Granular cell tumour of the bile duct mimicking distal cholangiocarcinoma: one case report and review of the literature

Granular cell tumours (Gcts) are rare and most commonly located in the oral cavity, skin or subcutaneous tissue. The occurrence of this tumour in the biliary tract is rare. A 26 year old African man presented with abdominal pain and obstructive jaundice was found to have a localised distal bile stricture suggestive of cholangiocarcinoma which was resected by pylorus preserving partial pancreaticoduodenectomy. Histology revealed a gct of the intrapancreatic portion of the distal bile duct. GCT of the biliary tract are important as they are benign and can mimic bile duct cancers. A review of the 77 reported cases of Gcts of the biliary tract showed that the common bile duct is the most common site of occurrence (n=35). Only 2 cases in literature have been reported to be diagnosed preoperatively. Since there are no characteristic radiological features, the GCTs are difficult to differentiate from cholangiocarcinoma, sclerosing cholangitis or other benign tumors.     

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.     

Duodenum-Preserving Subtotal and Total Pancreatic Head Resections for Inflammatory and Cystic Neoplastic Lesions of the Pancreas

Introduction For treatment of inflammatory and benign neoplastic lesions of the pancreatic head, a subtotal or total pancreatic head resection is a limited surgical procedure with the impact of replacing the application of a Whipple procedure. The objective of this work is to describe the technical modifications of subtotal and total pancreatic head resection for inflammatory and neoplastic lesions of the pancreas. The advantages of this limited surgical procedure are the preservation of the stomach, the duodenum and the extrahepatic biliary ducts for treatment of benign lesions of the pancreatic head, papilla, and intrapancreatic segment of the common bile duct. For chronic pancreatitis with an inflammatory mass complicated by compression of the common bile duct or causing multiple pancreatic main duct stenoses and dilatations, a subtotal pancreatic head resection results in a long-lasting pain control. Performing, in addition, a biliary anastomosis or a Partington Rochelle type of pancreatic main duct drainage, respectively, is a logic and simple extension of the procedure. The rationale for the application of duodenum-preserving total pancreatic head resection for cystic neoplastic lesions are complete exstirpation of the tumor and, as a consequence, interruption of carcinogenesis of the neoplasia preventing development of pancreatic cancer. Duodenum-preserving total head resection necessitates additional biliary and duodenal anastomoses. For mono-centric IPMN, MCN, and SCA tumors, located in the pancreatic head, total duodenum-preserving pancreatic head resection can be performed without hospital mortality and resurgery for recurrency. Based on controlled clinical trials, duodenum-preserving pancreatic head resection is superior to the Whipple-type resection with regard to lower postoperative morbidity, almost no delay of gastric emptying, preservation of the endocrine function, lower frequency of rehospitalization, early professional rehabilitation, and establishment of a predisease level of quality of life. Conclusion The limited surgical procedures of subtotal or total pancreatic head resection are simple, safe, ensures free tumour margins and replace in the authors institution the application of a Whipple-type head resection.     

Distal biliary malignancy

Distal cholangiocarcinoma (malignancy in the common bile duct from the cystic duct to the ampulla) remains a rare diagnosis. Most of these lesions are adenocarcinomas, and typically present with painless jaundice. If suspected, a high-quality CT scan and endoscopic retrograde cholangiopancreatography are required for diagnosis and staging. In addition, identification of risk factors, use of tumor markers, and advanced molecular testing may enhance diagnostic and prognostic capabilities. The treatment of choice for resectable disease is pancreaticoduodenectomy and the overall 5-year survival for resected distal cholangiocarcinoma remains 20% to 30%.     

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.     

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.     

Malignant papillomatosis of the common bile duct

Summary Bile duct papillomatosis is a rare entity with a high risk of malignant change. We report the case of a 60-year-old man with malignant papillomatosis of the distal common bile duct and a separate carcinoma of ampulla of Vater. The patient had previously undergone surgery for acute cholecystitis and common bile duct calculi. Three months later the patient developed jaundice and fever. An endoscopic retrograde cholangiopancreatography demonstrated multiple filling defects adherent to the wall of the distal common bile duct. Pancreatoduodenectomy was performed with complete resection of the extrahepatic bile duct. Histologic examination showed a multicentric papillary adenocarcinoma of the common bile duct associated with an adenocarcinoma of the ampulla of Vater. The resection margins were free of tumor.     

