Recurrent disease after microscopically radical (r0) resection of periampullary adenocarcinoma in patients without adjuvant therapy

The survival rate after microscopically radical resection of pancreatic duct adenocarcinoma is still poor. Patients with ampulla of Vater and distal common bile duct adenocarcinoma indicate a much more favorable prognosis. Controversy exists as to whether adjuvant therapy could improve the outcome in these patients after resection. The aim of the present study was to analyze the pattern of recurrence in patients with periampullary adenocarcinoma after pancreatoduodenectomy. Between January 1992 and December 2002, all patients with an R0 resection were identified and used for this analysis. A total of 190 patients underwent a microscopically radical resection and received no adjuvant therapy. Of those, 72 patients were diagnosed with pancreatic duct adenocarcinoma, 86 patients were diagnosed with ampulla of Vater adenocarcinoma, and 31 patients were diagnosed with distal common bile duct adenocarcinoma. Recurrent disease was indicated in 81% of the patients with pancreatic duct adenocarcinoma, 50% of the patients with ampulla of Vater adenocarcinoma, and in 74% of the patients with bile duct adenocarcinoma. Multivariate analysis revealed that lymph node metastases were prognostic for recurrent disease in patients with pancreatic duct adenocarcinoma (P = 0.038). The depth of invasion (T4, P < 0.032) and lymph node metastases (P < 0.001) were prognostic in patients with ampulla of Vater adenocarcinoma. Poor tumor differentiation (P < 0.001) was prognostic in patients with distal bile duct adenocarcinoma. Selected patients with periampullary malignancies exhibited a high recurrence rate and should be encouraged to enroll in clinical trials for adjuvant treatment including local therapy (radiotherapy) according to the identified prognostic factors. Presented at the Forty-Fifth Annual Meeting of the Society for Surgery of the Alimentary Tract, New Orleans, Louisiana, May 15–19, 2004 (oral presentation).     

Results of surgical treatment of periampullary tumors: a thirty-five-year experience

Data on 126 consecutive patients with periampullary tumors resected at the Cleveland Clinic between January 1950 and December 1984 were reviewed. One hundred five patients underwent pancreatoduodenal resection, 10 patients total pancreatectomy, and 11 patients local resection of the tumor. The site of tumor was ampulla of Vater (59), head of the pancreas (30), duodenum (20), and distal common bile duct (11). Six patients had benign disease. The operative mortality rate for radical resection for the entire period was 7.8%; it has declined to 5.4% since 1974. The operative mortality rate for local resection was 9.1% (one patient). The overall 5-year survival rate for all malignant tumors of the periampullary area was 28% and 25.5% for invasive adenocarcinoma. Survival was affected primarily by location and histologic findings. The 5-year survival rate for adenocarcinoma of the ampulla of Vater was 37.2%, 27.5% for the duodenum, 16.7% for the distal common bile, and 4.3% for the pancreas (p = 0.0001). Papillary adenocarcinoma had a 5-year survival rate of 49.2% in contrast to 18.4% for nonpapillary ductal adenocarcinoma (p = 0.002). Patients with ampullary adenocarcinoma treated by local resection had a 5-year survival rate of 40.9%. These data justify continued use of a selective radical approach in the resection of most periampullary tumors with local resection for small tumors in high-risk patients.     

保留十二指肠的胰头切除术实用外科血管解剖学研究

目的研究胰头和十二指肠之间的血管解剖 ,为临床上开展关于十二指肠、胰头以及胆总管末端手术提供解剖学基础。方法对 30例甲醛固定的成人尸体和 10例新鲜尸体的十二指肠、胆总管下段及Vater壶腹的血液供应进行解剖研究。结果十二指肠降部和水平部血运由胰十二指肠前、后动脉弓及分支供应 ;胆总管下段血运主要是由胰十二指肠上后动脉供应 ;Vater壶腹血运主要是由胰十二指肠上后动脉发出的乳头动脉供应。结论胰十二指肠前、后动脉弓是供应十二指肠降部和水平部、胆总管下段和Vater壶腹的主要动脉。在行保留十二指肠的胰头切除术时应注意保护前、后动脉弓。     

