A case of mammary-type myofibroblastoma of the inguinal region.

INTRODUCTION: Myofibroblastoma is usually occurred in the breast and extra-mammary disease is rare. PRESENTATION OF CASE: A 38-year-old man was admitted to our hospital for further examination of the left inguinal tumour, present and enlarging for 16 months. The tumor was 50 mm in diameter, well-circumscribed, firm, and painless. Ultrasonogaphy, computed tomography(CT) and magnetic resonance imaging (MRI) could not provide the definitive diagnosis. Surgical exploration confirmed a 50 mm tumour with a clear surface with a thin capsule. Complete excision was achieved. Histopathology confirmed the tumor had oval and spindle shaped fibroblastic cells with rich collagen deposition. It stained positive for ER, CD34, desmin and CD10 but negative for -smooth muscle actin and S-100. A mammary-type myofibroblastoma was diagnosed based on these results. DISCUSSION: An extra-mammary myofibroblastoma is very rare and first reported in 2001. Since then, over 160 cases have been reported. On immunohistochemistry, these lesions are characteristically positive for CD34 and desmin, with variable staining for α-smooth muscle actin. Once the diagnosis is made, regardless of size or location, this tumour behaves in a benign fashion after surgical excision reported so far. CONCLUSION: This case is rare, but the correct diagnosis and treatment is important for good prognosis.

Extraperitoneal Fluid Collection due to Chronic Pancreatitis.

A 39-year-old man was referred to our hospital for the investigation of abdominal fluid collection. He was pointed out to have alcoholic chronic pancreatitis. Laboratory data showed inflammation and slightly elevated serum direct bilirubin and amylase. An abdominal computed tomography demonstrated huge fluid collection, multiple pancreatic pseudocysts and pancreatic calcification. The fluid showed a high level of amylase at 4,490 IU/l. Under the diagnosis of pancreatic ascites, endoscopic pancreatic stent insertion was attempted but was unsuccessful, so surgical treatment (Frey procedure and cystojejunostomy) was performed. During the operation, a huge amount of fluid containing bile acid (amylase at 1,474 IU/l and bilirubin at 13.5 mg/dl) was found to exist in the extraperitoneal space (over the peritoneum), but no ascites was found. His postoperative course was uneventful and he shows no recurrence of the fluid. Pancreatic ascites is thought to result from the disruption of the main pancreatic duct, the rupture of a pancreatic pseudocyst, or possibly leakage from an unknown site. In our extremely rare case, the pancreatic pseudocyst penetrated into the hepatoduodenal ligament with communication to the common bile duct, and the fluid flowed into the round ligament of the liver and next into the extraperitoneal space.

Intrapancreatic gastric duplication cyst mimicking pancreatic cystic tumor.

Cystic lesions of the pancreas are sometimes difficult to diagnose. We report a case of a gastric duplication cyst (GDC) of the pancreas in an adult. A 45-year-old woman was admitted to our department for the investigation of anemia. Abdominal ultrasonography revealed a large cystic lesion, measuring about 40 × 70 mm with calcification in the tail of the pancreas. Contrast-enhanced computed tomography of the abdomen revealed a non-enhanced cystic lesion with non-enhanced wall. Endoscopic retrograde pancreatography revealed a mild extended main pancreatic duct and an aberrant pancreatic duct, but there was no communication with the cyst. We could not deny the malignancy, so distal pancreatectomy was performed. The pathological examination revealed that the inner wall of the cyst consisted of columnar epithelium and smooth muscle layer. Immunohistochemical analysis revealed the columnar epithelium to be immunopositive for cytokeratin 7 (CK7) and immunonegative for cytokeratin 20 (CK20) and the glands to be immunonegative for CK 7 and immunopositive for CK 20. Therefore, a diagnosis of GDC of the pancreas was made. On imaging, a GDC usually mimics a pancreatic pseudocyst or a cystic neoplasm of the pancreas. Therefore, even though it is rare, a GDC should be considered in the differential diagnosis of cystic tumors of the pancreas.

Carcinoid tumor of the extrahepatic bile duct: report of a case.

We report a rare case of carcinoid tumor of the extrahepatic bile duct. A 69-year-old woman with a history of hyperthyroidism was diagnosed to have a tumor of the extrahepatic bile duct. Laparotomy, for presumed cholangiocarcinoma, revealed a 2.5-cm-long, firm mass of the hilar-upper bile duct. The extrahepatic bile duct resection and lymphadenectomy was performed. Her postoperative course was uneventful and has been asymptomatic without recurrent tumor during 2 years of follow-up. Primary carcinoid tumors of the extrahepatic bile duct are very rare. Herein we report this rare case with a review of the literature.