Laparoscopic resection of a periampullary villous adenoma

Background: Adenomas of the duodenal papilla are rare lesions. Because of their malignant potential, resection is mandatory. Options for resection include endoscopic resection, transduodenal local excision, and pancreaticoduodenectomy. This report details a case of periampullary villous adenoma diagnosed endoscopically and resected laparoscopically via a transduodenal approach. Case report: A healthy 75-year-old woman with heartburn underwent an upper endoscopy for vague right upper abdominal pain. A periampullary tumor was diagnosed. Endoscopic biopsy results were consistent with a villous adenoma, and endoscopic ultrasound showed distal bile duct involvement. The patient underwent laparoscopic transduodenal local excision of the tumor with biliary reconstruction. Conclusions: Laparoscopic transduodenal resection of periampullary lesions provides advantages similar to those of an endoscopic resection by removal of the tumor using minimally invasive techniques. In addition, laparoscopic surgery maintains the surgical tenents of open transduodenal resection with en bloc tumor resection including the adjacent duodenal wall and ductal structures as necessary. As noted in this case, laparoscopic techniques resect ampullary lesions involving the ductal structures as well. Laparoscopic transduodenal ampullectomy is a valuable treatment option for benign and selected premalignant ampullary lesions.     

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).     

Local resection of tumors of the ampulla of Vater

Local resection of an ampullary tumor with reimplantation of the pancreatic and bile ducts was first described by William S. Halsted in 1899. Technical hazard and unsuitability in malignant ampullary tumors have unfortunately led to a disregard for this operation that is unwarranted. Radical pancreaticoduodenectomy is now the most common method of resecting benign and malignant ampullary tumors. Experience was gained with two high-risk patients with benign adenomatous polyps obstructing the ampulla of Vater. Their medical unsuitability for radical pancreaticoduodenectomy led us to revive the procedure of wide local excision of these tumors with reimplantation of the pancreatic and bile ducts. Operative time and blood loss were substantially less than radical resection and postoperative recoveries were relatively uncomplicated. Radical resection of benign ampullary tumors may be appropriate for good-risk patients in whom the risk of local recurrence outweighs the operative risk. We suggest that local resection of benign ampullary tumors is the procedure of choice in high-risk patients and that it be considered in palliation of limited local malignancies of the ampulla in high-risk patients.     

Transduodenal resection of ampullary tumors

Objective: Whether the treatment of benign ampullary tumors should be performed as transduodenal surgical excision or endoscopic ampullectomy depends on the size and spread of the tumor. In this videopaper we report technical hints on the surgical resection.Indications: Surgical resection is indicated for benign ampullary lesions if endoscopic resection is not possible. In addition, local resection can be performed in cases with high risk of malignancy or in a palliative intention.Procedure: The duodenum is mobilized by the Kocher maneuver. It is recommendable to perform a cholecystectomy to introduce a flexible catheter antegrade into the common bile duct through the cystic duct for identification of the papilla of Vater by digital palpation. An anterolateral oblique duodenotomy is made and thereby the tumor of the papilla is exposed, followed by a submucosal injection of epinephrine to elevate the tumor. Afterwards a 5-10 mm margin is scored circumferentially in the mucosa around the adenoma. The extent of the excision is based on the preoperative and intraoperative assessment; a submucosal or full thickness (for transmural lesions) excision can be performed. After submucosal excision the mucosa of the ampulla is approximated to the mucosa of the duodenum. In cases with full thickness ampullectomy the borders of the pancreatic and bile duct are approximated and then the entire complex is sutured to the full wall of the duodenum. Furthermore in some cases with extensive resection a separate reconstruction of the pancreatic and bile duct may be required. A terminal assessment of the ductal patency is imperative. The duodenectomy is closed and a paraduodenal drain is placed.Conclusion: Transduodenal resection of periampullary tumors can be technically demanding, but provides a stage-adapted treatment modality for benign and premalignant lesions of the papilla of Vater.     

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.     