Heterotopic pancreas: A rare cause of bile duct dilatation —Report of a case and review of the literature—

A case of a 77 year old woman with a heterotopic pancreas in the distal common bile duct is reported herein. The patient had no symptoms, but an ultrasound examination showed bile duct dilatation and subsequent endoscopic retrograde cholangiography demonstrated a spherical filling defect in the distal common bile duct. Under suspicion of a benign neoplasm in the common bile duct, resection of the common bile duct and hepaticojejunostomy using a Roux-en Y jejunal limb were successfully performed. Pathological examination revealed heterotopic pancreatic tissue in the distal common bile duct. This is only the ninth reported case of heterotopic pancreas occurring in the common bile duct or ampulla of Vater, and thus, a review of the literature is also given.     

Heterotopic pancreas: a rare cause of bile duct dilatation--report of a case and review of the literature

A case of a 77 year old woman with a heterotopic pancreas in the distal common bile duct is reported herein. The patient had no symptoms, but an ultrasound examination showed bile duct dilatation and subsequent endoscopic retrograde cholangiography demonstrated a spherical filling defect in the distal common bile duct. Under suspicion of a benign neoplasm in the common bile duct, resection of the common bile duct and hepaticojejunostomy using a Roux-en Y jejunal limb were successfully performed. Pathological examination revealed heterotopic pancreatic tissue in the distal common bile duct. This is only the ninth reported case of heterotopic pancreas occurring in the common bile duct or ampulla of Vater, and thus, a review of the literature is also given.     

胆胰十二指肠结合部切除治疗壶腹部周围癌的疗效分析

目的探讨胆胰十二指肠结合部切除治疗壶腹部周围癌的临床应用。方法自2005年1月—2006年7月采用该术式治疗壶腹部周围癌15例，其中乳头癌6例，壶腹癌5例，胆总管下端癌4例。合并心肺疾患7例，糖尿病2例。切除范围：十二指肠降段，距胆胰管汇合部切除胰头1—2cm及胆总管至左右肝管汇合处下方。术中注意清扫区域淋巴结，行冰冻病理切片检查证实各切缘无肿瘤残存。结果全组手术成功率为100％，术后未出现胃瘫、十二指肠漏、胆漏或明显胰漏等严重并发症，均痊愈出院。1例死于术后2个月上消化道应激性溃疡大出血，其余14例均存活。随访3—16个月，随访期间未发现肿瘤复发或转移。结论胆胰十二指肠结合部切除术是治疗壶腹部周围癌的一种新术式，比乳头局部切除术范围大，但是手术难度和创伤均较常规胰十二指肠切除术明显降低，近期疗效满意。     

Extrahepatische distale Gallenwegskarzinome

Malignancies of the biliary tree are classified into three groups according to location: intrahepatic, central (perihilar), and distal. Of all cholangiocarcinomas, 25% are located distally and can be subdivided into middle and lower bile duct carcinomas. Surgical approaches for achieving tumor-free resection margins (R0) are directly associated with the origin of the tumor. Intrahepatic and central cancers usually must be treated by liver surgery, whereas the majority of distal cholangiocarcinomas require pancreaticoduodenectomy. In case of a small, middle bile duct carcinoma, exclusive extrahepatic bile duct resection without pancreatic resection can be adequate. Five-year survival after radical resection is about 25%. Cancer of the distal bile duct has to be distinguished from ductal adenocarcinoma of the pancreas and carcinoma of the ampulla of Vater. Curative surgery is possible if the tumor is diagnosed early and radical resection is feasible. In this context, the role of an extended lymph node dissection remains unclear. To improve survival, future studies are needed to evaluate the role of novel adjuvant strategies (i.e., gemcitabine, capecitabine).     

