Difficulty Achieving a Preoperative Diagnosis of IgG4-Related Sclerosing Cholangitis.

A 75-year-old male was admitted to our hospital because of bile duct stenosis. He had no medical history of autoimmune disease. The level of tumor markers, serum IgG, and IgG4 were within normal ranges. Computed tomography showed perihilar and distal bile duct stenosis and wall thickening without swelling or abnormal enhancement of the pancreas. Endoscopic retrograde cholangiopancreatography showed perihilar and distal bile duct stenosis. A biopsy and cytology from the distal bile duct stenosis suggested adenocarcinoma, and cytology from the perihilar bile duct also suggested adenocarcinoma. A preoperative diagnosis of perihilar and distal bile duct cancer was made, and the patient underwent left hepatectomy and pancreaticoduodenectomy. Resected specimens showed wall thickening in the perihilar and distal bile duct; however, tumors were unclear. A histopathological examination revealed lymphoplasmacytic infiltration, storiform fibrosis, and obliterative phlebitis in the perihilar and distal bile ducts. Immunohistochemistry revealed diffuse infiltration of IgG4-positive plasma cells in the perihilar and distal bile ducts. Lymphoplasmacytic infiltration, inflammatory change, storiform fibrosis, and obliterative phlebitis were shown in the pancreas. A final diagnosis of IgG4-related sclerosing cholangitis (IgG4-SC) with autoimmune pancreatitis was made. We herein report a case in which a preoperative diagnosis of IgG4-SC was difficult due to normal serum IgG4 levels and no obvious pancreatic lesion.

Impaired Value of 99m Tc-GSA Scintigraphy as an Independent Risk Factor for Posthepatectomy Liver Failure in Patients with Hepatocellular Carcinoma.

BACKGROUND: Posthepatectomy liver failure (PHLF) was recently defined with the corresponding recommendations as follows: grade A, no change in clinical management; grade B, clinical management with noninvasive treatment; and grade C, clinical management with invasive treatment. In this study, we identified the risk factors for grade B and C PHLF in patients with hepatocellular carcinoma (HCC). METHODS: Of 339 HCC patients who underwent curative hepatic resection, 218 were included for analysis. The LHL15 index (uptake ratio of the liver to that of the liver and heart at 15 min) was measured by 99m Tc-GSA (99m technetium-labelled galactosyl human serum albumin); remnant LHL15 was calculated as LHL15 × [1 - (resected liver weight - tumor volume)/whole liver volume without tumor]. RESULTS: A total of 163 patients were classified as having no PHLF, whereas 17, 37, and 1 patient had PHLF grade A, B, and C, respectively. There were significant differences in indocyanine green R15, serum albumin, prothrombin time, Child-Pugh classification, LHL15 and remnant LHL15 between patients with grades B/C PHLF and patients with grade A or no PHLF. Only remnant LHL15 was identified as an independent risk factor for grades B/C PHLF (p = 0.023), with a cut-off value of 0.755. CONCLUSIONS: Remnant LHL15 was an independent risk factor for grades B/C PHLF. Patients with impaired remnant LHL15 value of <0.755 should be carefully monitored for PHLF.

Is Noncurative Hepatic Resection Justified for Advanced Hepatocellular Carcinoma?

It has been obscure whether or not noncurative hepatic resection (Hx) has a favorable impact on the clinical course in patients with advanced hepatocellular carcinoma (HCC). The aim of this study is to clarify the significance of noncurative Hx for advanced HCC. Among 666 consecutive patients undergoing Hx for HCC in our department, 79 patients underwent noncurative Hx. These patients were classified as Group A (presence of macrovascular invasion [MVI]; n = 29), Group B (residual tumors in the remnant liver; n = 37), Group C (residual tumors in the remnant liver with MVI; n = 7), or Group D (residual tumors in the remnant liver with distant metastasis [with or without MVI]; n = 6). The three-year survival rates were 49.6 per cent in Group A, 30.3 per cent in Group B, 14.3 per cent in Group C, and 0.0 per cent in Group D, respectively (Groups A and B vs Group D, P < 0.05). Moreover, the survival rate was significantly higher in patients with ≤3 tumors than in those with ≥4 tumors (P < 0.05), when Group B was divided into subgroups according to the number of residual tumors in the remnant liver. In conclusion, noncurative Hx might be acceptable for advanced HCC with MVI or ≤3 residual tumors in the remnant liver.

