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Current strategies in the therapeutic approach for adenocarcinoma of the ampulla of Vater

Authors:
  • Carol Davila University of Medicine and Pharmacy Bucharest
  • Spitalul Clinic de Urgenţă Bucureşti

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Introduction: Ampulla of Vater tumors, neoplastic diseases located at the confluence of the common bile duct with the main pancreatic duct; represent 0.2% of all gastrointestinal cancers. Method: Retrospective study of all patients admitted in the Emergency Hospital of Bucharest Romania between January 2008 and January 2013, the only selection criterion used being a pathology report which describes an ampulla of Vater carcinoma. We have also performed a review of the medical literature up to 2013, using the PubMed/Medline, Proquest Hospital Collection, Science Direct, Cochrane Library and Web of Science databases. We have used different combinations of the following keywords: "ampulla of Vater", "carcinoma", "resection", reviewing the reference list of retrieved articles for further relevant studies. Results: Forty eight patients with ampulla of Vater carcinoma were identified, of whom 59.6% men, 71% from urban areas, and a mean age of 66 ± 13.3 years. Most patients were admitted for obstructive jaundice (49%), right upper quadrant abdominal pain (19%), nausea and loss of appetite in 13%, loss of weight (13%) and upper digestive obstruction in 6% of cases. All patients were evaluated with abdominal transparietal ultrasonography and double contrast, pancreatic protocol, Mutidetector Row Computed Tomography. The abdominal Magnetic Resonance Imaging was performed in 10 cases, upper gastrointestinal endoscopy in 9 cases, and Endoscopic Retrograde Cholangiopancreatography in 39 cases. According to the AJCC Cancer Staging 9% were into stage I, 47% into stage II, 40% into stage III and 4% into stage IV of the disease. The therapeutic approach was surgical for 44 patients and an endoscopic palliation with stent insertion in 4 cases. The surgical procedure was represented by Whipple pancreatoduodenectomy in 27 cases, pylorus preserving pancreatoduodenectomy in 15 cases and exploratory laparotomy in 2 cases. Early morbidity was represented by pancreatic leakage in 4 cases. Conclusions: There are clinical scenarios in which it is quite challenging to distinguish a primary ampullary adenocarcinoma based on a preoperative workup. Nevertheless, an aggressive approach should be performed, knowing the higher resectability rates and a five-year survival for these patients. Complete surgical resection should be performed in all medically fit patients, candidates for pancreatoduodenectomy, by a high volume, trained surgeon, able to offer a low morbidity and mortality.
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... According to the morphology and location, the ampulla of Vater cancers can be divided into three different categories: intra-ampullary neoplasms, periampullary neoplasms, and mixed neoplasms [3]. Occasionally, malignancy at the ampulla of Vater may cause recurrent episodes of pancreatitis [4]. ...
... This case report raises awareness about the limitations of imaging and biopsies in establishing a definitive diagnosis, especially when dealing with suboptimal samples or focal lesions [4]. In cases with persistent clinical suspicion despite initial negative results, repeat investigations or alternative diagnostic modalities should be considered [5]. ...
Article
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The ampulla of Vater is a small opening located at the point where bile and pancreatic ducts join and empty their secretions into the small intestine. Ampullary cancers are rare but aggressive malignancies that can present with symptoms similar to those of acute pancreatitis, including abdominal pain, nausea, vomiting, and obstructive jaundice. Clinicians must rely on a combination of blood tests, imaging, and biopsies to diagnose ampullary cancer, which may be a hidden cause of acute pancreatitis. In this report, we present the case of a 66-year-old female who presented to our hospital with recurrent admissions due to abdominal pain, nausea, and vomiting. The patient was found to have repeated episodes of acute pancreatitis and was later diagnosed with cancer of the ampulla of Vater. This case proved extremely complex and diagnostically challenging.
