Two Cases of Disseminated Alveolar Echinococcosis: The Diagnosis, Management, and Differential Considerations for Liver Lesions.

Alveolar echinococcosis (AE), caused by Echinococcus multilocularis, is an aggressive and potentially critical infestation that primarily affects the liver and can metastasize to any part of the body. We herein report two cases of echinococcosis, which could be differentiated from malignancy on imaging studies, with infections of the liver and mediastinal lymph nodes, and also associated with systemic disseminated lesions. AE is a very invasive infectious disease, and in order to detect such lesions at an early stage when they are still resectable, it is necessary to understand the characteristic imaging findings and determine the patient's current medical history.

Total pancreatectomy with remnant stomach preservation in a patient with a history of proximal gastrectomy and interposed jejunal reconstruction with right gastroepiploic conduit preservation: a case report.

BACKGROUND: Pancreatic head resection following proximal gastrectomy jeopardizes the blood flow of the remnant stomach owing to right gastroepiploic conduit sacrifice, thereby necessitating total gastrectomy. However, owing to its high invasiveness, concomitant remnant total gastrectomy with pancreatectomy should be avoided as much as possible. Herein, we describe our experience of total pancreatectomy with right gastroepiploic conduit preservation in a patient with a history of proximal gastrectomy and reconstruction by jejunum interposition. CASE PRESENTATION: A 78-year-old woman with a history of gastric cancer was followed up at our institute for multiple intraductal papillary mucinous neoplasm, and main pancreatic duct stricture in the pancreatic head was newly detected. The cystic lesion was extended to the pancreatic body. Proximal gastrectomy and reconstruction by jejunal interposition were previously performed, and the mesenteric stalk of the interposed jejunum was approached through the retrocolic route. We planned total pancreatectomy with right gastroepiploic conduit preservation. Following adhesiolysis, the interposed jejunum and its mesentery lying in front of the pancreas were isolated. The arterial arcade from the common hepatic artery to the right gastroepiploic artery was detached from the pancreas. Furthermore, the right gastroepiploic vein was isolated from the pancreas. The pancreatic body and tail were pulled up in front of the remnant stomach, and the splenic artery and vein were resected. The pancreatic body and tail were pulled out to the right side, and the pancreatic head was divided from the pancreatic nerve plexus to the portal vein. The jejunal limb for entero-biliary anastomosis was passed through the hole behind the superior mesenteric artery and vein, and gastrointestinal anastomosis using the antecolic route and Braun anastomosis were performed. CONCLUSIONS: To avoid remnant total gastrectomy, right gastroepiploic conduit preservation is an optional procedure for pancreatic head resection in patients who have undergone proximal gastrectomy with reconstruction by jejunal interposition.

Pancreatic acinar cell carcinoma with pleomorphic and spindle cells: An autopsy-proven case.

Pancreatic acinar cell carcinomas are glandular and have amphophilic/eosinophilic cytoplasm, presenting acinar, solid, and trabecular structures. Unusual histological features of acinar cell carcinoma are known, such as oncocytic, pleomorphic, spindle, and clear cell variants, but their clinical significance has not been well described. A man in his 70s was referred to our hospital because of elevated serum pancreatic enzymes. Contrast-enhanced abdominal computed tomography revealed slight swelling of the pancreatic head and suspension of the main pancreatic duct in the pancreatic body. He died only 14 days after admission. Gross findings at autopsy showed an ill-defined tumor located in the pancreatic head, involving the gastric and duodenal walls. Peritoneal dissemination, liver metastases, and lymph node metastases were also observed. Microscopically, tumor cells had moderate-to-severe nuclear atypia and amphophilic cytoplasm showing pleomorphism, and diffusely proliferated in solid pattern without lumina, were admixed with spindle cells. Immunohistochemically, tumor cells including pleomorphic and spindle cells were positive for B-cell lymphoma/leukemia 10 and trypsin. Consequently, the diagnosis was pancreatic acinar cell carcinoma with pleomorphic and spindle cells. We encountered a rare variant of pancreatic acinar cell carcinoma with pleomorphic and spindle cells. Clinically, our case showed rapid progression.

A Case of Methimazole-Induced Acute Pancreatitis With an HLA Allele Causing Antithyroid Drug-Induced Agranulocytosis.