Short- and long-term outcomes of the Frey procedure for chronic pancreatitis: a single-center experience and summary of outcomes in Japan.

PURPOSE: To evaluate the short- and long-term outcomes of the Frey procedure for chronic pancreatitis (CP). METHODS: The subjects of this study were 12 patients who underwent the Frey procedure for CP between January, 2000 and December, 2016. We assessed pain relief, weight gain, and exocrine/endocrine insufficiency during follow-up. RESULTS: The study population comprised 11 men and 1 woman (91.7% vs. 8.3%; mean age, 50.3 ± 6.8 years; range 39-61 years). Pancreatitis was caused by alcohol in 9 (75%) patients and was idiopathic in 3 (25%) patients. The mean follow-up period was 82.5 ± 46.5 months (range 16.9-152.1 months). There was no operative mortality, but three patients (25%) suffered postoperative morbidity. All patients were pain-free at the time of discharge. There was no case of new-onset diabetes mellitus after surgery, although one patient (8.3%) suffered exocrine insufficiency. The body weight and body mass index of all patients improved during follow-up. Only one patient continued to suffer pain in the long term. CONCLUSION: The findings of this long-term follow-up of patients who underwent the Frey procedure suggest that it offers effective pain relief and is a safe technique for the management of CP.

Multifocal Mass Lesions in Autoimmune Pancreatitis.

A 59-year-old male patient with jaundice was referred to our hospital because of mass lesions in the pancreatic head and tail. An immunological examination revealed an elevated serum IgG4 level. Computed tomography showed two clear boundary mass lesions in the pancreatic head and tail. Magnetic resonance imaging showed that the mass lesions exhibited low intensity on T1-weighted images and iso-intensity on T2-weighted images. Magnetic resonance cholangiopancreatography showed an obstruction of the main pancreatic duct in the pancreatic head and tail. The possibility of malignant tumors could not be ruled out; therefore, we performed total pancreatectomy. A histopathological examination of the nodular lesions revealed severe lymphoplasmacytic infiltration and inflammatory change around the pancreatic ducts. Immunohistochemistry revealed diffuse infiltration of IgG4-positive plasma cells in the nodules. According to these pathological findings, we diagnosed the patient with IgG4-related multifocal mass lesions of autoimmune pancreatitis (AIP). It is difficult to distinguish between focal type AIP and pancreatic cancer. We herein report a rare case of multifocal mass lesions in AIP and include bibliographical comments.

Hepatectomy for Hilar Cholangiocarcinoma with Right-Sided Ligamentum Teres Using a Hepatectomy Simulation System.

Right-sided ligamentum teres (RSLT) is a rare congenital anomaly often accompanied by variation of the hepatic vasculature. We herein report a surgical case of a hilar cholangiocarcinoma with RSLT in whom preoperative hepatectomy simulation proved useful for understanding the anatomical structure of the liver. A 78-year-old male with obstructive jaundice was referred to our department for further examination. The patient was suspected of having a hilar cholangiocarcinoma originating from the left hepatic bile duct by contrast-enhanced computed tomography (CT), and CT also showed right umbilical portion (RUP). Three-dimensional images of the hepatic vasculature and biliary system reconstructed using a hepatectomy simulation system suggested that all portal branches ramified from RUP were right paramedian branches, and three leftward portal branches from these ran parallel to the peripheral bile ducts confluent with the left hepatic bile duct, where the tumor was present. Hepatic resection of part of the ventral area of the right paramedian sector and left hemiliver was performed along the demarcation line drawn after clamping the portal branches; the ratio of estimated liver resection volume was 28.9%. After the operation, bile leakage occurred. However, the leakage was treated with percutaneous drainage alone, and the patient was discharged 77 days after the operation. The patient is doing well without any signs of recurrence 21 months after the operation. The vascular and biliary anatomy in patients with RSLT is complicated and should be evaluated in detail preoperatively using a hepatectomy simulation system.