Simplified pancreatoduodenectomy for complex blunt pancreaticoduodenal injury

A 34-year-old man admitted to our de- partment with complex blunt pancreaticoduodenal injury af- ter a car accident. The wall of the first, second, and third portions of the duodenum was extensively lacerated, and the pancreas was longitudinally transected along the supe- rior mesenteric vein-portal vein trunk. The pancreatic head and the uncinate process were devitalized and the distal common bile duct and the proximal main pancreatic duct were completely detached from the Vater ampulla. The length of the stump of distal common bile located at the cut surface of remnant pancreas was approximately 0.6 cm. A simplified Kausch-Whipple＇s procedure was performed after debride- ment of the devitalized pancreatic head and resection of the damaged duodenum in which the stump of distal common bile duct and the pancreatic remnant were embedded into the jejunal loop. Postoperative wound abscess appeared that eventually recovered by conservative treatment. Dur- ing 16 months follow-up the patient has been stable and healthy. A simplified pancreaticoduodenectomy is a safe alternative for the Whipple procedure in managing complex pancreaticoduodenal injury in a hemodynamically stable patient.     

Collision Tumour of the Ampulla of Vater: Carcinoid and Adenocarcinoma

Obstructive jaundice is most commonly due to luminal stones or lesions of the head of the pancreas and more rarely ampullary and primary common bile duct lesions. Obstruction due to lesions of the ampulla of Vater may be due to adenocarcinoma which has a significantly better long term prognosis than carcinomas located in the head of the pancreas. A case is presented where two tumours were identified at the ampulla of Vater of the resected specimen one an adenocarcinoma and the other a carcinoid tumour representing a collision tumour.     

Choledochocele: Report of a case and review of the literatures

A case of choledochocele seen in a 68-year-old male was reported. Analysis of fourteen cases reported under a variety of designations featuring cystic dilatation of the distal common bile duct protruding into the duodenal lumen and the case presented here disclosed that these cases could be classified into two types of pathology; both of the bile duct and the pancreatic duct opened into the cyst of the ampulla in 6 cases, whereas the bile duct and the pancreatic duct joined at the papilla of Vater and distal end of the bile duct formed a cystic dilatation protruding into the duodenal lumen in the remaining 9 cases.     

Mirizzi syndrome secondary to an adenoma of the cystic duct

Bile duct adenomas are uncommon lesions that can cause obstructive jaundice. We report the unusual case of a 54-year-old man who developed Mirizzi syndrome secondary to a bile duct papillary adenoma located in the cystic duct remnant. A case report is presented, together with a review of extrahepatic bile duct adenomas published in the English-language literature, with special attention directed toward the clinical manifestations, locations, and prognosis of these tumors. Bile duct adenomas are very rare tumors. Although cholangiography can detect many of these lesions, few cases were correctly diagnosed preoperatively. Most lesions were located in the distal common bile duct or at the ampulla of Vater. Pathologic examination often revealed foci of carcinoma in situ, dysplasia, or atypia. Local resection was performed in most cases. There were no previous case reports of extrinsic common bile duct obstruction caused by tumors within the cystic duct. We describe here a very rare, acalculous variant of Mirizzi syndrome secondary to a solitary papillary adenoma of the cystic duct. In general, bile duct adenomas are uncommon lesions that are difficult to diagnoses preoperatively. These tumors usually present with jaundice secondary to intraluminal biliary obstruction. These lesions are premalignant and should be managed by complete surgical resection.     

Resection of the intrapancreatic bile duct preserving the pancreas

Benign neoplasms of the distal bile duct are rare, but pose a therapeutic challenge. Usually, these lesions are resected by means of ampullectomy if located in close proximity to the ampulla of Vateri or by partial pancreaticoduodenectomy if located intrapancreatic and distant from the ampulla. Here, we present a case of an intrapancreatic benign neuroendocrine tumor that was resected by performing a pancreas-preserving distal bile duct resection. First, a duodenotomy was carried out and a probe was inserted into the pancreatic duct to avoid inadvertent injury. Subsequently, the bile duct was divided proximal the lesion and dissected towards the ampulla. Pancreatic parenchyma was dissected dorsally and closed using absorbable interrupted sutures. The duodenal incision was closed, and reconstruction was performed by an end-to-side hepaticojejunostomy and a Roux-Y jejunojejunostomy. The postoperative course of the patient was uneventful. In conclusion, pancreas-preserving distal bile duct resection might be an option for intrapancreatic benign lesions of the distal bile duct that would otherwise require a partial pancreaticoduodenectomy.     

