1/89. Biliary cystadenocarcinoma of the liver: the need for complete resection. We report on a patient with biliary cystadenocarcinoma and review 112 previously published cases of this rare cystic hepatic neoplasm. This tumour mainly occurs in women at a ratio of 62% (female) to 38% (male), and at an average age of 56.2 years (range 18-88 years). The origin of these neoplasms is intrahepatic in 97% of cases and extrahepatic in the remaining 3%. The clinical symptoms are nonspecific and are not distinctive from benign cystic liver lesions unless invasive growth of the tumour occurs or distant metastases are present. Sonography and computed tomography (CT), as well as magnetic resonance imaging (MRI) demonstrate the multilocular nature of the tumour with septal or mural nodules. Discrete soft tissue masses, thick and coarse calcifications and varying density on CT or intensity on MRI within the loculi are additional non-specific imaging findings. The best therapeutic result with a 5-year survival rate of 100% and a recurrence rate of only 13% was achieved by complete excision (n = 16). Surgical removal of the tumour by complete excision is, therefore, the treatment of choice for biliary cystadenocarcinomas. ( info) |
2/89. learning from case reports: diagnostic issues in an epidemiologic study of pancreatic cancer. epidemiologic studies on exocrine pancreatic cancer show a large heterogeneity in diagnostic criteria applied to define "caseness." Reanalyses conducted after review of diagnostic information have yielded substantially different results than those based on more crude classifications of disease. During a multicenter prospective study on mutations in the K-ras gene in pancreatic and biliary diseases, hospital diagnoses from 602 patients were reviewed by a panel of experts. There were two main motivations to do so: a generic interest for the quality of the diagnostic data, and the anticipation that a firm diagnosis could be needed to assess whether patients whose tumors did not harbor the mutation were true negatives or false negatives. In addition, the review of diagnoses was helpful to minimize tissue misclassification, and it had a high educational value for clinicians and epidemiologists. This article illustrates why and how this was so through a brief presentation of the 10 most significant cases. With respect to selection and classification of subjects, the main issues that studies on pancreatic cancer need to address are the differential diagnosis of exocrine pancreatic cancer and pancreatitis, the differential diagnosis of exocrine pancreatic cancer and other abdominal tumors, and the use of survival as a hallmark of pancreatic cancer. In epidemiologic studies of pancreatic cancer, it is warranted that a panel of experts centrally reviews all the existing diagnostic evidence (cytohistological and other) of all patients, regardless of whether they have cytohistological confirmation and of their hospital discharge diagnosis. ( info) |
3/89. Pancreaticobiliary carcinoma associated with a large choledochal cyst: role of MRI and MR cholangiopancreatography in diagnosis and preoperative assessment. The role of magnetic resonance (MR) imaging and MR cholangiopancreatography is demonstrated in a case of pancreaticobiliary carcinoma associated with a large choledochal cyst. The size of the cyst presented considerable difficulty in evaluation with both endoscopic retrograde cholangiopancreatography and computed tomography. ( info) |
4/89. Biliary lymphoepithelioma-like carcinoma not associated with Epstein-Barr virus. We report the case of a 64-year-old man who presented with a hepatic mass and macronodular cirrhosis. The pathologic findings revealed a lymphoepithelioma-like carcinoma arising in the hepatobiliary tract that was morphologically identical to nasopharyngeal undifferentiated carcinoma. However, this tumor was not associated with Epstein-Barr virus infection in molecular studies. Macronodular cirrhosis associated with hepatitis c virus was present in the background liver. ( info) |