Laparoscopic Resection of an Epithelial Cyst in an Intrapancreatic Accessory Spleen.

An epithelial cyst in an intrapancreatic accessory spleen (ECIAS) is rare. We herein report a case of a patient with ECIAS who underwent laparoscopic surgery. A 57-year-old woman was referred to our hospital because of a pancreatic tail tumor. She was asymptomatic, and a physical examination revealed no remarkable abnormalities. The levels of the tumor marker carbohydrate antigen 19-9 (CA19-9) and s-pancreas-1 antigen (SPan-1) were elevated. Ultrasonography showed a well-defined homogeneous cystic tumor. Computed tomography showed a well-demarcated cystic tumor in the pancreatic tail. Magnetic resonance imaging showed that the cystic tumor exhibited low intensity on T1-weighted images and high intensity on T2-weighted images. The cystic tumor was diagnosed as mucinous cystic neoplasm preoperatively. The patient underwent laparoscopic spleen-preserving distal pancreatectomy. A histopathological examination revealed the cyst wall to be lined by stratified squamous epithelium within splenic parenchyma, and the ultimate diagnosis was ECIAS. The postoperative course was uneventful, and the patient was discharged on postoperative day 12. ECIAS is very difficult to diagnose preoperatively. Laparoscopic surgery is a safe and minimally invasive procedure for patients with difficult-to-diagnose pancreatic tail tumor suspected of having low-grade malignancy.

Bronchobiliary fistula caused after hepatectomy for hepatocellular carcinoma: a case report.

BACKGROUND: A bronchobiliary fistula, an intercommunication between the biliary tract and bronchial trees, is an extremely rare complication after hepatectomy. CASE PRESENTATION: A 70-year-old male underwent partial resection of the liver for recurrent hepatocellular carcinoma under a thoracoabdominal approach. The immediate postoperative clinical course was uneventful, but the patient was febrile and laboratory examinations revealed leukocytosis on the 15th postoperative day. An intraabdominal abscess was suspected based on the computed tomography findings, and percutaneous drainage was performed. Bile was drained, and fluoroscopy using a contrast medium from the drainage tube revealed a communication between the cavity and the common hepatic duct. Two weeks after drainage, bilioptysis was seen. Fistulography demonstrated the presence of the bronchus in the right lower lobe of the lung via the subphrenic space. Therefore, the patient was diagnosed to have a bronchobiliary fistula. Fistulography revealed closure of the communication with the bronchus about a month after drainage. However, the bile leakage and bilioptysis did not stop even after endoscopic nasogastric biliary drainage, and ethanol injection therapy were performed. Eventually, residual right bisectionectomy without resection of the fistulous tract and involved lung was performed to remedy the intractable bile leakage. The clinical course after the reoperation was good without bile leakage, bilioptysis, or pulmonary disorders, and the patient was discharged 40 days after reoperation. CONCLUSIONS: We experienced a rare case of bronchobiliary fistula that occurred after hepatectomy for hepatocellular carcinoma. Careful attention should be paid to prevent bile leakage during hepatectomy, since bile leakage has the potential to cause a bronchobiliary fistula.

Parenchyma-preserving hepatectomy based on portal ramification and perfusion of the right anterior section: preserving the ventral or dorsal area.

BACKGROUND: Anatomical hepatectomy aims to eliminate the spread of malignant tumor cells via portal vein systemically. An anatomical concept of the right anterior section (RAS) and preservation of the liver parenchyma within the RAS has been proposed. METHODS: We focused on the anatomical concept of the RAS based on portal perfusion and described surgical procedures to preserve the ventral or dorsal RAS using preoperative simulation. RESULTS: In 370 patients undergoing a preoperative simulation, the ramification of the tertiary portal branches of the RAS could be divided into three types including the cranio-caudal type; Couinaud's classification in 50% of patients, ventro-dorsal type in 26% of patients, and multiple type in 24% of patients. Then in 32 patients of the ventro-dorsal type, curative parenchyma-sparing hepatectomy of the RAS was performed, preserving the ventral and dorsal RAS in 14 and 18 patients, respectively. There were no differences in the postoperative complications and long-term survival compared with the results obtained after segment 5 or 8 resection (n = 33). CONCLUSION: Three-dimensional simulation revealed three types of portal vein ramification of the RAS. Parenchyma-preserving hepatectomy based on the precise portal ramification may contribute to safe and curative hepatectomy in selected cases with liver neoplasm involving the RAS.