... Whipple operation is the first choice in malignant adenocarcinoma in the first and second parts of the duodenum, and segmental resection is useful in distal duodenal tumors. 6,11 Pancreaticoduodenectomy (Whipple procedure) is a curative surgery and therapeutic option for tumors located in the first and second parts of the duodenum, giving a better prognosis than in duodenal resection. 2,9,11,12 Laparotomy is performed for resection and anastomosis of the affected part with a limit of 10 centimeters proximal and distal, including the mesentery and lymph nodes. ...
... 6,11 Pancreaticoduodenectomy (Whipple procedure) is a curative surgery and therapeutic option for tumors located in the first and second parts of the duodenum, giving a better prognosis than in duodenal resection. 2,9,11,12 Laparotomy is performed for resection and anastomosis of the affected part with a limit of 10 centimeters proximal and distal, including the mesentery and lymph nodes. If curative resection is not possible, palliative resection can be performed with anastomose or stoma. ...
Article
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Periampullary carcinoma is a malignancy that appears around the vatteri ampulla. This malignancy can originate from the pancreas, duodenum, and distal choledochal duct. Duodenal cancer is a very rare case, only about 0.3% of the gastrointestinal malignancies. Reported cases are increasing with the increasing use of esophagogastroduodenoscopy. The most frequently found carcinomas of the duodenum include adenocarcinoma, carcinoid, lymphoma, and leiomyosarcoma. The symptoms of these carcinomas are often not specific so the diagnosis is often late, leading to a poor prognosis. Early diagnosis and proper therapy provide a good prognosis. The case reported here representeda 52-year-old woman presented with hematemesis melena, anemia, jaundice, epigastric mass, right hypochondrial pain, and weight loss. From the esophagogastroduodenoscopy, the mass was found to obstruct half of the duodenum lumen and bleeding, which was easily triggered, was observed in the second part of the duodenum. CT scan revealed a mass in the head of the pancreas with gall bladder hydrops and obstruction of the intra and extra-hepatic billier system. After a Whipple operation Laparotomy, the histology showed papillary adenocarcinoma duodenum. Patients then underwen chemotherapy with 5 fluorouracil regimen. Patient's clinical condition is currently improving and no complaint is conveyed by the patient.
... Periampullary tumors arise within tissues near the confluence of the common bile duct with the main pancreatic duct, (i.e., the ampulla of Vater) [1]. These tumors have many different origins, with 60% arising from the pancreatic head, 20% from the ampulla of Vater, 10% from the distal common bile duct, and 10% from the duodenum [1]. ...
... Periampullary tumors arise within tissues near the confluence of the common bile duct with the main pancreatic duct, (i.e., the ampulla of Vater) [1]. These tumors have many different origins, with 60% arising from the pancreatic head, 20% from the ampulla of Vater, 10% from the distal common bile duct, and 10% from the duodenum [1]. It is often difficult to distinguish these cancer types using pretreatment needle biopsies. ...
Article
Herein, we report on a patient with known Lynch syndrome and periampullary adenocarcinoma that exhibited a pathological complete response to neoadjuvant nivolumab plus ipilimumab. Two MSH2 mutations, high microsatellite instability, high tumor mutational burden, and elevated PD-L1 expression were identified by next-generation sequencing and immunohistochemistry. Following FOLFIRINOX (Fluorouracil/Leucovorin/Irinotecan/Oxaliplatin) administration and disease progression, nivolumab (1 mg/kg) and ipilimumab (3 mg/kg) were administered every 3 weeks for four total cycles. The patient responded well with minimal adverse effects and significant improvement in epigastric pain, appetite, and body weight. She then underwent resection consisting of pancreaticoduodenectomy, which demonstrated pathological complete response. Complete genomic profiling of periampullary carcinomas is crucial for optimal treatment selection as true ampullary masses and pancreatic ductal adenocarcinoma have different genetic profiles. This case provides an example of a patient who may have further benefited from first-line nivolumab plus ipilimumab to avoid the reduced efficacy and significant side effects associated with chemotherapy. Key Points A patient with known Lynch syndrome and ampullary adenocarcinoma harboring two MSH2 mutations, high microsatellite instability (MSI-high), high tumor mutational burden (TMB), and elevated PD-L1 expression achieved pathological complete response with neoadjuvant nivolumab plus ipilimumab. The combination of nivolumab plus ipilimumab may be a better first-line option for patients with ampullary adenocarcinomas harboring deficient mismatch repair, MSI-high, and high TMB. Complete genomic profiling of periampullary adenocarcinomas is crucial for optimal treatment selection as true ampullary masses and pancreatic ductal adenocarcinoma have different genetic profiles. The presence of either MSI-high or high TMB could be an appropriate predictive biomarker for response to nivolumab plus ipilimumab in the context of Lynch syndrome.