Among the side effects of methimazole (MMI) for the treatment of Graves' disease, MMI-induced acute pancreatitis (MIP) is a rare adverse reaction, with only 7 cases being reported to date. However, 2 large-scale population-based studies recently revealed that the risk of MIP was significantly higher, ranging from 0.02% to 0.56%. Although MIP is common in middle-aged and elderly Asian women, its pathogenesis remains largely unknown. We herein present a case of a 72-year-old Japanese woman with Graves' disease who developed MIP 12 days after the initiation of MMI. The MMI was discontinued, the patient was switched to propylthiouracil (PTU) therapy, and pancreatitis gradually resolved. Serological human leukocyte antigen (HLA) typing identified HLA-DRB1*08:03:02. This HLA allele was previously detected in a patient with MIP and is one of the major risk factors for agranulocytosis induced by antithyroid drugs, including PTU as well as MMI. In cases of MIP, PTU is being considered as an alternative to MMI; however, its safety needs further investigation and patients require close monitoring after the switch to PTU. Further studies are warranted, particularly on the relationship between MIP and the presence of HLA alleles causing antithyroid drug-induced agranulocytosis.

Leiomyosarcoma of the splenic vein with a history of bilateral retinoblastoma: A case report and review of the literature.

Diagnosing leiomyosarcomas of the splenic vein is challenging, and a treatment strategy has not yet been established for this condition. We herein report the case of a 45-year-old female patient with a history of retinoblastoma who underwent pancreatosplenectomy for a primary leiomyosarcoma originating from the splenic vein and transcatheter arterial chemoembolization for metastatic hepatic lesions observed 5 months postoperatively. An initial medical check-up using abdominal ultrasound revealed a 40-mm mass behind the pancreatic tail. Imaging tests revealed a well-circumscribed mass compressing the pancreas posteriorly, without invasion into the pancreatic duct. The splenic vein was torn, with the epiploic veins developed as collateral blood vessels, which was an atypical finding for carcinoma. The patient was followed up regularly. At 5 years after the first visit, an abdominal ultrasound showed that the mass had increased in size to 50 mm, and had developed into a tumor embolus within the splenic vein, causing an obstruction of the vessel. Pancreatosplenectomy was performed due to suspicion of malignancy. The final diagnosis was leiomyosarcoma arising from the splenic vein. The patient was discharged on postoperative day 15 and was followed up regularly thereafter. Imaging studies performed 5 months postoperatively revealed four hepatic lesions. The hepatic masses were histologically diagnosed as metastatic leiomyosarcomas, and transcatheter arterial chemoembolization was performed using epirubicin. Since then, regular follow-ups have been conducted without observed recurrence. Leiomyosarcoma of the splenic vein is exceedingly rare, and the number of reported cases is not sufficient to establish clinical guidelines. Therefore, it is crucial to collect more reports on the occurrence and treatment of this disease.

Solid pseudopapillary neoplasm of the pancreas in a young male with main pancreatic duct dilatation: A case report.

BACKGROUND: Solid pseudopapillary neoplasms (SPNs) are rare tumors of the pancreas. Typically, they occur in young females, often have characteristic imaging features, such as cystic components and calcification, and have few effects on the pancreatic duct. CASE SUMMARY: A 31-year-old man was admitted to our hospital with the chief complaint of epigastric pain. There was only mild tenderness in his upper abdomen, and blood tests showed only a slight increase in alkaline phosphatase. Contrast-enhanced computed tomography showed a 40-mm-diameter, hypovascular mass in the head of the pancreas, and the main pancreatic duct upstream of the mass was severely dilated. Magnetic resonance imaging showed low intensity on T1-weighted images, with high intensity on T2-weighted image in some parts. Pancreatic ductal adenocarcinoma was the primary differential diagnosis. Portal vein infiltration could not be ruled out, so this case was a candidate for neoadjuvant chemotherapy. Subsequently, endoscopic ultrasound-guided fine needle aspiration was performed, and pathological evaluation and immunostaining suggested a diagnosis of SPN. Thus, pancreatoduodenectomy was performed. One year after the operation, the patient is alive with no recurrence. CONCLUSION: Main pancreatic duct dilatation is usually a finding of suspected pancreatic cancer. However, pancreatic duct dilatation can occur in SPN depending on the location and growth speed. Therefore, SPN should be considered in the differential diagnosis of tumors with pancreatic duct dilatation, and pathological evaluation by endoscopic ultrasound-guided fine needle aspiration should be actively performed.

Pancreatic ductal adenocarcinoma with mismatch repair deficiency resected after long-term observation.