Hepatic Pseudolymphoma with Fluorodeoxyglucose Uptake on Positron Emission Tomography.

A 69-year-old woman with chronic hepatitis B was admitted to our hospital with a hepatic tumor. The levels of 2 tumor markers, carcinoembryonic antigen and carbohydrate antigen 19-9, were slightly elevated; however, the α-fetoprotein and protein levels induced by vitamin K antagonist II were within the normal limits. Abdominal ultrasonography showed a well-defined peripheral hypoechoic mass that was isoechoic and homogeneous on the inside. Computed tomography showed a poorly enhanced tumor of 13 mm in diameter in the 5th segment of the liver. Fluorodeoxyglucose positron emission tomography showed a slight uptake (maximum standard uptake value 3.4) by the hepatic tumor. These findings suggested cholangiocellular carcinoma, and we performed anterior segmentectomy of the liver. A histopathological examination showed a hepatic pseudolymphoma. The patient's postoperative course was uneventful, and she remains alive without recurrence 5 months after undergoing surgery. In most cases, hepatic pseudolymphoma is preoperatively diagnosed as a malignant tumor and a definite diagnosis is made after resection. It is therefore necessary to consider hepatic pseudolymphoma as a differential diagnosis in patients with hepatic tumors.

Successful laparoscopic extirpation of peritoneal dissemination after hepatectomy for ruptured hepatocellular carcinoma.

Clinically, peritoneal dissemination of hepatocellular carcinoma (HCC) rarely occurs. We herein report a case that had a good outcome following laparoscopic extirpation of peritoneal dissemination after hepatectomy for ruptured HCC. A 66-year-old man underwent central bisectionectomy 12 days after emergency transcatheter arterial embolization for a ruptured HCC. Thereafter, pulmonary resection was performed twice for lung metastasis. About 8 months after the second pulmonary resection, a mass lesion was detected at the left subphrenic space on CT and (18) F-fluorodeoxyglucose PET scans. We made a diagnosis of peritoneal dissemination of HCC, and laparoscopic extirpation was performed. The patient is now doing well without any signs of recurrence 2 years after the last operation. Laparoscopic surgical resection for peritoneal dissemination that develops after hepatectomy for HCC may have a beneficial effect as a less-invasive approach and may improve the prognosis in select patients.

Development of hepatocellular carcinoma in cardiac congestive liver fibrosis: report of a case.

We report an extremely rare case of the development of hepatocellular carcinoma (HCC) in cardiac congestive liver fibrosis. A 62-year-old female presented to our hospital with a complaint of right upper quadrant pain. The patient had undergone cardiac surgery for pulmonary valve insufficiency, pulmonary stenosis and atrial septal defect when she was fifteen years of age. During the subsequent 47 years, she had occasionally suffered from various symptoms associated with right-sided heart failure due to pulmonary stenosis. Computed tomography revealed a liver tumor measuring 63 mm in diameter in segment 5 and other liver tumors in segments 5 (18 mm), 8 (17 mm) and 4 (12 mm), which were diagnosed as HCCs. There was no evidence of stenosis in any hepatic veins or inferior vena cava, and no infectious hepatitis or alcoholic liver damage. Anterior sectionectomy and partial resection of segment 4 was performed, and histological examination showed that these tumors were HCC accompanied by congestive liver fibrosis. Nine months later, multiple recurrent HCCs were detected in segment 6, and transcatheter arterial chemoembolization was employed thereafter. The patient died 40 months after surgery due to advanced recurrence.

Anatomic liver resection of right paramedian sector: ventral and dorsal resection.