A Case of Adenocarcinoma of the Duodenum Arising from Brunner's Gland.

We report a rare case of adenocarcinoma of the duodenum arising from Brunner's gland. A 70-year-old man with a history of hypertension was referred to us with the complaint of abdominal discomfort. Upper gastrointestinal endoscopy revealed an irregular elevated mucosa and a submucosal tumor with delle in the duodenal bulb. Biopsy specimens revealed adenocarcinoma (the former) and hyperplasia (the latter). We could not agree with the patient about performing pancreaticoduodenectomy, so under the diagnosis of primary duodenal carcinoma, we performed resection of the bulbus and the antrum. Pathological examination showed that one of the tumors was consistent with normal Brunner's glands, Brunner's gland hyperplasia and adenocarcinoma arising from Brunner's glands. The patient's postoperative course was good, but 15 months after, he developed lymph node recurrence at the site of the pancreas head. Under the diagnosis of lymph node metastasis of duodenal cancer, we performed pancreaticoduodenectomy this time. Pathological examination confirmed our preoperative diagnosis. The patient remained well after the surgery for 2 years.

Two cases of hepatocellular carcinoma located adjacent to the Glisson's capsule treated by laparoscopic radiofrequency ablation with intraductal chilled saline perfusion through an endoscopic nasobiliary drainage tube.

Radiofrequency ablation (RFA) is a commonly used local therapy for hepatocellular carcinoma (HCC). However, for tumors located adjacent to the Glisson's capsule in the hepatic hilar region, RFA may cause bile duct injury and may be difficult to perform using the standard procedure. We describe 2 HCC cases in which RFA was performed laparoscopically under general anesthesia while cooling bile ducts to prevent bile duct injury. An endoscopic nasobiliary drainage tube was preoperatively inserted, through which chilled saline was rapidly infused during laparoscopic RFA for HCC adjacent to the Glisson's capsule in the hepatic hilar region. The patient was discharged from hospital without intraoperative or postoperative complications. Follow-up contrast-enhanced CT revealed complete tumor cauterization and no evidence of late bile duct stenosis. This procedure is performed under general anesthesia and, unlike those performed under local anesthesia, is associated with minimal stress to patients and minimal risk of bile duct injury.

Role of triggering receptor expressed on myeloid cells-1 in experimental severe acute pancreatitis.

BACKGROUND/PURPOSE: Triggering receptor expressed on myeloid cells-1 (TREM-1) is a regulator of immunity and an amplifier of inflammatory signaling. The aim was to clarify the role of TREM-1 in the pathophysiology of experimental severe acute pancreatitis (SAP). METHODS: SAP was induced by retrograde injection of 3 and 20% sodium deoxycholate (DCA) into the biliopancreatic ducts in rats (DCA pancreatitis). Soluble TREM-1 levels in serum, ascitic fluid, pancreas, liver and kidney were determined with an established available enzyme-linked immunosorbent assay (ELISA) kit. To clarify the source of soluble TREM-1 in serum and ascitic fluid, peritoneal macrophage depletion was done. Moreover, the effect of blockade of TREM-1 pathway was examined using LP17 (a synthetic TREM-1 inhibitor). RESULTS: Soluble TREM-1 levels in serum and ascitic fluid were higher in SAP. Membrane-bound TREM-1 protein was increased in pancreas, liver and kidney in SAP. Peritoneal macrophage depletion resulted in the reduction of soluble TREM-1 levels in serum and ascitic fluid. Pretreatment with LP17 improved the hepatic and renal dysfunction (serum aspartate aminotransferase and blood urea nitrogen levels) in SAP. CONCLUSIONS: TREM-1 may act as an important mediator for inflammation and organ injury in SAP. TREM-1 may be a potential therapeutic target for the development of SAP and associated organ dysfunction.

Early infection of peripancreatic tissue in mild acute pancreatitis: report of a case.

Mild acute pancreatitis (AP) is rarely complicated by infection, and the value of prophylactic antibiotics is questionable. We report a case of mild AP complicated by infection, which developed within 1 week after the onset. A 66-year-old woman was referred to our hospital where a diagnosis of mild AP was made, based on laboratory data and computed tomography (CT) findings. She was managed conservatively with fluid resuscitation, intravenous antibiotics, and protease inhibitor. Her general condition improved initially, but a high fever redeveloped on hospital day 3. On hospital day 7, a repeat CT scan showed a peripancreatic fluid collection with gas, indicating peripancreatic abscess. A drainage operation was performed, and the organism cultured from the abscess was Escherichia coli. Her postoperative course was uneventful. We report this case to stress that infection may develop even in mild AP, and even in the early phase.