... The carcinoma of the ampulla of Vater represents 0.5% of all gastrointestinal tumors and 7% of all periampullary tumors, with approximately 6 cases per million population per year [1,8]. This neoplasm has a resectability percentage of 80% and 5-year survival of 30-70% [2,3,8]. ...
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A hypertensive, diabetic woman underwent a successful Whipple procedure at the age of 84 due to carcinoma of the ampulla of Vater. She presented an extremely rare complication 24 months after the surgery, consisting of acute cholangitis due to multiple biliary lithiases associated with a bilioenteric anastomotic stricture. The diagnosis was confirmed with computed tomography, magnetic resonance cholangiopancreatography, and cholangiography. The patient was successfully treated with multiple percutaneous transhepatic cholangioplasties.
... When the body composition parameters of three different pathologic types of periampullary tumors classi ed as benign tumors, pancreatic carcinoma and non-pancreatic carcinoma were compared, no signi cant difference were observed. Pancreas is the exocrine organ of great importance through secreting several enzymes assisting the digestion, and more than 50% of patients with pancreatic ductal carcinoma suffered from weight loss compared to the percentage about 10% in ampullary cancer group [30][31] . Causes of high incidence of weight loss in pancreatic carcinoma group can be attributed to tissue-brosis, exocrine and endocrine insu ciency, obstruction of duodenum and cancer induced cachexia [32][33] . ...
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Background To observe body composition parameters variance in patient with periampullary neoplasmas with different clinical characteristics and assess its predictive value for postoperative complications after pancreaticoduodenectomy. Methods In this study, we retrospectively reviewed the clinical and image data of 144 patients with periampullary neoplasmas.The area of subcutaneous adipose tissue (SAT), visceral adipose tissue (VAT) and total abdominal muscle area (TAMA) were measured from preoperative CT images at the 3rd lumbar vertebra level, the TAMA was normalized to stature and termed as skeleton muscle index (SMI). The perioperative and pathological data were collected. Results Of the included 144 patients, 80(55.6%), 29(20.1%) and 24(16.7%) patients were classified as sarcopenia, visceral obesity and sarcopenic obesity. 84(58.3%) patients were jaundiced and 28 (19.4%),50 (34.7%),66(45.8%) patients were diagnosed with benign pancreatic tumors, pancreatic cancer and non-pancreatic cancer respectively. The incidence rate of clinical postoperative pancreatic fistula(POPF) and other major complications were 38.2% and 16%.In the univariate analysis, jaundiced patients experienced more weight loss and had higher nutrition risk score, the TAMA[103.1(61.1-176.7) vs 111.8(74.1-198.2),P=0.021] and SMI(39.2±7.0 vs 42.6±9.1,P=0.012)were lower compared with non-jaundiced group. However, no significant difference were founded between different pathological results and it was not associated with occurrence of POPF and major complications. Conclusion Jaundiced patients may experience more weight loss and have lower TAMA and SMI. Body morphometric analysis of preoperative CT did not show predictive value for postoperative complications and further multicenter studies are needed. Trail registration Registration number:2021-437-01.
