A Comprehensive Approach to the Management of Benign and Malignant Ampullary Lesions: Management in Hereditary and Sporadic Settings.

PURPOSE OF REVIEW: The purpose of this review was to examine the historical roots of endoscopic management of ampullary lesions and explore emerging data on improved techniques, technologies, and outcomes. Of specific interest was answering whether there exists a reasonable body of data to support one resection technique or strategy above others. RECENT FINDINGS: Review of recent literature suggests the continued use of endoscopic ampullectomy is a safe and effective means of curative treatment of ampullary adenomas. Complications are relatively infrequent and complete endoscopic resection is possible in a majority of cases, with proper patient and lesion selection. Greater than 2 decades of experience with endoscopic ampullectomy have shown this to be a viable, well-tolerated, and highly effective means of treating ampullary adenomas. While few concrete guidelines exist to advise endoscopists on the ideal technique for resection, experience, patient selection, and prior planning can greatly influence the technical and clinical success of endoscopic ampullectomy.

Magnetic Resonance Imaging Findings of Intrahepatic Bile Duct Adenoma: A Report of 4 Cases.

Intrahepatic bile duct adenoma (BDA) is a rare type of benign hepatic lesions. In this study, 4 cases of BDA diagnosed from surgical resection pathology were examined. Their clinical and magnetic resonance imaging (MRI) data were retrospectively analyzed. The 4 cases (1 men and 3 women) were aged 21 to 55 years without obvious clinical symptoms. Three were identified through routine examination. Three had a history of chronic hepatitis B virus infection. Two cases were accompanied by hepatocellular carcinoma, and one had a higher level of α-fetoprotein. The MRI images of BDA all manifested as peripheral hepatic nodules with abnormal signals. The diameters of the lesions in the 4 cases were 7.7 to 17.0 mm. The MRI images showed slight hypointensity on T1WI and slight hyperintensity on T2WI in all cases, and they showed slight hyperintensity in 2 cases and hyperintensity in 2 cases on diffusion-weighted imaging. Dynamic contrast-enhanced MRI scans show hyperintensity in the arterial phase and slight hyperintensity in the late stage in 3 cases. The other case shows hyperintensity in the arterial and portal phases and isointensity at the delayed phase. During follow-up, 3 cases were recurrence-free. The other case was complicated by the reoccurrence of HCC. In general, BDA shows specific MRI characteristics, and peripheral hepatic nodules show slight hypointensity on T1WI and slight hyperintensity on T2WI. Dynamic contrast-enhanced MRI scans showed obvious enhancement in the arterial phase and continuous enhancement at the late stage.

Bile duct adenoma: a case report and literature review.

BACKGROUND: Bile duct adenoma (BDA) is a comparatively rare disease clinically, therefore, there are relatively few reported cases about it both in China and abroad. CASE PRESENTATION: Herein, we present a 51-year-old man, diagnosed preoperatively with enhanced-contrast abdominal computed tomography, as having a nodule in the left hepatic. The patient underwent a liver tumor resection, and the histological examination revealed bile duct adenoma (BDA). CONCLUSIONS: BDA is an extremely rare benign tumor, which is difficult to distinguish BDA from hepatocellular carcinoma definitely preoperatively, surgical resection is needed as a way of treatment.

Intraductal papillary neoplasm arising from peribiliary glands connecting with the inferior branch of the bile duct of the anterior segment of the liver.

