Prediction of the Probability of Malignancy in Mucinous Cystic Neoplasm of the Pancreas With Ovarian-Type Stroma: A Nationwide Multicenter Study in Japan.

OBJECTIVE: The aim of the study was to develop a formula for predicting the probability of malignancy of mucinous cystic neoplasm (MCN) of the pancreas with ovarian-type stroma. METHODS: A total of 364 patients were enrolled. A total score was calculated as the sum of the approximate integers of the odds ratios of the predictive factors identified by multivariate analysis. The relationship between the total score and pathological results was assessed. RESULTS: A total of 321 patients had benign MCN and 43 had malignant MCN. Five possible predictive factors were analyzed: 56 years or older, high serum carcinoembryonic antigen level, high carbohydrate antigen 19-9 level, tumor size of 51 mm or greater, and the presence of mural nodules. The total score was significantly higher in patients with malignant MCN (median, 24; range, 0-37) compared with benign MCN (median, 5; range, 0-33; P < 0.001). Receiver operating characteristic curve analysis demonstrated that the area under the curve was 0.86, and the sensitivity and specificity of the total score for discriminating malignant MCNs were 72% and 83%, respectively, using a cut-off value of 22. CONCLUSIONS: The current simple formula can predict the malignancy of MCN and may thus contribute to the adequate management of patients with MCN.

A case of poorly differentiated mucinous carcinoma of the gallbladder that exhibited a peculiar morphology.

A 65-year-old female presented with an elevated lesion that was identified in the gallbladder fundus via abdominal ultrasound during a medical checkup. The tumor was a pedunculated lesion, measuring 30mm in diameter, that exhibited a blood flow pattern with gradual dense staining throughout the equilibrium phase on the abdominal contrast computed tomography and a high signal intensity on T2-weighted magnetic resonance imaging. Histopathological findings revealed the proliferation of poorly differentiated adenocarcinoma, including signet ring cells, throughout the tumor along with the formation of a mucous lake. The patient was consequently diagnosed with poorly differentiated mucinous carcinoma of the gallbladder.

No Benefit of Oral Diclofenac on Post-Endoscopic Retrograde Cholangiopancreatography Pancreatitis.

BACKGROUND: Pancreatitis following endoscopic retrograde cholangiopancreatography (ERCP) is a serious complication. Rectal diclofenac (100 mg) has been shown to reduce the incidence of pancreatitis; however, this dosage form is unavailable in several countries. AIMS: We aimed to investigate the preventive effect of oral diclofenac on pancreatitis after ERCP in a multicenter, randomized, prospective, placebo-controlled, double-blind trial. METHODS: Patients undergoing a first ERCP in seven high-volume centers between July 2012 and August 2014 were considered eligible. Participants were administered oral diclofenac (50 mg) or placebo before and after ERCP. The primary endpoint was the incidence of pancreatitis. A subgroup analysis was performed for patients at high or low risk of pancreatitis. Secondary endpoints were pancreatic enzyme levels (amylase and lipase). RESULTS: We initially enrolled 430 patients (216 in the diclofenac and 214 in the placebo group), and 23 were excluded after randomization. The overall incidence of pancreatitis was 9.8 % (20/205) and 9.4 % (19/202) in the diclofenac and placebo groups, respectively (p = 0.90). The incidence of pancreatitis was 20.3 % (13/64) and 21.3 % (13/61) in patients at high risk of pancreatitis (p = 0.78) and 5.0 % (7/141) and 4.3 % (6/141) in patients at low risk of pancreatitis in the diclofenac and placebo groups (p = 0.94), respectively. There were no significant differences in serum amylase and lipase levels between the two groups before and 24 h after ERCP. CONCLUSIONS: Oral administration of diclofenac before and after ERCP showed no benefit in the prevention of pancreatitis. CLINICAL TRIALS REGISTRY NO: UMIN000008109.

A case report of primary neuroendocrine carcinoma of the perihilar bile duct.