The purpose of anatomic resection of the liver is to systemically eliminate malignant tumors that spread via the portal vein. Moreover, it results in reducing bleeding and bile leakage from the cut surface of the liver because Glisson's pedicle resection leads to parenchyma transection. Anatomical resection includes hemi-hepatectomy, sectionectomy, and segmentectomy. Recently, it has been noticed that this concept is not always appropriate for the liver resection including the right paramedian sector. It can be divided vertically into the ventral and the dorsal area according to the ramification of the third order of the portal veins. In the present study, we focused on the right paramedian sector and described techniques of surgical procedures of hepatectomy including resection of the ventral or dorsal areas.

Outcomes of hepatic resection for large hepatocellular carcinoma: special reference to postoperative recurrence.

Safety and efficacy of hepatic resection for large hepatocellular carcinomas (HCCs 10 cm or greater in diameter) remain controversial. Surgical results of patients with HCCs 10 cm or greater (n = 24) who underwent hepatic resection over an 11-year period were compared with those of patients with HCCs less than 10 cm (n = 291). There was no significant difference in mortality between the two groups (P > 0.99). Overall 5-year survival rate was 44.6 per cent among patients with HCCs 10 cm or greater and 70.5 per cent among those with HCCs less than 10 cm (P = 0.010); however, there was no significant difference in disease-free survival rate between the two groups (P = 0.16). Incidence of synchronous intra- and extrahepatic recurrence was higher in patients with HCCs 10 cm or greater than in those with HCCs less than 10 cm (P = 0.0012). Macrovascular invasion alone was an independent risk factor for poor prognosis (hazard ratio [HR],: 11.1) and recurrence (HR, 6.02) after hepatic resection for HCCs 10 cm or greater, which was correlated with synchronous intra- and extrahepatic recurrence. Hepatic resection for large HCCs is safe and efficacious. However, incidence of synchronous intra- and extrahepatic recurrence is high, especially in patients with macrovascular invasion.

Primary adenocarcinoma of the minor duodenal papilla with mass-forming chronic pancreatitis: report of a case.

A 70-year-old male was treated for gastric ulcers. Follow-up upper gastrointestinal endoscopy revealed an irregular, elevated tumor in the second portion of the duodenum. Upon pathological inspection of a biopsy specimen, a diagnosis of adenocarcinoma was made, and the patient was admitted to our hospital. Computed tomography showed an irregular mass in the pancreatic head and dilatation of the main pancreatic duct and bile duct. Pancreatic head carcinoma with infiltration of the duodenum was diagnosed, and pylorus-preserving pancreaticoduodenectomy was performed. A histopathological examination of the resected specimen showed moderately differentiated adenocarcinoma in the minor duodenal papilla and chronic pancreatitis in the pancreatic head. Therefore, primary adenocarcinoma of the minor duodenal papilla with mass-forming chronic pancreatitis was diagnosed. Currently, the patient is alive without recurrence 17 months after the surgery. Primary adenocarcinoma of the minor duodenal papilla is extremely rare. We herein report this case, and also provide a review of the literature.

Prediction of Postoperative Hepatic Failure after Liver Resection for Hepatocellular Carcinoma: Significance of the Aspartate Aminotransferase-to-Platelet Ratio Index.

BACKGROUND/AIMS: The serum aspartate aminotransferase-to-platelet ratio index (APRI) is a biomarker for hepatic fibrosis. The relationship between the APRI and postoperative hepatic failure is unclear. METHODOLOGY: The risk factors for postoperative hepatic failure and the APRI were evaluated in 457 patients who underwent liver resection for HCC. RESULTS: Nineteen patients (4.2%) experienced postoperative hepatic failure and five (1.1%) died. An increased APRI (p = 0.039), increased total bilirubin (p = 0.044), longer operation (p = 0.035) and increased intraoperative blood loss (p = 0.028) were independent risk factors in the multivariate analysis. Incidence of postoperative hepatic failure in patients with an APRI ≥ 1.57 (13/127, 10%) was significantly higher than in patients with an APRI < 1.57 (6/330,1.8%, p = 0.0002). Moreover, incidence of hepatic failure in high APRI cases with both an operation ≥ 500 min and intraoperative blood loss ≥ 1L (6/33 (18.1%)) tended to be higher than in those with lower values (7/94 (7.4%), p = 0.051). CONCLUSIONS: Increased APRI (≥ 1.57) may be a preoperative predictor of postoperative hepatic failure. Meticulous surgery with shorter operations and reduced blood loss may reduce the incidence of postoperative hepatic failure, even in patients with a high APRI.