Intraductal papillary neoplasms of the bile duct are generally thought to arise from neoplastic papillary proliferation of epithelial cells lining the bile duct. We herein report a case with findings that strongly suggested that the biliary cystic tumor might have derived from a peribiliary gland. A 69-year-old female was found to have a cystic lesion with intracystic protrusions at the anterior segment of the right hepatic lobe and underwent hepatic anterior segment resection. Fluoroscopy of the resected specimen injected with contrast medium into the cyst revealed a connection between the cystic lesion and the bile ducts. The cyst was multilocular in appearance. On microscopic examination, the cyst was located within the portal tract of the inferior branch of the anterior segment and connected with the inferior branch of the bile duct. The wall of the hepatic cyst lacked an ovarian-like stroma. The tumor was composed of papillary and glandular components, and the tumor cells were similar to gastric foveolar and pyloric gland epithelia and regarded as adenoma. These tumor cells were positive for MUC 5AC, MUC6, and HIK1083. The tumor was finally diagnosed as an intraductal papillary neoplasm of the bile duct (adenoma, gastric type) arising from a peribiliary gland.

Imaging features of bile duct adenoma: case series and review of literature.

PURPOSE: We aimed to evaluate the imaging features of bile duct adenoma (BDA) on ultrasonography (US), computed tomography (CT), and magnetic resonance imaging (MRI). METHODS: Retrospective search in our institution database was performed for histologically confirmed BDA. Their imaging studies before histologic confirmation were reviewed. The search identified seven adults (mean age, 52.9 years) with histologically proven single BDA each. US (n=3), CT (n=5), and MRI (n=3) were performed before histologic confirmation. Additionally, a systematic English literature review for BDA and reported imaging findings since 2000 was also conducted using the following search criteria "bile duct adenoma, peribiliary hamartoma, biliary adenoma, CT, ultrasound, MRI" (date range: 01/01/2000 through 08/31/2016). The imaging findings of those cases reported were summarized and compared with our series. RESULTS: All seven individual nodules were well circumscribed. Five lesions were located in the right hepatic lobe and two in the left hepatic lobe. On US, lesions appeared hypoechoic (n=2) and hyperechoic (n=1). BDA was hypodense on unenhanced CT images (n=1). On MRI, BDA were hypointense on T1 (n=3), hyperintense on T2 (n=3), and hyperintense on diffusion-weighted images (n=2). On contrast-enhanced CT and MRI, BDAs showed arterial phase hyperenhancement that persisted on portal venous/delayed phase images. CONCLUSION: BDA demonstrates characteristic arterial phase hyperenhancement that persisted into the portal venous and delayed phases on CT and MRI, which may be useful in differentiating from other hepatic lesions.

Relapsing acute pancreatitis due to ampullary adenoma in a patient with familial adenomatous polyposis.

A patient with familial adenomatous polyposis (FAP) presented with a relapsing attack of acute pancreatitis. Evaluation using computed tomography, ultrasonography, and duodenoscopy revealed an ampullary adenoma, which was classified as Spigelman's stage III according to Spigelman's criteria. The patient underwent a pylorus-resected pancreatoduodenectomy, and has had no abdominal pain suggesting acute pancreatitis for 1 year after surgery. Only a few reports of acute pancreatitis due to ampullary neoplasms in patients with FAP are available. Relapsing acute pancreatitis is another surgical indication for premalignant periampullary neoplasms in FAP.

Pancreatitis complicating mucin-hypersecreting common bile duct adenoma.

Villous adenomas of the bile ducts are extremely uncommon. We describe a 58-year-old man presenting with clinical signs and laboratory findings of acute pancreatitis and obstructive jaundice. Preoperative investigation demonstrated a dilated papillary orifice with mucus exiting (fish-mouth sign) and a filling defect in the distal common bile duct. He underwent a modified Whipple operation and histological examination of the surgical specimen showed villous adenoma with rich secretion of mucus.

Magnetic resonance findings of bile duct adenoma with calcification.

Computed tomographic (CT) and magnetic resonance (MR) appearances of bile duct adenoma (BDA in a patient who underwent partial hepatectomy of segment 8 are presented. BDA showed a ring-shaped hyperdense area suggesting calcification and ring enhancement on CT. It appeared hypointense on both T1- and T2-weighted MR images. Preoperatively, the diagnoses considered included metastatic carcinoma, cholangiocarcinoma, tuberculosis, and rare tumors such as epithelioid hemangioendothelioma. The radiological findings were confirmed by pathological investigation.