Pancreatic ductal adenocarcinoma (PDAC) with mismatch repair (MMR) deficiency is a rare subtype, clinicopathological features of which have not been fully understood. A 70-year-old woman was admitted for the investigation of a 20-mm pancreatic tumor in the pancreatic head, detected during the cause scrutiny of exacerbation of diabetes mellitus and panhypopituitarism. The tumor decreased in size after administration of hydrocortisone for panhypopituitarism. Autoimmune pancreatitis, complicated with hypophysitis, was suspected, and prednisolone treatment was administered. The tumor did not show enlargement for 3 years during which a dose of prednisolone was maintained. However, 1.5 years after the cessation of prednisolone administration, the tumor size increased again. On endoscopic ultrasonography, the tumor was found to be a 25.2-mm mass lesion with almost uniformly low echogenicity and blood flow signal, and anisonucleosis on cytodiagnosis was revealed. Pancreatoduodenectomy was performed, and on histological analysis, moderately differentiated tubular adenocarcinoma with massive lymphocytic infiltration was observed. Immunohistochemistry revealed a concomitant loss of MSH2 and MSH6 in the tumor cells, which implicated mutant MSH2 gene. She has remained well with no recurrence for 2.9 years since her surgery. We herein report a case of PDAC with MMR deficiency, resected after long-term observation.

Simultaneous macroamylasemia and macrolipasemia in a patient with mucosa-associated lymphoid tissue lymphoma.

We report a case of simultaneous macroamylasemia and macrolipasemia complicated with mucosa-associated lymphoid tissue (MALT) lymphoma. A 78-year-old man presented with hyperamylasemia and hyperlipasemia for 2 years and was misdiagnosed with chronic pancreatitis at another hospital. However, his other pancreatic enzymes were normal, his amylase-creatinine clearance ratio was low, and no definite findings of pancreatic disease were evident. Immunological analyses revealed that both amylase and lipase were bound to immunoglobulin (Ig) A-κ, and that serum IgA was high (827.1 mg/dL). He was diagnosed with simultaneous macroamylasemia and macrolipasemia. Since these diseases are associated with malignancy, an additional investigation was performed which revealed the complication of MALT lymphoma, and polymerase chain reaction analysis showed monoclonal immunoglobulin light chain gene rearrangement (κ >> λ). In this case, macroamylasemia and macrolipasemia may have developed due to the formation of macroenzymes resulting from excess IgA-κ secreted by the MALT lymphoma. Simultaneous macroamylasemia and macrolipasemia are very rare and difficult to diagnose and can lead to diagnostic and therapeutic errors. When encountering atypical clinical features associated with hyperamylasemia and hyperlipasemia, the possibility of macroenzymes and underlying diseases such as lymphoproliferative disorders should be considered.

Multicenter study of endoscopic preoperative biliary drainage for malignant hilar biliary obstruction: E-POD hilar study.

BACKGROUND AND AIM: Endoscopic nasobiliary drainage (ENBD) is often recommended in preoperative biliary drainage (PBD) for hilar malignant biliary obstruction (MBO), but endoscopic biliary stent (EBS) is also used in the clinical practice. We conducted this large-scale multicenter study to compare ENBD and EBS in this setting. METHODS: A total of 374 cases undergoing PBD including 281 ENBD and 76 EBS for hilar MBO in 29 centers were retrospectively studied. RESULTS: Extrahepatic cholangiocarcinoma (ECC) accounted for 69.8% and Bismuth-Corlette classification was III or more in 58.8% of the study population. Endoscopic PBD was technically successful in 94.6%, and adverse event rate was 21.9%. The rate of post-endoscopic retrograde cholangiopancreatography pancreatitis was 16.0%, and non-endoscopic sphincterotomy was the only risk factor (odds ratio [OR] 2.51). Preoperative re-intervention was performed in 61.5%: planned re-interventions in 48.4% and unplanned re-interventions in 31.0%. Percutaneous transhepatic biliary drainage was placed in 6.4% at the time of surgery. The risk factors for unplanned procedures were ECC (OR 2.64) and total bilirubin ≥ 10 mg/dL (OR 2.18). In surgically resected cases, prognostic factors were ECC (hazard ratio [HR] 0.57), predraiange magnetic resonance cholangiopancreatography (HR 1.62) and unplanned re-interventions (HR 1.81). EBS was not associated with increased adverse events, unplanned re-interventions, or a poor prognosis. CONCLUSIONS: Our retrospective analysis did not demonstrate the advantage of ENBD over EBS as the initial PBD for resectable hilar MBO. Although the technical success rate of endoscopic PBD was high, its re-intervention rate was not negligible, and unplanned re-intervention was associated with a poor prognosis in resected hilar MBO.

Recurrent Cholangitis in a Patient with Autosomal Dominant Polycystic Kidney Disease (ADPKD) and Caroli's Disease.

