A 17-years old woman with a combination of unilateral pheochromocytoma and an asymptomatic islet cell tumor of the pancreas is presented. The unusual coincidence of pheochromocytoma and pancreatic islet cell tumor in the patient is of interest as a possible crossover between MEN I and MEN II. It has been suggested that MEN I represents an abnormality of the APUD(amine precursor uptake and decarboxylation) cells of ectodermal origin. However, the possibility of a common progenitor cell in the neural crest for all APUD cells has been suggested by Pearse and Polak(1971). Recent clinical reports suggest that overlap between the two syndroms may occur. The overlapping of elements of the classical endocrine neoplasia should alert clinicians to the possibility of such associations in any particular patients. Since islet cell tumors may occur in association with pheochromocytomas and may be clinically silent, exploration of the pancreas during surgery for pheochromocytomas would seem to be useful.
Adenoma, Islet Cell ; Adolescent ; APUD Cells ; Ectoderm ; Female ; Humans ; Islets of Langerhans* ; Male ; Multiple Endocrine Neoplasia Type 1 ; Multiple Endocrine Neoplasia Type 2a ; Neural Crest ; Pancreas ; Pheochromocytoma* ; Pyrus ; Stem Cells

The prognosis of distal pancreatic cancer is even poorer than that of pancreatic head cancer because of its late symptom onset and tendency of aggressive retroperitoneal invasion at diagnosis. Since the first Appleby's operation in 1952, it has been performed in stomach cancer by several surgeons. But the survival benefit is still controversial. Appleby's operation includes total gastrectomy, splenectomy, distal pancreatectomy, celiac trunk division with ligation of common hepatic artery(CHA) and retroperitoneal lymph node dissection. In 1976, Nimura first adopted this method for distal pancreatic cancer. We report a case of modified Appleby's operation for distal pancreatic cancer. The patient was 44- year old female. Her chief complaint was epigastric pain for 2 weeks. Preoperative liver function test was within normal range and CA19-9 was elevated to 200 U/ml. Preoperative CT angiography showed 4.5x3cm sized, pancreatic mass in body and tail area invading splenic artery and celiac trunk from its left side. In operation, splenectomy and distal pancreatectomy was initially performed. After ligation of CHA, we confirmed intact proper hepatic artery(PHA) flow by doppler and then divided CHA. After confirmation of intact gastric blood flow, left gastric artery(LGA) was divided. Celiac axis(CA) was divided near its origin. Then we dissected retroperitoneal lymph nodes. There was minor pancreatic leakage controlled by conservative management. CA19-9 was normalized to 33 U/ml on the 16th postoperative day. She was discharged on the 28th postoperative day and underwent adjuvant chemotherapy and radiotherapy. There is no evidence of recurrence for 15 months of follow-up. We suggest that modified Appleby's operation should be considered for radical resection of distal pancreatic cancer which is invading CA or major CA branch but not involving PHA and superior mesenteric artery(SMA), if the CA root is resectable and PHA flow is intact from SMA after ligation of CHA.
Angiography ; Chemotherapy, Adjuvant ; Diagnosis ; Female ; Follow-Up Studies ; Gastrectomy ; Head and Neck Neoplasms ; Humans ; Ligation ; Liver Function Tests ; Lymph Node Excision ; Lymph Nodes ; Pancreatectomy ; Pancreatic Neoplasms* ; Prognosis ; Radiotherapy ; Recurrence ; Reference Values ; Splenectomy ; Splenic Artery ; Stomach Neoplasms