Accuracy of computerized tomography in determining hepatic tumor size in patients receiving liver transplantation or resection.

Computerized tomography (CT) of liver is used in oncologic practice for staging tumors, evaluating response to treatment, and screening patients for hepatic resection. Because of the impact of CT liver scan on major treatment decisions, it is important to assess its accuracy. Patients undergoing liver transplantation or resection provide a unique opportunity to test the accuracy of hepatic-imaging techniques by comparison of findings of preoperative CT scan with those at gross pathologic examination of resected specimens. Forty-one patients who had partial hepatic resection (34 patients) or liver transplantation (eight patients) for malignant (30 patients) or benign (11 patients) tumors were evaluable. Eight (47%) of 17 patients with primary malignant liver tumors, four (31%) of 13 patients with metastatic liver tumors, and two (20%) of 10 patients with benign liver tumors had tumor nodules in resected specimens that were not apparent on preoperative CT studies. These nodules varied in size from 0.1 to 1.6 cm. While 11 of 14 of these nodules were less than 1.0 cm, three of 14 were greater than 1.0 cm. These results suggest that conventional CT alone may be insufficient to accurately determine the presence or absence of liver metastases, extent of liver involvement, or response of hepatic metastases to treatment.

The role of radiotherapy in the treatment of bile duct carcinoma.

Forty-two patients with irresectable bile duct carcinoma (n = 31) or with microscopic evidence of tumor rest after aggressive surgery for bile duct carcinoma (n = 11) were given radiotherapy consisting intentionally of external-beam therapy and intraluminal 192Iridium (192Ir) wire application(s) following bile drainage procedures. The treatment was well tolerated; complications were mainly infectious and related to the success of the drainage. A median survival of 10 months was achieved for the group as a whole. Patients treated following microscopically incomplete resection survived longer than patients with an irresectable tumor (15 vs 8 months median survival, p = 0.06). Gross lymph node involvement also proved to be a prognostic factor.

Liver transplantation for primary hepatic cancer.

Although early survival following transplantation for primary hepatic cancer is excellent, previously reported high recurrence rates have generally discouraged liver replacement for this indication. Since the inception of the Boston Center for Liver Transplantation (BCLT) in 1983, 33 of 383 (8.6%) liver allograft recipients have undergone orthotopic transplantation as definitive treatment for otherwise unresectable cancer. Diagnoses included hepatocellular carcinoma (HCCA) in 24 patients (73%), and cholangiocarcinoma (CHCA) in 9 patients (27%). Actuarial survival rates for patients with hepatocellular carcinoma were 71%, 56%, and 42% at 1, 2, and 3 years, respectively. The actuarial survival rates for patients with cholangiocarcinoma were 89% at 6 months, and 56% at 1, 2, and 3 years. Of the nine patients with cholangiocarcinoma, 56% (5/9) developed recurrent disease. Although this recurrence rate is disheartening, because of the lack of other morbidity, long-term survival in these patients is comparable to patients with HCCA. In contrast, recurrent hepatocellular carcinoma developed in 25% of recipients (5/20) who survived longer than 3 months posttransplantation. Other causes of death in patients with hepatocellular carcinoma included perioperative complications, 16.6% (4/24); sepsis, 8.3% (2/24); coronary artery disease, 4.2% (1/24); and lymphoma, 4.2% (1/24). Favorable prognostic factors included: primary tumor less than 3 cm in size and absence of associated cirrhosis. These results emphasize that orthotopic liver transplantation can provide a long-term cure for approximately 50% of patients whose primary hepatic malignancy is unresectable by conventional procedures.

Hepatobiliary cancer--surgical considerations.