Cancer of the ampulla of vater, bile duct, and duodenum

The presenting features of periampullary cancer have been reviewed. Unless the neoplasm is discovered as an incidental finding at surgery, diagnosis is not made until the onset of symptoms (pain, jaundice, weight loss), which is not early, even though these tumors are close to the bile duct or ampulla of Vater. Individual philosophies aside, the prognosis for ampullary, duodenal, or distal bile duct tumors reflects the grade and stage of tumor as much as the operation done. Pancreatoduodenal resection, standard or modified, should be done with an attempt to cure and not to palliate. Statistics that show superior results after resection compared with palliative procedures reflect earlier stages lesions, healthier patients, and, most importantly, patient selection.     

Report of two cases of large cell neuroendocrine carcinoma of duodenal ampulla with contrasting outcomes following pancreaticoduodenectomy according to the use of adjuvant chemotherapy

IntroductionLarge-cell neuroendocrine carcinoma (LCNEC) in the duodenal ampulla of Vater is a rare malignant tumor, with frequent postoperative recurrence and poor prognosis even following complete resection. Effective adjuvant chemotherapy is expected to offer longer survival.Presentation of caseWe present two patients with LCNEC accompanied by components of tubular adenocarcinoma/adenoma in the duodenal ampulla of Vater who underwent pancreaticoduodenectomy (PD), resulting in longer survival of 1 patient. The first patient was an 81-year-old man in whom a 14-mm protruding solid tumor of the ampulla was observed. Pylorus-preserving PD (PPPD) was performed for the diagnosis of adenocarcinoma of the ampulla, and the final histological diagnosis of the resected specimen was LCNEC with an adenoma component. The patient showed a liver metastasis 4 months after surgery and died of carcinoma after 11 months. The second patient was a 72-year-old man with a 24-mm ulcerative solid tumor of the ampulla. PPPD was also performed in this patient, and the final histological diagnosis was LCNEC with mixed adenocarcinoma component (21%). Adjuvant chemotherapy of cisplatin and etoposide was administered, and the patient survived without tumor relapse for 24 months after surgery.ConclusionIn the surgical treatment of LCNEC of the ampulla showing malignant behaviour, an accurate preoperative diagnosis and effective adjuvant chemotherapy after curative resection are necessary for longer survival.     

Carcinoid tumors of the extrahepatic biliary tract: report of four cases

Carcinoid tumors located in the biliary tree are exceedingly rare, accounting for 0.2%–2% of all gastrointestinal carcinoids. This study describes four cases of carcinoid tumors of the extrahepatic biliary tract. Four carcinoid tumors arising in the common bile duct (Case 1, 59-year-old man), gallbladder (Case 2, 49-year-old man; Case 3, 65-year-old man), and ampulla of Vater (Case 4, 52-year-old woman) were studied. All of the cases were misdiagnosed before surgery as proximal bile duct cancer, stomach cancer with liver metastasis, gallbladder cancer, and adenocarcinoma of ampulla of Vater, respectively. The clinicopathological characteristics and clinical course were reviewed. Treatment depends on the location of the tumor and the extent of the disease. Aggressive surgical therapy with a curative intention therefore offers the only chance for cure and has to be considered whenever possible.     

经胆总管刮取组织法诊断壶腹癌（附13例报告）

经胆总管刮取组织法诊断壶腹癌13例,11例刮取组织中腺癌9例,腺瘤伴高度不典型增生、原位癌1例,绒毛乳头状瘤样增生1例。2例疑壶腹癌者刮取未得组织。常规胆总管探查术中适时应用此法有助于诊断未经注意的壶腹癌。在疑壶腹癌病例中采用本法诊断时多能得到阳性结果,但对刮取未得组织者应改用其它方法明确诊断。刮取组织病检诊为腺癌或绒毛肿瘤时有可能遗漏隐藏的恶性病变,故仍应将其完全切除病检。     

Papillomatosis Confined to the Distal Biliary Tract — A Rare Cause of Obstructive Jaundice: Report of a Case

Based on the findings of a patient with recurrent obstructive jaundice due to papillomatosis of the distal bile duct, we herein describe the diagnostic difficulties and therapeutic options in this very rare disease. Endoscopic retrograde cholangiopancreatography and, in particular, cholangioscopy are the imaging procedures of choice if biliary papillomatosis is suspected. Due to the tendency of such patients to demonstrate malignant transformation and develop biliary cirrhosis with septic complications, an early and radical surgical resection is recommended in rare cases of localized papillomatosis. This approach may offer the only chance of a cure although the potential risk of multifocal recurrence cannot be ruled out. When considering a radical resection, intraoperative cholangioscopy is strongly recommended to confirm any localized papillomatosis and rule out any diffuse papillomatosis of the entire biliary tract.     