The gallbladder neck cancer and perihilar cholangiocarcinoma present as painless progressive surgical obstructive jaundice. Sometimes it becomes difficult to differentiate between them even on cross-sectional imaging studies including computed tomography and magnetic resonance imaging. Staging laparoscopy and positron emission tomography may be useful in detecting metastases in gallbladder neck cancer, but are not recommended in perihilar cholangiocarcinoma. Most patients with gallbladder neck cancer and perihilar cholangiocarcinoma require preoperative biliary drainage. The differentiation is, however, important because their behavior and prognosis are totally different. Gallbladder neck cancer is biologically aggressive, thus long-term surviver are rare even after major resection. On the other hand, perihilar cholangiocarcinoma is often less aggressive and major proceduresresections are justified. Gallbladder neck cancer and perihilar cholangiocarcinoma, though not siblings, they tend to look alike sometimes.
Cholangiocarcinoma* ; Drainage ; Gallbladder* ; Hand ; Head and Neck Neoplasms* ; Humans ; Jaundice, Obstructive ; Laparoscopy ; Magnetic Resonance Imaging ; Neoplasm Metastasis ; Positron-Emission Tomography ; Prognosis ; Siblings*

Abnormal attachment of the gallbladder to the liver is the main cause for gallbladder torsion. However, the present study reports a rare case of gallbladder torsion in which a portion of fundus is rotated along the axis of body. So far, very few similar cases have been reported. An 87-year-old woman complaining right upper quadrant abdominal pain for 4 days was admitted. Her body temperature was 38.5degrees C with moderate dehydration. A large tender mass was palpated on the right abdomen extending to the right iliac fossa. Computed tomography of abdomen showed a large cavity with a diameter of 15 cm containing a big stone and a small normal looking gallbladder. Ultrasonographic scan showed a twisted portion of the gallbladder torsion. During emergency laparotomy, the middle portion of the gallbladder was found to be twisted counterclockwise with huge gangrenous gallbladder distal. The proximal body of the gallbladder was spared and attached firmly to the liver. Cholecystectomy was performed and the patient was discharged 2 weeks later without postoperative complications. Histological findings of specimen were consistent with operative findings. The current study reports on a rare case of gallbladder torsion by reviewing previous studies.
Abdomen ; Abdominal Pain ; Aged, 80 and over ; Axis, Cervical Vertebra ; Body Temperature ; Cholecystectomy ; Dehydration ; Emergencies ; Female ; Gallbladder* ; Gallstones ; Humans ; Laparotomy ; Liver ; Postoperative Complications

The diagnosis of gallstone ileus is occasionally challenging due to the variability of its presentation. We herein present a very rare case of gallstone ileus inducing obstructive jaundice at the afferent loop of Roux-en-Y hepaticojejunostomy after 10 years of bile duct cancer surgery. We describe the case of a 74-year-old Korean woman with obstructive jaundice, treated conservatively. She showed severely impaired liver function test and obstructive jaundice. The computed tomography (CT) scan led to a diagnosis of very rare type of gallstones ileus at the afferent jejunal loop. Since the clinical manifestation was improved, we decided to observe her closely. On the next follow-up CT scan, the gallstone disappeared with mild distension of the afferent bowel loop, implicating spontaneous passage of the gallstone. She recovered and returned to normal life after 10 days of initiation of clinical manifestations. We presume that the gallstone may enter the afferent jejunal loop through the hepaticojejunostomy and later increase in size. The presence of narrow tract of intestine may facilitate the incidence of gallstone ileus. It appears to be the first report on this rare type of gallstone ileus inducing obstructive jaundice.
Aged ; Bile Duct Neoplasms* ; Diagnosis ; Female ; Follow-Up Studies ; Gallstones* ; Humans ; Ileus* ; Incidence ; Intestines ; Jaundice, Obstructive* ; Liver Function Tests ; Tomography, X-Ray Computed