Extensive hilar bile duct resection using a transhepatic approach for patients with hepatic hilar bile duct diseases

BACKGROUND: Extensive hilar bile duct resection beyond the second- or third-order intrahepatic biliary radicals is usually required for patients with hilar cholangiocarcinoma as well as those with benign inflammatory stricture. Most hilar cholangiocarcinoma is resected with combined major hepatectomy to obtain free surgical margins. The purpose of this study was to show the surgical procedure and the usefulness of extensive hilar bile duct resection using a transhepatic approach for patients with hilar bile duct diseases. METHODS: Five patients with hepatic hilar bile duct disease and who were unfit for major hepatectomy for several reasons underwent extensive hilar bile duct resection by way of a transhepatic approach. Four of the patients had hilar bile duct cancer, including 1 with mucous-producing bile duct cancer of low-grade malignancy and 1 with a postsurgical benign bile duct stricture. RESULTS: After extensive hilar bile duct resection, bile duct stumps ranged in number from 3 to 7 mm (mean 4.4). Surgical margins at bile duct stump were free of cancer in all 4 cancer patients. The long-term outcomes were as follows: 3 patients are alive at the time of publication, and 2 patients have died. CONCLUSIONS: A transhepatic approach may be useful when performing extensive hilar bile duct resection bile duct stricture of biliary disease at the hepatic hilus, especially in high-risk patients who are unfit for major hepatectomy as well as in those having benign bile duct stricture and low-grade malignancy.     

Adenofibromyomatous hyperplasia of the extrahepatic bile duct — A report of two cases—

Two patients with adenofibromyomatous hyperplasia located at the distal end of the extrahepatic bile duct are reported herein. The chief symptom in both patients was upper abdominal pain and the lesions found involved the proliferation of submucosal mucous glands and fibrous elements. Further progress in imaging diagnostic techniques would increase the frequency with which such lesions are discovered. But, even now, if mural irregularities and shadow defects are found in the extrahepatic bile duct, particularly in the distal end of the common bile duct, the possibility of a benign lesion such as adenofibromyomatous hyperpasia should be taken into consideration when making a diagnosis.     

Resective operations for biliary carcinoma

Records of 25 consecutive patients who underwent resection for proximal bile duct tumor (3 extended right hepatic lobectomies, 6 left hepatic lobectomies, 16 skeletonization resections) and records of 21 patients who underwent pancreatoduodenectomy for distal bile duct carcinoma were reviewed to assess the value of resective therapy. The operative mortality rate for patients with resected proximal bile duct tumor was 4 per cent (0 per cent for liver resection) and that of distal bile duct tumor, 4.6 per cent. The 3- and 5-year actuarial survival rates for patients with proximal bile duct tumor were 44 per cent and 35 per cent, respectively; all except one patient eventually died of disease. Survival was better for patients who had curative resection (margins microscopically free of tumor). The 5-year actuarial survival rate for patients with distal bile duct carcinoma was 58±12 (SE) per cent, with patients who had negative nodes surviving longer than patients with positive nodes. When major hepatic resection and pancreatoduodenectomy can be performed in selected patients with low operative mortality, patients with bile duct carcinoma should be assessed by an experienced hepatobiliary multidisciplinary group before a decision is made in favor of palliative, endoscopic, or percutaneous techniques because surgical resection appears to offer the best possible long-term survival and probably the best quality of palliation. This report is the basis of a paper read by R.L.R. at the 90th Annual Meeting of the Japanese Surgical Society, Sapporo, Japan, 1990     

Adenofibromyomatous hyperplasia of the extrahepatic bile duct--a report of two cases

Two patients with adenofibromyomatous hyperplasia located at the distal end of the extrahepatic bile duct are reported herein. The chief symptom in both patients was upper abdominal pain and the lesions found involved the proliferation of submucosal mucous glands and fibrous elements. Further progress in imaging diagnostic techniques would increase the frequency with which such lesions are discovered. But, even now, if mural irregularities and shadow defects are found in the extrahepatic bile duct, particularly in the distal end of the common bile duct, the possibility of a benign lesion such as adenofibromyomatous hyperplasia should be taken into consideration when making a diagnosis.     

Carcinoma of the distal and middle bile duct: surgical results, prognostic factors, and long-term follow-up

BACKGROUND/PURPOSE: Carcinoma of the distal bile duct is associated with poor prognosis. Surgical resection remains the only potentially curative treatment. We conducted a retrospective study to identify prognostic factors determining longterm survival. METHODS: From 1990 to 2006, 95 patients with distal and/or middle bile duct carcinoma had resections. Fifty-four patients underwent pylorus-preserving pancreaticoduodenectomy (57%) and 41 patients underwent standard Kausch-Whipple pancreaticoduodenectomy (43%). Nine patients underwent pancreaticoduodenectomy including portal vein resection (9%). RESULTS: Overall 1-, 3-, and 5-year survival rates were 60%, 36%, and 29%, respectively. Five-year survival after R0 resection was 34%, and after R1 resection it was 0%. Four patients died during their hospital stay (4%). Multivariate analysis showed negative resection margins (P = 0.040), lymphatic vessel invasion (P = 0.036), and portal vein infiltration (P = 0.027) as strong predictors for survival, whereas the location of the tumor (distal bile duct vs middle bile duct) and lymph node status were not identified as independent prognostic factors. CONCLUSIONS: Five-year survival depends strongly on negative resection margins, independent of nodal status. Portal vein resections in patients with portal vein involvement fail to ameliorate long-term survival. Primary tumor site--middle bile duct or distal bile duct--did not determine prognosis.     

