Cases reported "Biliary Tract Neoplasms"

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1/38. 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.
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2/38. 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.
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3/38. 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.
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4/38. Palliative transhepatic biliary drainage and enteral nutrition.

    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.
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5/38. 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.
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6/38. Biliary stricture due to neuroma after an innocent blunt abdominal trauma.

    A traumatic neuroma of the biliary tract is rarely associated with biliary obstruction. However, when it arises in the common bile duct (CBD) and is associated with obstructive jaundice, it is difficult to distinguish it from bile duct cancer. We describe a patient who developed obstructive jaundice and itching, due to CBD stricture, 8 years after innocent blunt abdominal trauma. The stricture was resected and hepatico-jejunal anastomosis was performed. Histological examination revealed a traumatic neuroma and a fibrous scar around the common bile duct. Symptoms disappeared following surgical removal of the lesion. Blunt abdominal injury may cause the late onset of a fibrous scar and traumatic neuroma in the common bile duct. To our knowledge, a traumatic neuroma of the biliary tract after blunt abdominal trauma has not been reported previously. We review the clinical picture of this relatively rare problem, along with its diagnosis, pathogenesis and treatment.
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7/38. A case of biliary cystadenocarcinoma arising in the liver with a congenital retention of indocyanine green.

    A case of biliary cystadenocarcinoma that occurred in a 45-year-old woman is reported. ultrasonography and computed tomography clearly revealed papillary projections in the cyst of the liver. Percutaneous transhepatic cystography showed connection between the cyst and the common bile duct. The tumor was surgically resected and proved to be a mucinous papillary adenocarcinoma arising from a biliary cystadenoma. The patient is doing well 4 years after surgery. Interestingly, this is the first reported case of a biliary cystadenocarcinoma in the liver with markedly diminished excretion of indocyanine green.
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8/38. Hepatobiliary cystadenocarcinoma connected to the hepatic duct: a case report and review of the literature.

    We present a rare case of hepatobiliary cystadenocarcinoma with biliary communication. A 74-year-old woman had a liver cyst that had enlarged from 5 cm to 8 cm in diameter over the last 2 years. A mural nodule, 1 cm in diameter, was first demonstrated by computed tomography in a multilocular cyst in segment IV. The nodule showed hypervascularity at angiography and computed tomography during arteriography. Percutaneous transhepatic cystography demonstrated a communication between the cyst and the biliary tract. The cyst was filled with mucinous and gelatinous fluid and was revealed to contain cancer cells. The patient underwent total tumor extirpation with the surrounding hepatic parenchyma. We confirmed and closed the biliary fistula connected to the right hepatic duct. Histologically, the cyst wall was composed of cystadenoma and the mural nodule showed in situ papillary adenocarcinoma. The patient is doing well 9 months after surgery. Complete tumor extirpation and closing of the biliary fistula is the treatment of choice.
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9/38. Photodynamic therapy for malignant biliary obstruction: a case series.

    We describe four elderly patients (age range 73-85 years) with bile duct carcinoma who were treated with photodynamic therapy. These patients could not be treated surgically because of the presence of cardiopulmonary disease and the extent of the bile duct carcinoma. Prior to photodynamic treatment the patients underwent percutaneous transhepatic biliary drainage (PTBD) to relieve jaundice. The photodynamic therapy was carried out under percutaneous transhepatic cholangioscopy, 2 days after intravenous administration of sodium porfimer (2 mg/kg). Excimer dye laser was used to irradiate the obstructive lesion with an energy dosage of 50 J/cm2 (total irradiation dose 150-250 J/cm2) and stenotic site with a dosage of 50-100 J/cm2. Photodynamic therapy was achieved safely without occurrence of cholangitis in all patients, and was followed by metallic stent placement, resulting in the improvement of performance levels in three patients who did not have liver metastases. Photodynamic therapy via the PTBD route is a safe and promising palliative therapy for selected elderly patients with bile duct carcinoma.
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10/38. Embryonal rhabdomyosarcoma within a choledochal cyst.

    The authors describe a 6-year-old girl in whom they found an embryonal rhabdomyosarcoma in a choledochal cyst; they found no previous reports of such an association. The patient presented with recurrent jaundice and loss of weight and appetite. A large abdominal mass was palpable. ultrasonography and computed tomography revealed a large subhepatic mass, which contained multiple septations and mural masses delineating cystic areas. The common hepatic duct opened directly into the mass, and the intrahepatic radicles were dilated. Intraoperative cholangiography revealed that the cysts in the mass were continuous with the hepatic and intrahepatic radicles. The mass was completely excised, and a Roux-en-Y hepaticojejunostomy was performed. Postoperative adjunct chemotherapy and radiotherapy were instituted.
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