Plasma tissue factor pathway inhibitor levels in patients with acute pancreatitis.

BACKGROUND: In acute pancreatitis (AP), disorders of the coagulation-fibrinolysis system are closely related to the severity of the AP and to organ dysfunctions. We previously reported that plasma tissue factor (TF) levels were elevated in patients with AP, particularly in cases of alcoholic AP with pancreatic necrosis. Tissue factor pathway inhibitor (TFPI) is a key regulator of the extrinsic coagulation pathway, but plasma TFPI levels in AP have not yet been determined. METHODS: Plasma TFPI concentrations were measured by enzyme-linked immunosorbent assay in 44 patients with AP on admission. The relationships between AP severity, pancreatic necrosis, organ dysfunction, infection, and prognosis were analyzed. RESULTS: Plasma TFPI levels were increased in AP patients compared with healthy volunteers. Plasma TFPI levels in severe AP were greater than those in mild AP. Plasma TFPI levels significantly correlated with Ranson score, APACHE II score, and Japanese severity score. Plasma TFPI levels in patients with pancreatic necrosis were greater than those in patients without pancreatic necrosis. Plasma TFPI levels in patients with organ dysfunction were greater than those in patients without organ dysfunction. In patients with pancreatic necrosis, the TF/TFPI ratios in non-survivors were lower than those in survivors. Moreover, the mortality rates in patients with TF/TFPI ratios > or = 2.0 were lower than those in patients with TF/TFPI ratios < 2.0. CONCLUSIONS: Plasma TFPI levels were significantly increased in patients with AP, and the elevation was markedly related to the severity, pancreatic necrosis and organ dysfunctions. The imbalance of TF and TFPI may influence the disease state and thereby the prognosis in AP.

Utility of the new Japanese severity score and indications for special therapies in acute pancreatitis.

BACKGROUND: The Japanese severity score (JSS) for acute pancreatitis was revised in 2008. As special therapies for severe acute pancreatitis (SAP), continuous regional arterial infusion of protease inhibitor and antibiotics (CRAI) and enteral nutrition (EN) are now utilized in Japan. We investigated the usefulness of the new JSS and the indications for CRAI and EN based on the new JSS. METHODS: We assessed the new JSS in 138 patients with SAP according to the previous Japanese criteria. Usefulness of the new JSS for the prediction of mortality rates was compared with conventional scoring systems by receiver-operator characteristic curve analysis. We analyzed the relationship between the new JSS and prognosis in patients with and without CRAI and EN, respectively. RESULTS: Forty-five patients (33%) were assessed as having mild acute pancreatitis, and 93 patients (67%) were assessed as having SAP. Their mortality rates were 7 and 40%, respectively. The area under the curve for the prediction of mortality rates with the new JSS was 0.822 and was the highest among conventional scoring systems. In patients with new JSS >or= 6, the mortality rate was lower in patients with CRAI than in patients without CRAI (P = 0.129). In patients with new JSS >or= 4, the mortality rate was lower in patients with EN than in patients without EN (P = 0.016). CONCLUSIONS: The new JSS is useful and easier to use for the prediction of prognosis compared to the conventional scoring systems. EN was effective in reducing the mortality rate in patients with a new JSS >or= 4.

Diagnostic and prognostic value of peritoneal cytology in biliary tract cancer.

BACKGROUND/AIMS: Evaluation of peritoneal cytology provides valuable prognostic information in abdominal cancers. The aim of this study is to assess the incidence and the prognostic value of conventional peritoneal cytology in biliary tract cancers. METHODOLOGY: A total of consecutive 41 patients with biliary cancers (17 bile duct, 20 gallbladder, 4 ampulla of Vater) underwent surgery between July 2003 and July 2005. Peritoneal cytology was performed in these patients at the beginning of laparotomy. On opening the abdomen, 100mL of normal saline were instilled into the subhepatic space and retrieved. Cytologic analysis was performed using the Papanicolau technique. RESULTS: The overall incidence of positive cytology findings was 9.8% (4/41). When analyzed by disease factors or stage, the prevalence of positive cytology was 0% in T1/T2, 6% in T3, 38% in T4 (p=0.03), 0% in N0, 25% in N1 (p=.03), 3% in M0, 27% in M1 (p=0.02), 0% in Stage I/II and 27% in Stage III/IV cases (p<0.01), respectively. Although survival was worse in patients with positive peritoneal cytology, when adjusting TNM stage the positive peritoneal cytology did not have significant prognostic value. CONCLUSIONS: Peritoneal cytology associates positively with advanced disease but does not increase prognostic information in biliary tract cancers.