... Ung thö vuø ng boù ng Vater laø beä nh lyù thöôø ng xuyeâ n gaë p ôû vuø ng oá ng tieâ u hoù a, trong ñoù ung thö ñaà u tuï y chieá m 80%, u boù ng Vater (10%), ung thö ñoaï n cuoá i oá ng maä t chuû (5%) vaø ung thö taù traø ng (5%). Rieâ ng u boù ng Vater chieá m khoaû ng 6% caù c khoá i u vuø ng quanh boù ng Vater vaø chieá m khoaû ng 0,2% taá t caû caù c beä nh ung thö ñöôø ng tieâ u hoù a [1,2]. So vôù i ba loaï i ung thö coø n laï i, ung thö boù ng Vater coù tyû leä soá ng theâ m 5 naê m sau moå chieá m 30%-50% khi chöa coù di caê n haï ch [3]. ...
Article
Tóm tắt Đặt vấn đề: Đánh giá kết quả bước đầu, chỉ định và biến chứng trong phẫu thuật nội soi hỗ trợ cắt khối tá tràng đầu tụy. Phương pháp nghiên cứu: Mô tả tiến cứu 15 trường hợp được phẫu thuật tại Bệnh viện Bạch Mai từ 9/2016 – 9/2017. Kết quả: Chỉ định mổ bao gồm: u bóng Vater (12 người bệnh), u đầu tụy (2 người bệnh), u nang đầu tụy (1 người bệnh). Tuổi trung bình: 53,6 + 11,8 (dao động 37 – 72 tuổi), thời gian mổ trung bình 265,3 + 55 phút trong đó thời gian mổ nội soi 139,5 + 44,3 phút với đường mổ mở dài 8,6 + 3,4 cm, tổng số hạch nạo vét trung bình 9+ 2,6 hạch. Ba người bệnh chuyển mổ mở (20%) với lượng máu mất trong mổ trung bình 438 + 305 ml, thời gian nằm viện 18,3 ngày. Tai biến và biến chứng gặp: 1 người bệnh cắt phải động mạch mạc treo tràng trên (6,7%), 6 người bệnh rò tụy (40%) chủ yếu mức độ A (26,6%), 4 người bệnh rò mật (26,7%), 3 người bệnh chậm lưu thông dạ dày (20%), 1 người bệnh tử vong (6,7%). Kết luận: Phẫu thuật nội soi hỗ trợ có thể áp dụng điều trị các khối u vùng bóng Vater trên những người bệnh được lựa chọn. Hiệu quả và mức độ an toàn của phẫu thuật cần theo dõi thêm với số lượng lớn hơn. Abstract Introduction: We report the clinical short-term outcomes of laparoscopic-assisted pancreatoduodenectomy (LAPD) for periampullary tumors. Material and Methods: A retrospective review of patients who underwent LAPD from 9/2016 to 9/2017 at Bach Mai University Hospital. Results: Fifteen patients were included in this study. The preoperative diagnoses were ampullary carcinoma (n = 12), pancreatic head tumors (n = 2) and intraductal papillary mucinous neoplasm (n = 1). The median age was 53.6 years (range 37 – 72 years). The median operating time was 265.3 minutes (range 180 – 360 minutes) with the median time of laparoscopic approach was 139.5 mins and the median estimated blood loss was 438 ml (range 150 - 1241 ml). The median incision length for laparotomy was 8.6 cm (range 5 – 15 cm). The averaged lymph node collection was 9 + 2.6 nodes. The median hospital stay was 18.3 days with three patients that underwent conventional open surgery. One patient with injury superior mesenteric artery (SMA) during laparoscopic approach that needed be to repaired. Postoperative complications were pancreatic fistula (40%), bile leakage (26.7%), delayed empty gastric (20%) and mortality (6.7%). Conclusion: LAPD is a technically safe and feasible alternative treatment for selected patients with periampullary tumors. The long-term outcomes and potential benefits of this technique need to be obsevered in a larger patient population. Keyword: Pancreatoduodenectomy, Laparoscopic-assisted pancreatoduodenectomy, Laparoscopic pancreatoduodenectomy assisted by mini laparotomy.