BACKGROUND: Although neuroendocrine tumors are most commonly found in the digestive system, neuroendocrine tumors originating from the bile duct are rare, and neuroendocrine carcinomas derived from the perihilar bile duct are extremely rare. This report presents the clinical course and clinicopathological features of neuroendocrine carcinomas arising from the extrahepatic bile duct. CASE PRESENTATION: A 70-year-old Japanese woman was preoperatively diagnosed with perihilar cholangiocarcinoma, and a radical resection with an extended left hepatic lobectomy and a choledochojejunostomy was performed. From the histopathological findings, we diagnosed the tumor as a neuroendocrine carcinoma of the bile duct (small cell type) with lymph node metastasis. The patient was treated with the same adjuvant chemotherapy as that used for small cell carcinoma of the lung. At 10 months after surgery, there was no recurrence of the disease. CONCLUSION: Neuroendocrine carcinoma of the extrahepatic biliary tracts is a very rare and highly malignant disease with a poor prognosis. A multidisciplinary approach could improve the prognosis for this neoplasm.

[A case of pancreatic neuroendocrine tumor presenting with main pancreatic duct invasion].

A 52-year-old male visited a local clinic with a subjective complaint of pain in the left side of his abdomen. Abdominal CT revealed the presence of a pancreatic body tumor. On EUS, the tumor presented hypoechoic signals with an obscure boundary, which continued from the pancreatic parenchyma to the inside of the main pancreatic duct. Abdominal contrast CT revealed a hypervascular tumor with densely stained pancreatic parenchyma. ERP findings revealed that main pancreatic duct invasion was suspected based on partial radiolucency in the duct. Distal pancreatectomy was performed, and a definitive diagnosis of pancreatic neuroendocrine tumor (WHO class G1) was made histopathologically.

Localized type 1 autoimmune pancreatitis superimposed upon preexisting intraductal papillary mucinous neoplasms.

A 70-year-old woman was found to have 2 cystic lesions in the head of the pancreas on abdominal ultrasonography during a routine medical examination. Endoscopic ultrasonography (EUS) and magnetic resonance cholangiopancreatography showed multilocular cysts in the head of the pancreas without dilation of the main pancreatic duct. The patient was followed-up semiannually with imaging studies for suspected branch duct-type intraductal papillary mucinous neoplasm (IPMN). At 3 years after initial presentation, hypoechoic lesions were observed around each pancreatic cyst by EUS. Diffusion-weighted imaging showed high-intensity regions corresponding to these lesions. Therefore, a diagnosis of invasive carcinoma derived from IPMN could not be excluded, and subtotal stomach-preserving pancreaticoduodenectomy was performed. The macroscopic examination of the surgical specimen showed whitish solid masses in the head of the pancreas, with multilocular cysts within each mass. Microscopically, each solid mass consisted of inflammatory cells such as lymphocytes and plasma cells. Furthermore, immunochemical staining revealed immunoglobulin G4-positive cells, and many obliterating phlebitides were observed. The cysts consisted of mucus-producing epithelial cells and showed a papillary growth pattern. Based on these findings, we diagnosed multiple localized type 1 autoimmune pancreatitis occurring only in the vicinity of the branch duct-type IPMN.

[A case of dermoid cyst of the pancreas].

A 58-year-old man visited a local clinic after experiencing epigastric and dorsal pain. A computed tomography scan revealed a hypovascular mass in the pancreatic tail. T1-weighted magnetic resonance imaging (MRI) of the tumor showed a heterogeneous isointense signal and T2-weighted MRI showed a hyperintense signal. Contrast studies revealed contrast enhancement, showing a cystic wall and parts of a septal structure and lumen. Endoscopic ultrasonography revealed a multilocular hypoechoic tumor containing large quantity of debris. We experienced difficulty arriving at a diagnosis and are reporting this case of a pancreatic dermoid cyst, which was diagnosed based on pathological findings.

Acute pancreatitis caused by an ectopic mediastinal parathyroid adenoma.

A 22-year-old man was admitted to our hospital because of epigastric pain. Blood tests showed leukocytosis (8940 cells/mm(3)) and increased serum amylase levels (787 IU/L); an abdominal computed tomography (CT) scan revealed an enlarged pancreas and peripancreatic fluid collection. On the basis of these findings, he was diagnosed with acute pancreatitis. Hypercalcemia (13.5 mg/dL) and increased levels of parathormone (>3200 pg/dL) were also detected using a high-sensitivity assay; we therefore considered hypercalcemia and primary hyperparathyroidism to be the possible causes of the acute pancreatitis. A (99m)Tc-sestamibi scan showed accumulation of parathyroid tissue in the left mediastinum, and a tumor was noted on the left side of the aortic arch on a thoracic CT scan. Our final diagnosis was acute pancreatitis due to hypercalcemia induced by an ectopic mediastinal parathyroid adenoma. Ectopic parathyroid tumors can thus cause acute pancreatitis, and (99m)Tc-sestamibi and CT scans are useful for their diagnosis and localization.