Ovarian metastases represent about 3-5% of all ovarian malignancies. Most of these tumors originate in the digestive tract and cholangiocarcinoma rarely involves the ovary. A 60-year-old woman was admitted for the investigation of abdominal distension that had lasted 1 week. One and a half years prior, the patient had undergone choledochal cyst excision, Roux-en Y hepaticojejunostomy and cholecystectomy. Computed tomography scans of the abdomen revealed a papillary mass in the remnant distal common bile duct and enlargement of both ovaries with a huge amount of ascites. An explorative laparotomy disclosed no peritoneal seeding with resectable cholangiocarcinoma and bilateral ovarian mass. Pylorus-preserving pancreatoduodenectomy and bilateral salphingo-oophorectomy with hysterectomy were performed. Histologically, it was a well-differentiated adenocarcinoma and all surgical margins were free of tumor. Both ovarian masses were consistent with metastatic adenocarcinoma from the common bile duct. The patient received six cycles of postoperative adjuvant systemic chemotherapy, dying after 10 months due to pulmonary embolism.
Abdomen ; Adenocarcinoma ; Ascites ; Cholangiocarcinoma ; Cholecystectomy ; Choledochal Cyst* ; Common Bile Duct* ; Drug Therapy ; Female ; Gastrointestinal Tract ; Humans ; Hysterectomy ; Laparotomy ; Middle Aged ; Neoplasm Metastasis* ; Ovarian Neoplasms ; Ovary ; Pancreaticoduodenectomy ; Pulmonary Embolism

The wall of normal proximal bile duct is occasionally thin with close approximation to the right hepatic artery. Thus, isolation of this hepatic artery can result in excessive weakening of the remnant proximal bile duct wall during hemiliver graft harvest. This type of injury can induce ischemic stricture of the donor common bile duct. This study aimed to review the clinical sequences of such ischemic type donor bile duct injuries primarily managed with endoscopic and radiological interventional treatments. A retrospective review of medical records was performed for two living donors who suffered from ischemic type donor bile duct injury. They were followed up for more than 10 years. The right and left liver grafts were harvested from these two donors. Bile duct anatomy was normal bifurcation in one and anomalous branching in the other. Bile duct stenosis was detected in them at 2 weeks and 1 week after liver donation. They underwent endoscopic balloon dilatation and temporary stent (endoscopic retrograde biliary drainage tube) insertion. After keeping the tube for 2 months, the tube was successfully removed in one donor. However, endoscopic treatment was not successful, thus additional radiological intervention was necessary in the other donor. On follow-up over 10 years, they are doing well so far with no recurrence of biliary stricture. Based on our limited experience, interventional treatment with subsequent long-term follow-up appears to be an essential and choice treatment for ischemic type biliary stricture occurring in liver living donors.
Bile Ducts ; Common Bile Duct ; Constriction, Pathologic* ; Dilatation ; Drainage ; Follow-Up Studies ; Hepatic Artery ; Humans ; Ischemia ; Liver* ; Living Donors* ; Medical Records ; Recurrence ; Retrospective Studies ; Stents ; Tissue Donors ; Transplants

We present a rare case of functional stenosis of the jejunal loop following left hepatectomy and hepaticojejunostomy long after pylorus-preserving pancreaticoduodenectomy (PPPD), which was successfully managed by balloon dilation. A 70-year-old Korean man had undergone PPPD 6 years before due to 1.8 cm-sized distal bile duct cancer. Sudden onset of obstructive jaundice led to diagnosis of recurrent bile duct cancer mimicking perihilar cholangiocarcinoma of type IIIb. After left portal vein embolization, the patient underwent resection of the left liver and caudate lobe and remnant extrahepatic bile duct. The pre-existing jejunal loop and choledochojejunostomy site were used again for new hepaticojejunostomy. The patient recovered uneventfully, but clamping of the percutaneous transhepatic biliary drainage (PTBD) tube resulted in cholangitis. Biliary imaging studies revealed that biliary passage into the afferent jejunal limb was significantly impaired. We performed balloon dilation of the afferent jejunal loop by using a 20 mm-wide balloon. Follow-up hepatobiliary scintigraphy showed gradual improvement in biliary excretion and the PTBD tube was removed at 1 month after balloon dilation. This very unusual condition was regarded as disuse atrophy of the jejunal loop, which was successfully managed by balloon dilation and intraluminal keeping of a large-bore PTBD tube for 1 month.
Aged ; Bile Duct Neoplasms ; Bile Ducts, Extrahepatic ; Cholangiocarcinoma ; Cholangitis ; Choledochostomy ; Constriction ; Constriction, Pathologic* ; Diagnosis ; Drainage ; Extremities ; Follow-Up Studies ; Hepatectomy* ; Humans ; Jaundice, Obstructive ; Liver ; Muscular Disorders, Atrophic ; Pancreaticoduodenectomy* ; Portal Vein ; Radionuclide Imaging