Long-term outcome of severe acute pancreatitis.

BACKGROUND/PURPOSE: This study was undertaken to evaluate the post-discharge outcome of severe acute pancreatitis (SAP) and to clarify the prognostic factors for poor outcome. METHODS: In 45 patients, recurrence of acute pancreatitis (AP), transition to chronic pancreatitis (CP), and development of diabetes mellitus (DM) were evaluated. Relationships of the outcome with the findings on admission and the presence/absence of alcohol intake were analyzed. RESULTS: The mean follow-up period was 56+/-6 months. Recurrence of AP was noted in 19% of the patients. The recurrence rate was higher in patients with necrotizing pancreatitis than in those without this feature. C-reactive protein and white blood cell (WBC) count were higher in patients with recurrence of AP. Transition to CP was noted in 22% of patients. The transition rate was higher in those with alcoholic SAP than in those with biliary SAP. In patients with transition to CP, the WBC count, hematocrit, Ranson score, and Japanese severity score were higher, and base excess (BE) was lower, compared with these features in patients without this transition. Development of DM was noted in 39% of patients. Blood glucose and BE were higher in patients who developed DM than in those who did not. CONCLUSIONS: The degree of inflammation and pancreatic necrosis found on admission for SAP may be related to the recurrence of AP. Alcoholic SAP in which the disease is very severe may contribute to the transition to CP. Patients with impaired glucose tolerance readily develop DM after SAP.

Increased levels of soluble triggering receptor expressed on myeloid cells-1 in patients with acute pancreatitis.

OBJECTIVE: To determine the contribution of triggering receptor expressed on myeloid cells (TREM)-1 in acute pancreatitis (AP). DESIGN: Prospective study. SETTING: General intensive care unit at Kobe University Hospital. PATIENTS: Forty-eight patients with AP and seven patients as control. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: We measured serum concentrations of soluble TREM-1 (sTREM-1) at the time of admission by enzyme-linked immunoadsorbent assay. Serum sTREM-1 levels increased significantly in AP (63 +/- 11 pg/mL) and correlated with Ranson score (R = .628, p < .001) and Acute Physiology and Chronic Health Evaluation II score (R = .504, p < .001). Serum TREM-1 levels were higher in patients with early organ dysfunction (which occurred within 7 days after onset) than those without early organ dysfunction (101 +/- 19 vs. 25 +/- 4 pg/mL, p < .001). Incidences of early organ dysfunction in patients whose serum sTREM-1 levels were < or = 40 and > 40 pg/mL were 17% and 83%, respectively (p < .001). The usefulness of serum sTREM-1 in detecting early organ dysfunction was superior to that of C-reactive protein, interleukin-6, interleukin-8, Ranson score, and Acute Physiology and Chronic Health Evaluation II score. Serum sTREM-1 levels decreased with resolution of early organ dysfunction. CONCLUSIONS: Serum sTREM-1 levels were significantly increased and correlated with disease severity and early organ dysfunction in patients with AP. Serum sTREM-1 level may be a useful marker for early organ dysfunction in AP.

Prediction of early death in severe acute pancreatitis.

BACKGROUND: In severe acute pancreatitis (SAP), it is clinically important at the time of admission to predict the likelihood of early death. This investigation aimed to clarify the factors predicting early death in SAP. METHODS: Early death was defined as death within 10 days after disease onset. Prediction factors for early death were evaluated from data obtained on admission from 93 patients with SAP, and the characteristics of patients who died early were analyzed. RESULTS: Between the early-death and early-survival groups, significant factors were base excess (BE), serum creatinine (Cr), blood sugar, serum glutamate oxaloacetic transaminase, and serum calcium. Multivariate analysis revealed that BE was an independent prediction factor for early death. The early-death rate in patients with BE < -5.5 mEq/l and Cr >or= 3.0 mg/dl was 31% and 36%, respectively. The combination of BE and Cr raised the positive predictive value to 50%, and was equally able to predict early death as the Japanese Severity Score (JSS), which was the most useful of the three conventional scoring systems used. All early-death patients had pancreatic necrosis, and their JSS was >or= 15 (stage 4). Characteristically, early-death patients had lactate dehydrogenase (LDH)>1300 IU/l, or they had serious preexisting comorbidities. CONCLUSIONS: As a single parameter, BE was most useful for predicting early death. The combination of BE and Cr could predict early death as well as the JSS. An extreme rise of LDH and serious preexisting comorbidity may also be risk factors for early death.