Tumors of the ampulla of Vater--local or radical resection?

Duodenal ampulla is a complex anatomical and histological site and a tumor may arise from one of three types of epithelium: duodenal mucosa, pancreatic duct and distal common bile duct. Neoplasia from each of these locations may exhibit different growth models and though it is difficult to establish their origin before operation, surgical treatment for all resectable tumors is essentially alike. The outcome of these tumors is vitally influenced by the type of epithelium from which they derive. Benign adenomas appear to be a frequent precursor of carcinoma of the ampulla of Vater, therefore, a local resection can lead to an under treated early cancer which would have benefited from a radical excision, with a much better long term result. We consider acceptable to perform an ampullectomy whenever is possible to safely state the benignity of the tumor or when a major procedure is hazardous.     

Surgical management of neoplasms of the ampulla of Vater: local resection or pancreatoduodenectomy and prognostic factors for survival

BACKGROUND: Neoplasms of the ampulla of Vater have a better 5-year survival than pancreatic and bile duct neoplasms after resection. This study was performed to analyze the outcome after local resection and pancreatoduodenectomy (PD) and to identify predictive factors for survival. METHODS: We used a prospective database to evaluate 145 patients (1992-2002) with a neoplasm of the ampulla of Vater. RESULTS: The median age of the entire cohort was 66 years. Agreement between preoperative biopsies and definite resection specimen was 58% for invasive adenocarcinoma. Local resection was performed in 25 patients, but the operation was adequate therapy in only 16 patients (64%). Subsequent PD (n=9) was performed in the remaining patients because of an R1 resection. Other patients (n=120) underwent an elective PD. Hospital mortality was 4.0% (1/25) after local resection and 5.0% (6/120) after PD. Multivariate analysis revealed that advanced invasion and nodal status were independent predictive factors for survival. The overall 5-year actuarial survival of patients with adenocarcinoma after PD was 37%. CONCLUSIONS: Preoperative biopsies have a poor diagnostic accuracy. Local resection is an adequate surgical treatment for adenomas. In experienced hands, PD is the preferred treatment for patients with adenocarcinoma.     

Villous tumor of the papilla of Vater presenting with hypopotassemia

We report a case of villous tumor of the papilla of Vater associated with hypopotassemia. The patient was a 73-year-old woman who presented with jaundice and fever. She had a history of diabetes mellitus and liver dysfunction. Laboratory studies revealed that levels of total bilirubin, alkaline phosphatase, and C-reactive protein, and the white blood cell count were elevated (suggestive of cholangitis) and that the serum potassium level was markedly reduced, to 1.9 mEq/l (normal value 3.5–5.0 mEq/l). Duodenoscopy showed a villous tumor arising in the papilla of Vater. Percutaneous transhepatic biliary drainage was performed. Approximately 700–1500 ml of bile with viscous mucoid fluid was drained daily. Percutaneous transhepatic cholangioscopy showed a papillary lesion in the distal common bile duct. Biopsied specimens from both percutaneous transhepatic cholangioscopy and duodenoscopy disclosed tubulovillous adenoma. Endoscopic ultrasonography showed that the tumor had spread to the main pancreatic duct as well as to the common bile duct. The patient underwent pylorus-preserving pancreaticoduodenectomy. Pathology examination disclosed well differentiated adenocarcinoma, carcinoma in situ, in tubulovillous adenoma. The cancer cells were observed at the bottom of the tumor spreading in the common bile duct. This is a rare case of a patient presenting with hypopotassemia associated with a tubulovillous tumor of the papilla of Vater that secreted mucoid material.