Most cancers of the hepatobiliary system will have grown beyond the limits of curative resection by the time they become clinically evident. This reality has fostered therapeutic nihilism, and most physicians and surgeons in their pessimism have failed to study the early modes of spread of these tumors--patterns of growth that are relevant to proper treatment of those patients who do have lesions that can be removed with hope of achieving a cure. Moreover, anatomic and technical complexities may beget surgical reluctance in the management of potentially curable lesions. Therefore, this review is offered for orientation and perspective for those who would hope to offer optimal treatment for patients who have primary cancers of the liver, gallbladder, or biliary ducts. The review includes considerations of (1) surgical anatomy, (2) modes of spread, (3) assessment of resectability, (4) surgical technique, and (5) results of operative treatment in relation to "curative" resection. Also offered are some guides to palliative surgical management of tumors that have reached the hepatic hilus.

Surgical treatment of intrahepatic cholangiocarcinoma: four patients surviving more than five years.

BACKGROUND: To find the rational surgical strategy for the treatment of intrahepatic cholangiocarcinoma (ICC), clinical features of ICC were studied in 20 patients who underwent hepatic resection in the National Cancer Center Hospital from 1980 to 1990. METHODS: According to the morphologic pattern, we classified the ICCs into two subcategories, mass-forming and infiltrating, which correlated with their biologic behavior. RESULTS: Of 10 patients who underwent hepatectomy for mass-forming ICC, three survived more than 5 years without recurrence. The 1-, 3-, and 5-year survival rates were 59.3%, 44.4%, and 44.4%, respectively. Of 10 patients who underwent hepatectomy for infiltrating ICC, one survived more than 5 years without recurrence. The 1-, 3-, and 5-year survival rates were 72.0%, 27.0%, and 27.0%, respectively. The pathologic findings and recurrences indicated that the salient feature of the mass-forming type was its tendency for intrahepatic metastasis especially near a main lesion, and of the infiltrating type was the infiltrative spread via Glisson's capsule and hilar lymph nodal metastasis. CONCLUSIONS: An anatomic and extensive liver resection should be performed for mass-forming ICC, whereas a hepatectomy with excision of the extrahepatic bile duct and hilar lymph nodal dissection is recommended for infiltrating ICC.

Major liver resection: perioperative course and management.

This report investigates the perioperative course in 81 consecutive major liver resections, performed mainly because of primary liver cancer or colorectal liver secondaries. The liver was resected transabdominally with or without prior ligation of hilar structures. Intravenous nutrition consisted of 10% dextrose alone and was started preoperatively. Albumin or plasma was used rarely and only in conjunction with massive intraoperative transfusion of blood. Major complications, including four operative deaths (4.9%), consisted of bleeding and/or infection in eight (10%) patients and overt liver failure in two patients (2%) and occurred only after right and extended right lobectomies. Intraoperative blood loss was significantly larger in patients with postoperative complications than in patients with an uneventful postoperative course. The direct parenchymal approach was associated with a shorter operative time and an unchanged intraoperative bleeding. Coagulopathy and hypoalbuminemia did not cause any problems. Blood glucose levels were stable, and no patient suffered from hypoglycemia. It is concluded that major liver resection should be based on prevention of intraoperative bleeding and that preresection ligation of hilar structures offers no advantage in this respect. Infusion of hypocaloric glucose solutions should be started the day before operation, and routine administration of other nutrients does not seem necessary.

Intrahepatic abscess as a complication of long-term percutaneous internal biliary drainage.

One hundred five patients with obstructive jaundice have undergone percutaneous transhepatic internal biliary drainage at the Johns Hopkins Hospital. Many of these patients subsequently underwent corrective or palliative surgery, whereas other died of malignant disease after relatively short periods of catheter decompression, Seven of these patients with percutaneous internal biliary drainage, however, have been followed for over 8 months. Three of these seven patients developed intrahepatic abscesses at a mean of 16 months after catheter placement. Two of the three patients died of sepsis. In two of the patients the abscesses communicated with the biliary tree, in the third it did not. Intrahepatic abscess formation may be a common complication of long-term percutaneous transhepatic internal biliary drainage, and it should be suspected in any patient with fever or signs of sepsis who has been followed with catheter drainage for over 6 months.