Histological features of lymph node metastasis in patients with biliary tract cancer.

BACKGROUND AND OBJECTIVES: The presence of lymph node (LN) metastases is an important prognostic factor in patients with biliary cancers. The aim of this study was to characterize systematically the morphological features of metastatic LNs in biliary cancers. METHODS: Four hundred ninety-six LNs (including 112 para-aortic LNs) dissected from 47 patients with biliary cancer were examined. The diameter of the long axis (size) and the percent metastatic area relative to whole-node area were measured from histologic specimens. RESULTS: The average size of metastatic LNs (9.5 mm) was significantly larger than those without metastasis (6.5 mm; P < 0.01). The optimum cut-off size for positive LNs was >7.5 mm, but the sensitivity of this predictor of metastasis was low (60.8%). In general, metastatic area correlated significantly with the size of metastatic LNs (P = 0.023). Para-aortic LNs contained metastasis in 7.1% of cases, and only 25% of para-aortic LNs with a high ratio of metastatic area could be evaluated from preoperative CT scans. CONCLUSIONS: Although large LNs are highly suggestive of metastasis, poor detection of many small LNs with a low percentage of metastatic area can increase risk in patients with biliary tract cancer.

Expression of toll-like receptor 2 and 4 in intestinal mucosa in experimental severe acute pancreatitis.

BACKGROUND/AIMS: In severe acute pancreatitis (SAP), endotoxin/bacterial translocation (E/BT) from the gut is main cause of infection, which is contributor to high mortality. Toll-like receptors (TLRs) recognize micro-organisms and are involved in the host defense. This study aimed to investigate the expression of TLR2 and TLR4 in intestinal mucosa in SAP. METHODOLOGY: SAP was induced by injection of 3% sodium deoxycholate into the biliopancreatic duct in rats. Terminal ileum was removed 2, 6, 12, and 18 hours after the induction. Expression of TLR2/4 was analyzed by western blotting and immunohistochemistry. NF-kappaB activation was assessed by enzyme immunoassay. E/BT was also evaluated. RESULTS: TLR2/4 proteins were increased 2 and 6 hours, and were decreased 12 and 18 hours after the induction. Immunoreactivities for them were detected at the top of villi and crypt in control rats. They were increased 6 hours, and were diminished 12 hours after the induction. Activated NF-kappaB was increased 6 hours, and was decreased 18 hours after the induction. E/BT occurred 18 hours after the induction. CONCLUSIONS: Intestinal immune response enhances in the early phase and suppresses in the late phase of SAP, and TLRs may be implicated in the mechanism of E/BT.

Serum immunosuppressive acidic protein levels in patients with severe acute pancreatitis.

OBJECTIVES: In severe acute pancreatitis (SAP), immunologic impairment in the early phase may be linked to subsequent infectious complications. Immunosuppressive acidic protein (IAP) is an immunosuppressive factor to be present in serum and ascites of cancer patients, and it is used as a tumor marker and an index of immune status of cancer hosts. METHODS: We measured serum IAP levels in 42 patients with SAP (Japanese severity score [JSS] > or = 2) on admission and analyzed the relationships with disease severity, pancreatic necrosis, blood biochemical parameters on admission, and clinical outcome (infection and death). RESULTS: Serum IAP level increased 791 +/- 285 microg/mL (range, 159-1430 microg/mL) on admission and recognized abnormal high level (normal range, < 500 microg/mL) in 37 patients (88.1%). Serum IAP level was significantly lower in patients of stages 3 and 4 (JSS > or = 9) (678 +/- 187 microg/mL) than that in patients of stage 2 (2 < or = JSS < or = 8) (848 +/- 311 microg/mL). It was also significantly lower in patients whose Ranson score was 5 or higher (674 +/- 287 microg/mL) than that in patients whose Ranson score was 4 or less (910 +/- 287 microg/mL). Moreover, it was significantly lower in patients with pancreatic necrosis (693 +/- 194 microg/mL) than that in patients without pancreatic necrosis (922 +/- 336 microg/mL). Among the blood biochemical parameters on admission, serum IAP was significantly negatively correlated with hematocrit, serum lipase, and serum interferon gamma and was significantly positively correlated with serum total protein. Serum IAP levels in patients of stage 2 reached higher peak at 7 days after admission and decreased more rapidly than those in patients of stages 3 and 4. CONCLUSIONS: Serum IAP levels were elevated in patients with SAP but were significantly lower in patients with higher grade of severity or pancreatic necrosis. These results suggest that serum IAP levels may be related to systemic inflammatory response and reflect the immunoresponsiveness in patients with SAP.