5/89. Primary smooth muscle tumor of the liver encasing hepatobiliary cystadenoma without mesenchymal stroma. We describe a 59-year-old Japanese woman with a large mass of her liver encasing cystic components. Radiologic imaging showed the mass to be hypervascular, and surgical resection disclosed a white tumor. The solid portion was immunohistochemically characterized as a smooth muscle tumor. The cystic components were multilocular and lined with columnar epithelium, consistent with a hepatobiliary cystadenoma. The epithelium strongly stained for CA19-9. The subepithelial space was occupied by collagenous connective tissue interspersed with a small number of spindle-shaped cells. The cystic lesions lacked the mesenchymal stroma between the epithelium and connective tissue layer. There have been no previous reports of a hepatic smooth muscle tumor encasing a hepatobiliary cystadenoma. Because of the pathogenesis of the cystadenoma, it is possible to assume that the smooth muscle tumor also arose from the cells composing the biliary duct in association with the development of the cystadenoma. ( info) |
6/89. granular cell tumor with cirrhosis and transplantation. granular cell tumor is a rare cause of hepatic dysfunction. We report here on a patient who underwent liver transplantation for this ailment. In our literature review, the common bile duct was most commonly involved (56%). A wide variety of therapies were advanced for this type of lesion, spanning three decades of care. Twenty-eight patients (49%) had no follow-up reported, and another 2 (3%) were found at autopsy. Sixteen patients (28%) were followed more than 1 year, with 72% followed less than 1 year if at all. We present the first case of a granular cell tumor being treated with liver transplantation. Although adequate early excisional surgery should obviate the need for transplantation in these cases, widely disparate therapy and poor follow-up may mask generally inadequate therapy for this lesion. The authors recommend thorough excision and long-term follow-up for patients with this entity to avoid secondary biliary cirrhosis and to eliminate the preventable need for transplantation. ( info) |
| Simultaneous intestinal and biliary obstruction is a rare but agonizing complication of metastatic abdominal cancer. Although endoscopic procedures exist that relieve jaundice or restore enteral nutrition, they can be impossible to perform for technical or anatomical reasons. We propose a palliative approach for these patients that includes transcutaneous common bile duct drainage, progressive dilation of the transhepatic channel over 1 wk, and, finally, insertion of a permanent silicon catheter that drains bile into the duodenum and is combined with an enteral feeding line. We report three patients whose metastatic abdominal tumors had led to simultaneous jaundice and gastric outlet obstruction, neither of which could be treated endoscopically. In all patients, the transcutaneous bile drainage catheter combined with the enteral feeding line was inserted and tumor symptoms resolved rapidly. As a result, the patients chose to return to home care with enteral nutrition and pain medication. The creation of a transhepatic access for simultaneous enteral bile drainage and nutrition is a technically simple procedure that causes little discomfort to a terminally ill patient. It relieves the symptoms of tumor obstruction, and the option of enteral nutrition and medication can obviate the need for intravenous infusions. ( info) |
8/89. granular cell tumor of the biliary tree in a pediatric patient. granular cell tumor of the biliary tree is a rare benign tumor that occurs most commonly in young African-American girls. granular cell tumor rarely is included in the differential diagnosis of biliary obstruction, yet complete surgical excision is curative. The authors report a case of granular cell tumor of the biliary tree in a pediatric patient that required a pancreaticoduodenectomy for cure. ( info) |
9/89. Rabdomyosarcoma of the biliary tree. Rabdomyosarcoma of the biliary tree is one of the rare causes of biliary tract obstruction in childhood. Nevertheless it is the most common cause of obstructive jaundice due to neoplastic biliary obstruction. We present a two-year-old child with obstructive jaundice secondary to an embryonal rhabdomyosarcoma of the biliary tree. She underwent surgery and, after total excision of the mass, a hepaticojejunostomy and Roux-en-Y anastomosis were performed. She was referred to the Pediatric Oncology Group for follow-up. rhabdomyosarcoma of the bilary tree, although rare, must be considered in the etiology of obstructive jaundice in children. ( info) |
10/89. Paraganglionoma of extrahepatic biliary tract causing obstructive jaundice. We report a young woman with paraganglionoma arising from the extrahepatic bile duct presenting with acute obstructive jaundice. The patient underwent excision of the gall bladder and extrahepatic bile duct with the tumor, and Roux-en-Y hepaticojejunostomy. She is asymptomatic 9 months later, with normal biochemical investigations and imaging. ( info) |