Implications of histological grade of tumour for the prognosis of radically resected periampullary adenocarcinoma.

OBJECTIVE: To study the influence of histological grade of tumour on the prognosis of radically resected periampullary cancers. DESIGN: Retrospective study. SETTING: Teaching hospital, Austria. SUBJECTS: 156 patients (papilla of Vater, n = 34, head of the pancreas, n = 105, and distal common bile duct, n = 17) who underwent partial pancreaticoduodenectomy for periampullary adenocarcinoma between 1 January, 1967 and 31 December, 1996. OUTCOME MEASURES: The relation between grade of tumour and site, T and N classification, extramural growth, invasion of vessels and resection margins, tumour volume, and survival time. RESULTS: Well differentiated lesions were significantly more common in the papilla of Vater (n = 15, 44%, p = 0.01) than in the pancreatic head or the common bile duct (n = 20, 19%, and n = 5, 29%, respectively). Only in ampullary lesions did the grade of tumour significantly affect the incidence of other histopathological risk factors (T p = 0.003; nodal status p = 0.01; extramural growth p = 0.0001; tumour volume p = 0.02) and survival time (p = 0.02); no significant correlations were found in cancers of the head of the pancreas or common bile duct. CONCLUSIONS: There was a significant difference in the distribution of grade of tumour between the different sites of origin of resected periampullary cancers. Grade of tumour correlated with T and N classification, tumour volume, extramural growth, and survival only in ampullary lesions.     

Periampullary adenocarcinoma: analysis of 5-year survivors.

OBJECTIVE: This single-institution experience retrospectively reviews the outcomes in a group of patients treated 5 or more years ago by pancreaticoduodenectomy for periampullary adenocarcinoma. SUMMARY BACKGROUND DATA: Controversy exists regarding the benefit of resection for periampullary adenocarcinoma, particularly for pancreatic tumors. Many series report only Kaplan-Meier actuarial 5-year survival rates. There are believed to be discrepancies between the actuarial 5-year survival data and the actual 5-year survival rates. METHODS: From April 1970 through May 1992, 242 patients underwent pancreaticoduodenal resection for periampullary adenocarcinoma at The Johns Hopkins Hospital. Follow-up was complete through May 1997. All pathology specimens were reviewed and categorized. Actual 5-year survival rates were calculated. The demographic, intraoperative, pathologic, and postoperative features of patients surviving > or =5 years were compared with those of patients who survived <5 years. RESULTS: Of the 242 patients with resected periampullary adenocarcinoma, 149 (62%) were pancreatic primaries, 46 (19%) arose in the ampulla, 30 (12%) were distal bile duct cancers, and 17 (7%) were duodenal cancers. There was a 5.3% operative mortality rate during the 22 years of the review, with a 2% operative mortality rate in the last 100 patients. There were 58 5-year survivors, 28 7-year survivors, and 7 10-year survivors. The tumor-specific 5-year actual survival rates were pancreatic 15%, ampullary 39%, distal bile duct 27%, and duodenal 59%. When compared with patients who did not survive 5 years, the 5-year survivors had a significantly higher percentage of well-differentiated tumors (14% vs. 4%; p = 0.02) and higher incidences of negative resection margins (98% vs. 73%, p < 0.0001) and negative nodal status (62% vs. 31%, p < 0.0001). The tumor-specific 10-year actuarial survival rates were pancreatic 5%, ampullary 25%, distal bile duct 21%, and duodenal 59%. CONCLUSIONS: Among patients with periampullary adenocarcinoma treated by pancreaticoduodenectomy, those with duodenal adenocarcinoma are most likely to survive long term. Five-year survival is less likely for patients with ampullary, distal bile duct, and pancreatic primaries, in declining order. Resection margin status, resected lymph node status, and degree of tumor differentiation also significantly influence long-term outcome. Particularly for patients with pancreatic adenocarcinoma, 5-year survival is not equated with cure, because many patients die of recurrent disease >5 years after resection.