... Ampulla of Vater cancer (AVC) is defined as a malignancy that arises within the ampullary complex, distal to the confluence of the distal common bile duct and the pancreatic duct. AVC is a rare malignancy and accounts for 0.2% of all digestive malignancies, with an annual incidence of 0.4-0.5 cases per 100,000 people per year [1,2]. ...
Article
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Several studies have compared laparoscopic pancreaticoduodenectomy (LPD) and open pancreaticoduodenectomy (OPD) in patients with periampullary carcinoma; however, only a few studies have made such a comparison on patients with ampulla of Vater cancer (AVC). We compared the perioperative and oncologic outcomes between LPD and OPD in patients with AVC using propensity-score-matched analysis. A total of 359 patients underwent PD due to AVC during the study period (76 LPD, 283 OPD). After propensity score matching, the LPD group showed significantly longer operation time than did the OPD group (400.2 vs. 344.6 min, p < 0.001). Nevertheless, the LPD group had fewer painkiller administrations (8.3 vs. 11.1, p < 0.049), fewer Grade II or more severe postoperative complications (15.9% vs. 34.8%, p = 0.012), and shorter postoperative hospital stays (13.7 vs. 17.3 days, p = 0.048), compared with the OPD group. There was no significant difference in recurrence-free outcomes and overall survival between the two groups (p = 0.754 and 0.768, respectively). Compared with OPD, LPD for AVC had comparative oncologic outcomes with less pain, less postoperative morbidity, and shorter hospital stays. LPD may serve as a promising alternative to OPD in patients with AVC.
... Surgical resection can be performed with pancreaticoduodenectomy as the standard approach. In high-risk patients, local or endoscopic treatment can be offered [8]. Five-year survival rates are about 10% in pancreatic adenocarcinoma and 40% in AoV adenocarcinoma after adequate resection [7]. ...
Article
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Periampullary neoplasms are a heterogeneous group of tumors arising within 2 cm of the ampulla of Vater. Neuroendocrine tumors can originate throughout the entire body, from neuroendocrine cells. These neoplasms exhibit deep differences, according to their origin and biological behavior. We describe a case of a 79-year-old man who underwent pancreaticoduodenectomy for adenocarcinoma of the ampulla of Vater after proper staging. At gross histology, an incidental pancreatic neuroendocrine tumor was also documented. Despite two synchronous neoplasms, the patient survived 34 months with no evidence of recurrence at follow-up. The synchronous presence of a second primitive tumor in patients affected by a neuroendocrine tumor is reported in the literature; incidence is variable and the most common site is the gastrointestinal tract. Diagnostic workup for ampullary neoplasms includes abdominal computed tomography (CT) scan, magnetic resonance imaging (MRI) and endoscopic ultrasound (EUS). These investigations infrequently may detect subcentimetric lesions. We believe this case is currently extremely rare. Preoperative diagnosis of synchronous PanNET would not have changed our approach since surgical therapy represents the gold standard in resectable ampullary neoplasms, and it has a primary role in the prognosis of the present patient.
... Working up the various types of periampullary tumors, and making an accurate preoperative diagnosis, can be challenging. 1,2 Intraoperative pancreatic ductoscopy (IPD) has been reported to have greater sensitivity and specificity in detecting surgical pathology when compared to endoscopic retrograde cholangiopancreatography (ERCP) and endoscopic ultrasound (EUS). IPD has been shown to be safe and effective in evaluating main duct intraductal papillary mucinous neoplasms (MD-IPMN), with a specific advantage of diagnosing multicentric lesions. ...