Non-B non-C Hepatocellular Carcinoma: the Risk Factors for Recurrence and the Types of Recurrence Following Hepatic Resection.

BACKGROUND/AIMS: Risk factors for recurrence and types of recurrence following hepatic resection for non-B non-C hepatitis hepatocellular carcinoma (NBC-HCC) have not yet been established. METHODOLOGY: The clinicopathological data of 76 patients with NBC-HCC were retrospectively reviewed. Risk factors for postoperative recurrence were analyzed using univariate and multivariate analyses. In addition, types of intrahepatic recurrence were investigated. RESULTS: Of the 76 patients, 38 (50%) developed recurrence during the follow-up period, with disease-free survival rates at 1/3/5 years of 72%/46%/40%, respectively. Of the 38 patients with recurrence, 36 (95%) were found to have recurrence within three years after surgery. Of the 38 patients, 34 exhibited intrahe patic recurrence. In multivariate analysis, Child-Pugh B (p = 0.009) and microscopic vascular invasion (MVI) (p = 0.002) were independent risk factors for postoperative recurrence. Based on our definitions, of the 34 patients with intrahepatic recurrence, recurrence at the stump was present in one patient, multicentric recurrence in 11 patients and intrahepatic metastasis in 22 patients. CONCLUSIONS: Child-Pugh B and MVI are independent risk factors for the postoperative recurrence. Although most recurrences occurred within three years after hepatic resection, incidence of multicentric recurrence is not negligible. Preventing recurrence according to types of recurrence is therefore considered to be essential.

Safety of hepatic resection for hepatocellular carcinoma in obese patients with cirrhosis.

PURPOSE: This study aimed at investigating the safety of hepatic resection for hepatocellular carcinoma (HCC) in obese patients with cirrhosis in Japan. METHODS: We reviewed the clinical records of 202 patients with liver cirrhosis, who underwent hepatic resection for HCC between January, 2001 and August, 2011. The patients were divided into three groups according to their body mass index (BMI): the normal body weight (BMI < 24.9 kg/m(2)), obese class I (BMI 25.0-29.9 kg/m(2)), and obese class II (BMI ≥ 30 kg/m(2)) groups. We compared the patient backgrounds, intraoperative factors, and postoperative complications among the three groups. RESULTS: The normal body weight, obese class I, and obese class II groups comprised 138 (68.3 %), 55 (27.2 %), and 9 (4.5 %) patients, respectively. The incidence of non-B non-C cirrhosis was higher in the obese class II group (22 %) than in the normal body weight group (14 %, p = 0.034). Intraoperative blood loss tended to be higher in the obese class II patients than in the other two groups. Postoperative complications and mortality did not differ significantly among the three groups. According to multivariate analysis, obesity was not a risk factor for postoperative complications (Clavien-Dindo classification Grade III or higher) or mortality. CONCLUSION: Hepatic resection for HCC can be performed safely in obese patients with cirrhosis.

Laparoscopic management of giant splenic true cyst with partial splenectomy: a case report.

Non-parasitic splenic cysts are relatively rare, and the optimal surgical treatment for them remains controversial. Laparoscopic unroofing is a relatively safe and easy technique, but a significant number of recurrences has been reported. Thus, complete cystectomy with partial splenectomy is recommended by several surgeons. However, patients sometimes suffer from intraoperative bleeding. Here, we report a patient with a giant non-parasitic splenic cyst who underwent subtotal cystectomy with partial splenectomy. After the dissection of the vessels circulating the upper pole at the splenic hilum, the resection line of the splenic parenchyma was on the ischemic side of the cyanotic demarcation line. A vessel sealing system and laparoscopic coagulation shears were used for the resection. We intentionally left about 10% of the cyst wall to avoid bleeding from the non-ischemic splenic parenchyma and remaining vessels. No recurrence has been detected after 6 months of observation. We believe this method could be a useful alternative procedure for the treatment of non-parasitic splenic cysts and preservation of the splenic parenchyma.