We herein present a rare case of an autosomal dominant polycystic kidney disease (ADPKD) patient with Caroli's disease, a congenital embryonic biliary tree ductal plate abnormality often associated with autosomal recessive polycystic kidney disease. A 76-year-old woman with ADPKD on hemodialysis was admitted to our hospital with recurrent cholangitis and hepatobiliary stones. Caroli's disease was diagnosed according to typical imaging findings of cystic intrahepatic bile duct dilatation and the central dot sign. Hepatobiliary system abnormalities such as Caroli's disease should be considered in febrile ADPKD patients, even in the absence of typical clinical signs or symptoms.

Treatment of biliary tract stones after gastrectomy in the era of laparoscopic cholecystectomy.

BACKGROUND: The incidence of biliary tract stones has widely been reported to be high after gastrectomy. Treatment of biliary tract stones in post-gastrectomy patients was examined retrospectively, particularly minimally invasive therapy with laparoscopic cholecystectomy for gallbladder stones and endoscopic bile duct lithotomy for common bile duct stones. METHODS: Treatment of 122 post-gastrectomy patients with biliary tract stones was examined retrospectively from 1995 to April 2015. RESULTS: Symptomatic biliary tract stones were found in 3.7% of post-gastrectomy patients. The frequency of bile duct stones was higher in post-gastrectomy patients with gallbladder stones (44%) than in the nongastrectomy patients (11%). Laparoscopic cholecystectomy was carried out in 84% of the patients with gallbladder stones. Endoscopic bile duct lithotomy was feasible in 89% of patients with common bile duct stones. CONCLUSIONS: Laparoscopic cholecystectomy was feasible for removing gallbladder stones after gastrectomy. Endoscopic bile duct lithotomy followed by laparoscopic cholecystectomy was a good option for patients in whom endoscopic intervention could be performed. On the other hand, choledochoenterostomy could be a good option for patients in whom endoscopic bile duct lithotomy cannot be performed, particularly considering the risk of recurrent bile duct stones.

Multicenter study of endoscopic preoperative biliary drainage for malignant distal biliary obstruction.

AIM: To determine the optimal method of endoscopic preoperative biliary drainage for malignant distal biliary obstruction. METHODS: Multicenter retrospective study was conducted in patients who underwent plastic stent (PS) or nasobiliary catheter (NBC) placement for resectable malignant distal biliary obstruction followed by surgery between January 2010 and March 2012. Procedure-related adverse events, stent/catheter dysfunction (occlusion or migration of PS/NBC, development of cholangitis, or other conditions that required repeat endoscopic biliary intervention), and jaundice resolution (bilirubin level < 3.0 mg/dL) were evaluated. Cumulative incidence of jaundice resolution and dysfunction of PS/NBC were estimated using competing risk analysis. Patient characteristics and preoperative biliary drainage were also evaluated for association with the time to jaundice resolution and PS/NBC dysfunction using competing risk regression analysis. RESULTS: In total, 419 patients were included in the study (PS, 253 and NBC, 166). Primary cancers included pancreatic cancer in 194 patients (46%), bile duct cancer in 172 (41%), gallbladder cancer in three (1%), and ampullary cancer in 50 (12%). The median serum total bilirubin was 7.8 mg/dL and 324 patients (77%) had ≥ 3.0 mg/dL. During the median time to surgery of 29 d [interquartile range (IQR), 30-39 d]. PS/NBC dysfunction rate was 35% for PS and 18% for NBC [Subdistribution hazard ratio (SHR) = 4.76; 95%CI: 2.44-10.0, P < 0.001]; the pig-tailed tip was a risk factor for PS dysfunction. Jaundice resolution was achieved in 85% of patients and did not depend on the drainage method (PS or NBC). CONCLUSION: PS has insufficient patency for preoperative biliary drainage. Given the drawbacks of external drainage via NBC, an alternative method of internal drainage should be explored.

Adrenal Metastasis and Hemorrhage Secondary to Hepatocellular Carcinoma.

A 66-year-old man was admitted to undergo treatment for the sudden onset of hypogastric pain radiating to the left flank. Based on the clinical presentation and radiological findings, left adrenal hemorrhage secondary to hepatocellular carcinoma was diagnosed. Although the patient was hemodynamically stable, anemia progressed over the first four days after admission and a blood transfusion was subsequently initiated, which was effective. According to the results of interval imaging with computed tomography, elective transcatheter embolization (TAE) was performed nine days after admission to treat the bleeding from the adrenal tumor. After TAE, the patient's abdominal pain resolved, with no further progression of anemia.