The role of U tube palliative treatment in high bile duct carcinoma.

Twenty-one patients with cholangiocarcinoma at the confluence of the main right and left hepatic ducts were referred to our professorial surgical unit between 1968 and 1982. All were evaluated, treated, and documented prospectively with follow-up to mid 1986. No lesion was deemed resectable. The U tube palliative bypass developed during the course of the study was used in 14 patients, and its role in treating high bile duct carcinoma was evaluated. Histologic confirmation of the diagnosis was obtained in 71% of patients. Seven patients received additional treatment with radical radiotherapy. The 30-day overall hospital mortality rate was 19%. The 1- and 2-year survival rates were 57% and 33%, respectively. The quality of survival was usually good. The need for centralized referral and treatment of these difficult patients is stressed. The case against radical resection for this lesion is presented. It is concluded that radical resection is seldom possible, and therefore the U tube palliative procedure is advocated in most patients.

Benign fibrosing disease at the hepatic confluence mimicking Klatskin tumors.

BACKGROUND: Hilar obstructions remain a challenge with regard to diagnosis and treatment. METHODS: In the period from 1984 to 1990, 82 patients underwent resective surgery under the presumptive diagnosis of hilar cholangiocarcinoma (Klatskin tumor). The diagnosis was based on the combined appearances on direct cholangiography and ultrasonography in all cases, with the use of various other imaging modalities in some cases. RESULTS: The perioperative findings from an experienced surgical team were usually thought to be compatible with bile duct carcinoma. However, histologic examination of the resected specimens revealed benign fibrosing or localized sclerosing lesions in 11 patients (13.4%). CONCLUSIONS: The current state of diagnostic imaging fails as yet to discriminate reliably between benign and malignant hilar lesions. Whereas the immediate therapeutic consequences may be equal (resection followed by hepaticojejunostomy), the late consequences differ in a major way because benign disease has a much better prognosis. In the presence of suspicious hilar obstruction, operable lesions should not be treated by "palliative" intubational techniques and radiation therapy without a firm diagnosis of malignancy. However, overtreatment (extended liver resection, vascular reconstruction, and liver transplantation) should be avoided as well when a benign lesion has not been ruled out.

Hepatic transplantation for primary and metastatic cancers of the liver.

Long-term results of transplantation for primary and metastatic hepatic malignancies were evaluated retrospectively in 637 patients. Recurrence rates and 2-year and 5-year patient survival rates were calculated. The overall recurrence rate was 40%, with 81% of deaths from recurrence occurring within 2 years after transplantation. Best results were obtained with uncommon tumors: incidental hepatomas (13% recurrence; 57% 2-year and 5-year follow-up); epithelioid hemangioendotheliomas (33% recurrence; 82% and 43% 2-year and 5-year survival); hepatoblastomas (33% recurrence; 50% 2-year and 5-year survival); and fibrolamellar hepatomas (39% recurrence; 60% and 55% 2-year and 5-year survival). Hemangiosarcomas had 64% recurrence, and all patients died within 27.5 months. Tumors metastatic to the liver had 59% recurrence, with 38% and 21% 2-year and 5-year survival rates. Transplantation should be abandoned for hemangiosarcomas and most metastatic tumors, except possibly for some slowly growing neuroendocrine tumors. The usual hepatomas had 39% recurrence with 2-year and 5-year survival rates of 30% and 18%, respectively. Cholangiocarcinomas had 44% recurrences with 2-year and 5-year survival rates of 30% and 17%, respectively. Transplantation for hepatomas and cholangiocarcinomas should be reserved for patients with favorable risk factors or when combined with well-defined chemotherapy protocols before and after operation.