable the choice of optimal surgical procedure for patients with stage T1 CPV. METHODS: A retrospective study of 94 consecutive patients with CPV who underwent PD in our center from 2013 to 2018 was conducted. RESULTS: A total of 44 patients (46.8%; 44 of 94) had lymph node metastasis. T1 tumors were subdivided into layer I (the mucosa) and layer II (the submucosa) based on anatomical stratification, and lymph node metastasis did not occur in patients with layer I invasion. The nodal metastasis rate was up to 25% (6 of 24) in patients with layer II invasion. The gross appearance, depth of duodenal invasion, pT stage and perineural invasion were risk factors related to nodal involvement. Only the depth of duodenal invasion remained a significant independent factor (P=0.003). Multivariate Cox analysis indicated that depth of duodenal invasion (P=0.001), nodal involvement (P<0.001), and venous invasion (P<0.001) were independent prognostic factors. The depth of duodenal invasion is the only independent risk factor related to nodal involvement and prognosis. CONCLUSIONS: The optimal surgical option should be PD with radical lymphadenectomy for patients with stage T1 CPV; only patients with duodenal invasion limited to the mucosa are suitable for local resection. A modified T category needs to be proposed based on the detailed depth of duodenal invasion.

Hidden papilla and periampullary diverticulum: Curtain up for stacked folds.

Proper identification of the Vaterian papilla is a vital prerequisite for success in endoscopic retrograde cholangiopancreatography. Overall, a "hidden papilla" situation is uncommon, often attributable to, for example, intradiverticular localizations in a setting of periampullary diverticulum. Stacked duodenal folds precluding proper endoscopic visualization of the papilla is less well discussed, with virtually no systematic data available. A concurrent presence of the former potential obstacles to an immediate recognition of the papilla has not been reported before. With a view of the sometimes challenging task of finding (and cannulating) a periampullary diverticulum-related papilla, suggestions are made to first scrutinize curtaining duodenal folds, for example, by endoscopic probing with an endoscopic retrograde cholangiopancreatography catheter as this may represent a more easily remediable cause underlying a "hidden papilla.".

A Rare Case of Ampullary Goblet Cell Carcinoid.

An asymptomatic 70-year-old woman was referred to our hospital because of liver enzyme elevation. Enhanced abdominal computed tomography demonstrated a small, round-shaped tumor with dilation of the common bile duct and main pancreatic duct. A biopsy specimen from the papilla showed mucin-containing cells that were positive for endocrine markers on immunohistochemical staining. Endoscopic snare resection was done, and there was a positive vertical margin on pathology. Pancreaticoduodenectomy was then performed later. The final diagnosis was goblet cell carcinoid, pT2N0M0, pStage IIA [Union for International Cancer Control (UICC) 7th edition]. Ampullary goblet cell carcinoid is an extremely rare disease of which there have been no recent reports.

Experience of pancreaticoduodenectomy and the diagnosis of carcinoma of the papilla of Vater / 中华肝胆外科杂志

Objective To summarize our experience in the diagnosis and treatment of carcinoma of the papilla of Vater (CPV).Methods From April 2005 to May 2012,21 consecutive patients were enrolled in Dongguan Affiliated Hospital of Southern Medical University.The clinical characteristics,diagnosis,surgical data and follow-up results of these patients were retrospectively analyzed.Results Painless jaundice was the primary symptom in 18 patients.An elevated CEA level was observed in 3 patients preoperatively,while CA19-9 was increased in 10 patients.17 patients with periampullary tumors were detected by MRCP or CT,and in 12 patients,they were detected by ultrasound examination.Gastroscopy was performed in 16 patients,among whom 8 patients were diagnosed with neoplasm of the duodenal papilla initially,and 5 patients were ultimately diagnosed with CPV by pathological examination after biopsy.All the 21 patients were successfully treated with pancreaticoduodenectomy.8 patients developed postoperative complications.No death occurred in the perioperative period.The complications included pancreatic fistula (2 patients),gastroparesis (2 patients),incisional infection (2 patients),gastrointestinal hemorrhage (1 patient) and concurrent gastrointestinal and intraperitoneal haemorrhage (1 patient).A reoperation consisting of pancreaticoduodenal repair and drainage was performed in 1 patient.3 patients underwent adjuvant chemotherapy.Long-term follow up was obtained in 18 patients.The 1-year,3-year and 5-year survival rates were 80.9％,66.7％ and 47.6％,respectively.The longest overall survival was 152 months.Conclusions Early diagnosis of CPV was feasible.Pancreaticoduodenectomy as a treatment strategy for this cancer was safe and effective.