Intraductal tubulopapillary neoplasm of the pancreas with somatic BRAF mutation.

Intraductal tubulopapillary neoplasm (ITPN) is a rare pancreatic tumor belonging to a newly recognized entity that is coined in the 2010 WHO classification. We present a case of ITPN-associated microinvasive adenocarcinoma that developed in an asymptomatic 78-year-old patient. The tumor demonstrated all the clinical, radiological, and pathological characteristics of ITPN, but it differs from other reported cases of ITPN in molecular analysis, which revealed a somatic mutation in BRAF (c.1799T>A; p.V600E) but no mutation in PIK3CA. Post-operative recurrence was discovered 34 months after tumor resection with negative margins and a 6-month course of adjuvant chemotherapy. To the best of our knowledge, this is the first case of ITPN with BRAF mutation. This case suggests that an activation of RAS-mitogen-activated protein kinase signaling pathway may play a role in development of some of ITPNs. A possible mechanism of tumor recurrence in ITPN is also discussed. Further case series with molecular study are awaited to delineate the clinicopathological and molecular characteristics of ITPN.

[One case of pancreatic mucinous carcinoma discovered due to acute pancreatitis].

The patient was a woman, aged 69, diagnosed with acute pancreatitis by a local physician; simultaneously, with US, a low-echo tumor was indicated in the pancreas' uncinate process. Diagnosis was made of acute pancreatitis resulting from a pancreatic IPMN, and the patient was referred. Ultrasound showed hypoechoic tumor images accompanied by posterior echo enhancement. With radiography-CT, from the pancreas parenchymal phase, the peripheral portion was densely stained, while internally, images showed densely stained dendriforms towards the equilibrium phase. With MRI T1-weighted images, there was appearance at low intensity, and with T2-weighted images, there was appearance at high intensity; with MRCP, there was depiction at relatively high intensity. In the final pathological diagnosis, there was prominent formation of mucinous nodules, and mucinous carcinoma including large quantity of mucous.

[Clinical features of acute acalculous cholecystits-nosocomial manner and community-acquired manner].

To clarify the clinical features of acute acalculous cholecystitis (AAC), we reviewed 113 cases of AAC experienced from January, 2000 to December, 2007 in regard to patient background, etiology, imaging diagnosis, treatment, and outcomes. Functional bile stasis caused AAC in 34 of 113 cases (30.1%), and 28 of those developed in a nosocomial manner (82.4%). On the contrary, mechanical bile stasis caused 37 cases (32.7%), and all but one case developed in a community-acquired manner. mechanical bile stasis included 9 cases of biliary tract neoplasm (5, cystic duct cancer; 3, gallbladder cancer; 1, papillary adenoma). Since considerable cases of AAC were caused by biliary tract neoplasm, we have to pay attention to their existences.

Preoperative biliary drainage for hilar cholangiocarcinoma.

Hilar cholangiocarcinomas grow slowly, and metastases occur late in the natural history. Surgical cure and long-term survival have been demonstrated, when resection margins are clear. Preoperative biliary drainage has been proposed as a way to improve liver function before surgery, and to reduce post-surgical complications. Percutaneous transhepatic biliary drainage (PTBD) with multiple drains was previously the preferred method for the preoperative relief of obstructive jaundice. However, the introduction of percutaneous transhepatic portal vein embolization (PTPE) and wider resection has changed preoperative drainage strategies. Drainage is currently performed only for liver lobes that will remain after resection, and for areas of segmental cholangitis. Endoscopic biliary drainage (EBD) is less invasive than PTBD. Among EBD techniques, endoscopic nasobiliary drainage (ENBD) is preferable to endoscopic biliary stenting (EBS), because secondary cholangitis (due to the retrograde flow of duodenal fluid into the biliary tree) does not occur. ENBD needs to be converted to PTBD in patients with segmental cholangitis, those with a prolonged need for drainage, or when the extent of longitudinal tumor extension is not sufficiently well characterized.