BACKGROUNDS/AIMS: Both preoperative transcatheter arterial chemoembolization (TACE) alone and portal vein embolization (PVE) alone have a detrimental prognostic effect on the post-resection outcomes in patients with hepatocellular carcinoma (HCC). The main objective of this study was to assess the prognostic impact of preoperative TACE on the long-term survival outcomes in patients undergoing preoperative PVE and right liver resection for solitary HCC. METHODS: Patients who underwent macroscopic curative right liver resection of solitary HCC that lied between 3.0 and 7.0 cm (n=113) with or without preoperative TACE and PVE were selected for the study, making these subjects were divided into three groups; the TACE-PVE group (n=27), the PVE-alone group (n=13), and the control group (n=73). The subjects in the three groups were followed up for > or =36 months or until death. RESULTS: The 1-, 3-, 5-, and 10-year overall patient survival rates of all 113 patients were 96.5%, 88.2%, 81.3% and 65.0%, respectively. The 1-, 3-, 5-, and 10-year overall patient survival rates were 96.3%, 83.4%, 83.4% and 47.6% respectively in the TACE-PVE group; 84.6%, 76.9%, 57.7% and 19.2% respectively in the PVE-alone group; and 98.6%, 91.7%, 85.1% and 81.7% respectively in the control group (p=0.047). Patients were also sub-grouped according to tumor size, and those with a tumor of up to cutoff at 5 cm showed no prognostic difference (p=0.774), but tumor size >5 cm was associated with inferior patient survival only in the TACE-PVE group (p=0.018). CONCLUSIONS: Preoperative sequential TACE and PVE appear to be compliant to the conventional oncological concept in addition to induction of the future remnant liver regeneration. Therefore, we suggest that preoperative TACE should be come first whenever preoperative PVE for major hepatectomy is planned, especially in patients with hypervascular HCC tumors.
Carcinoma, Hepatocellular* ; Hepatectomy* ; Humans ; Liver ; Liver Regeneration ; Portal Vein* ; Survival Rate

BACKGROUNDS/AIMS: Stem cell therapies for liver disease are being studied by many researchers worldwide, but scientific evidence to demonstrate the endocrinologic effects of implanted cells is insufficient, and it is unknown whether implanted cells can function as liver cells. Achieving angiogenesis, arguably the most important characteristic of the liver, is known to be quite difficult, and no practical attempts have been made to achieve this outcome. We carried out this study to observe the possibility of angiogenesis of implanted bio-artificial liver using scaffolds. METHODS: This study used adipose tissue-derived stem cells that were collected from adult patients with liver diseases with conditions similar to the liver parenchyma. Specifically, microfilaments were used to create an artificial membrane and maintain the structure of an artificial organ. After scratching the stomach surface of severe combined immunocompromised (SCID) mice (n=4), artificial scaffolds with adipose tissue-derived stem cells and type I collagen were implanted. Expression levels of angiogenesis markers including vascular endothelial growth factor (VEGF), CD34, and CD105 were immunohistochemically assessed after 30 days. RESULTS: Grossly, the artificial scaffolds showed adhesion to the stomach and surrounding organs; however, there was no evidence of angiogenesis within the scaffolds; and VEGF, CD34, and CD105 expressions were not detected after 30 days. CONCLUSIONS: Although implantation of cells into artificial scaffolds did not facilitate angiogenesis, the artificial scaffolds made with type I collagen helped maintain implanted cells, and surrounding tissue reactions were rare. Our findings indicate that type I collagen artificial scaffolds can be considered as a possible implantable biomaterial.
Actin Cytoskeleton ; Adult ; Animals ; Artificial Organs ; Biocompatible Materials ; Collagen Type I* ; Humans ; Liver Diseases ; Liver* ; Membranes, Artificial ; Mice ; Stem Cells* ; Stomach ; Tissue Scaffolds ; Vascular Endothelial Growth Factor A