A case report of the histologic transformation of primary follicular lymphoma of the duodenum.

A 46-year-old woman underwent upper endoscopy for evaluation of anemia, which revealed whitish granules at the duodenal papilla, diagnosed as duodenal follicular lymphoma (DFL) by biopsy. Computed tomography and abdominal ultrasonography revealed that follicular lymphoma was confined to the duodenum. Seven years after the diagnosis, fluorine-18 fluorodeoxyglucose positron emission tomography scanning revealed multiple lesions including in bone marrow and lymph nodes. Bone marrow biopsy of the right iliac bone revealed diffuse large B-cell lymphoma, indicating systemic dissemination and histologic transformation of the DFL. The patient responded to chemotherapy and has been progression-free for 2.5 years. Although DFL is usually indolent even without any treatment, systemic dissemination with histologic transformation can occur. This case suggests that the life-time follow-up that is usually done for patients with nodal follicular lymphoma should be provided to patients with DFL.

Recurrent common bile duct stones composed of ursodeoxycholic acid: a report of four cases.

Ursodeoxycholic acid (UDCA) is often used to treat cholesterol gallstones. UDCA makes cholesterol stones soluble, thereby improving biliary emptying. Conversely, however, UDCA can also form stones via an unknown mechanism, as shown in a few previous reports of cholangitis caused by the formation of UDCA stones in the common bile duct (CBD). We herein report four cases of recurrent cholangitis resulting from UDCA stones. The withdrawal of UDCA administration was highly effective in these patients. The details of these four cases suggest that clinicians must rethink the indications for UDCA treatment in cases in which cholangitis caused by CBD stones frequently recurs over a short period of time.

Ultrasonographic imaging of bile duct lesions in autoimmune pancreatitis.

OBJECTIVES: Patients with autoimmune pancreatitis (AIP) commonly have lesions in the bile duct itself and show stenosis of the bile duct system; however, no detailed study has evaluated the ultrasonographic findings of bile duct lesions in AIP. In this study, we monitored the clinical course and imaging findings, mainly ultrasonographic, of bile duct lesions in AIP. METHODS: We retrospectively analyzed the incidence of bile duct lesions, imaging findings, and clinical course in 37 patients with AIP. RESULTS: Characteristic bile duct and gallbladder wall thickening was recognized on ultrasound in 37.8% (14/37) of AIP patients. We divided the patients into 2 types according to the ultrasonographic findings of bile duct wall thickening: (1) 3-layer type (64.3%) and (2) parenchymal-echo type (35.7%). All 14 cases were treated with prednisolone, with immediate resolution of the bile duct lesions. CONCLUSION: Sclerosing cholangitis is one of the extrapancreatic lesions that are commonly detected in AIP patients; it is detected on ultrasonographic imaging as characteristic wall thickening. Our ultrasonographic findings reflect the fact that bile duct wall thickening in AIP is an inflammatory process that responds to prednisolone therapy. Ultrasonography is a useful tool in detecting biliary tract lesions in AIP.

Pancreatic regeneration after ethionine-induced acute pancreatitis in rats lacking pancreatic CCK-A receptor gene expression.

BACKGROUND: We examined the effects of cholecystokinin (CCK) on the development of ethionine-induced pancreatitis and pancreatic recovery. We used Otsuka Long-Evans Tokushima Fatty (OLETF) rats, a model lacking pancreatic CCK-A receptor gene expression. METHODS: Ethionine-induced pancreatitis was induced in the 7-week-old male OLETF rats and in a control group that does not lack the pancreatic CCK-A receptor, Long-Evans Tokushima Otsuka (LETO) rats. The two groups were maintained on a low-protein diet for 11 days. During the last 4 days of the low-protein diet, dl-ethionine 20 mg/100 g body weight was administered intraperitoneally once daily. Histologic and biochemical examinations of the pancreas were performed, and plasma CCK concentrations were measured on days 1, 4, and 7 after the last ethionine administration. RESULTS: Pancreatic histologic scores for inflammation, hemorrhage, and necrosis in the LETO and OLETF rats were highest on days 1 and 4, respectively. Pancreatic weight, DNA content, and protein level per DNA content in both groups decreased during the low-protein diet, and recovery signs were delayed in the OLETF rats. The highest plasma CCK concentrations in the LETO and OLETF rats were reached on days 1 and 4, respectively. CONCLUSIONS: Ethionine-induced pancreatitis developed in the OLETF rats, and their pancreatic regeneration was delayed in comparison to that in the LETO rats. Our results suggested that CCK plays an important role in the development of pancreatitis as well as in the pancreatic repair process.