Article
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Background: Periampullary neoplasms can be challenging to work up and diagnose preoperatively. Herein, we report the case of a patient whose preoperative workup failed to detect a malignancy, yet, underwent a pylorus-preserving pancreaticoduodenectomy (PPPD) with intraoperative pancreatic ductoscopy (IPD) and was ultimately found to have an ampullary adenocarcinoma. Presentation: A 78-year-old woman presented with 4 weeks of nausea, weight loss, jaundice, and light-colored stools. She underwent outpatient diagnostic studies, including magnetic resonance cholangiopancreatography, endoscopic ultrasound, and endoscopic retrograde cholangiopancreatography with pancreatic duct (PD) stenting and papillotomy. These revealed common bile duct dilatation measuring 2 cm, PD dilatation measuring 7 mm, a 17 mm cyst in the head of the pancreas, and a firm nodule noted between the biliary and pancreatic orifices. Cytologic and pathologic analyses were initially nondiagnostic. A repeat ampullary biopsy was negative for dysplasia and malignancy. A computed tomography scan was then performed and showed cystic pancreatic lesions with pancreatic ductal dilation. Suspicion remained high for periampullary tumor or a main duct intraductal papillary mucinous neoplasm, and the patient underwent a PPPD with IPD and tolerated the procedure well. Her final specimen pathology revealed well-to-moderately differentiated ampullary adenocarcinoma, pancreaticobiliary type with positive nodal disease. Conclusions: Given the relatively poor prognosis of patients with node-positive pancreaticobiliary-type ampullary adenocarcinoma, clinical suspicion should remain high for malignancy in patients with lesions located in the periampullary region and a negative preoperative workup, as aggressive treatment approaches are warranted to maximize their chance for survival.
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Duodenal tumors are less frequently than those of stomach and colon. The habitual ones are carcinomas, lymphomas and malignant or bening stromal tumors, duodenal polyps are usually asymptomatic. The haemorraghe is the most frequent sign. The great size lesions can show a suboclussive presentation and the periampullary ones can produce. A 68-year-old female patient to whom cholecystectomy was performed was presented in this article. The patient was admitted due to recurrent jaundice, episodes of dark urine, acholia, generalized pruritus, weight loss and epigastric pain. Ultrasonography of the liver, biliary tract and pancreas was performed in which bile duct dilatation was evident, duodenum was also explored and a lesion was observed. Biopsy was done and an adenoma of duodenal papilla was diagnosed. Surgical ampulectomy was performed without adenocarcinoma findings in the surgical biopsy, the jaundice disappeared and none postsurgical complication was observed.
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Intestinal and pancreaticobiliary types of Vater's ampulla adenocarcinoma have been considered as having different biologic behavior and prognosis. The aim of the present study was to determine the best immunohistochemical panel for tumor classification and to analyze the survival of patients having these histological types of adenocarcinoma. Ninety-seven resected ampullary adenocarcinomas were histologically classified, and the prognosis factors were analyzed. The expression of MUC1, MUC2, MUC5AC, MUC6, CK7, CK17, CK20, CD10, and CDX2 was evaluated by using immunohistochemistry. Forty-three Vater's ampulla carcinomas were histologically classified as intestinal type, 47 as pancreaticobiliary, and seven as other types. The intestinal type had a significantly higher expression of MUC2 (74.4% vs. 23.4%), CK20 (76.7% vs. 29.8%), CDX2 (86% vs. 21.3%), and CD10 (81.4% vs. 51.1%), while MUC1 (53.5% vs. 82.9%) and CK7 (79.1% vs. 95.7%) were higher in pancreatobiliary adenocarcinomas. The most accurate markers for immunohistochemical classification were CDX2, MUC1, and MUC2. Survival was significantly affected by pancreaticobiliary type (p = 0.021), but only lymph node metastasis, lymphatic invasion, and stage were independent risk factors for survival in a multivariate analysis. The immunohistochemical expression of CDX2, MUC1, and MUC2 allows a reproducible classification of ampullary carcinomas. Although carcinomas of the intestinal type showed better survival in the univariate analysis, neither histological classification nor immunohistochemistry were independent predictors of poor prognosis.
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Eighty of 89 patients who underwent radical resection (resectability 89.9%) for carcinoma of the papilla of Vater between 1976 and 1992 were retrospectively reviewed. Seventy-three patients underwent pancreaticoduodenectomy (PD) and 7 underwent pylorus-preserving pancreaticoduodenectomy (PPPD). The postoperative mortality rate was only 3.8% (3 patients). The 3- and 5-year survival rates were 63.6% and 57.4%, respectively. Important factors influencing long-term survival were Stage (clinical stage = Stage), microscopic lymph node metastasis (n), duodenal wall invasion (d), vascular invasion (v), and the epithelium of origin. Early carcinoma of the papilla of Vater is defined as tumor in which invasion is limited within the papilla of Vater; in particular, carcinomatous invasion is within the muscle of Oddi (d0) with n0. PD and/or PPPD with radical lymph node dissection should be performed for carcinoma of the papilla of Vater, as these procedures can be performed with low morbidity and mortality.
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Carcinomas co-occur in the pancreas, extrahepatic bile ducts, and ampulla of Vater. We investigated whether cancers originating in these sites represent a field effect similar to that observed in the lung and upper aerodigestive tract. To determine whether a field effect for carcinogenesis exists in the ampulla of Vater, extrahepatic bile ducts, gallbladder, and pancreas. Data were obtained from National Cancer Institute's Surveillance Epidemiology and End Results Program from 1973 through 2005. Cases were compared by age frequency density plots, age-specific incidence rates, and logarithmic plots of the age-specific incidence rates and age of diagnosis. Incidence rates were 11.71, 1.43, 0.88, and 0.49 per 100,000 persons at risk for pancreatic, gallbladder, extrahepatic bile ducts, and ampullary carcinomas, respectively. Age frequency density plots were congruent for cancers originating in all 4 sites. Logarithmic plots of the age-specific incidence rates with age of diagnosis produced parallel linear rate patterns for the 4 sites indicative of similar populations for tumor development. However, density and logarithmic plots of pancreatic endocrine carcinomas, a tumor of different cellular differentiation and carcinogenic pathway, served as a comparison. The endocrine carcinomas showed a different age distribution and nonparallel rate patterns with ductal carcinomas. Carcinomas of the pancreas, gallbladder, extrahepatic bile ducts, and ampulla have a common embryonic cellular ancestry, differentiation pathways, mucosal histologic patterns, and population-related tumor development indicating a field effect in carcinogenesis. Parallel linear rate patterns indicate (1) the rate of cancer development is similar in all 4 sites even though the absolute incidence rates vary and (2) regardless of location, the ductal epithelium is equally susceptible to malignant transformation. If carcinogenic pathways to cancer are similar, then the different incidence rates seen clinically may depend on the relative surface area of the ductal system in these sites. Pancreatic cancers are most common because the surface area of the pancreas' ductal system is greater than that of the gallbladder, extrahepatic bile ducts, and ampulla.
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One hundred four consecutive patients who underwent radical resection for ampullary cancer between 1965 and 1989 were retrospectively reviewed. Frequent clinical findings included jaundice (67%), significant (greater than 10%) weight loss (42%), and anemia (27%). Eighty-seven patients (84%) underwent a subtotal pancreatectomy, and 17 patients (16%) underwent a total pancreatectomy. The postoperative mortality was 5.7% (six patients), and reoperation for postoperative complications was required in six patients. The 5- and 10-year survival rates were 34% and 25%, respectively. Eight patients died of tumor recurrence more than 5 years after resection. Patient survival was significantly impaired by microscopic lymphatic invasion, regional nodal metastasis, tumor grade, and the epithelium of origin. In a multivariate analysis, only microscopic lymphatic invasion significantly reduced patient survival. Radical resection for ampullary cancer can be performed with a low morbidity and mortality and should remain the procedure of choice for ampullary carcinoma.
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