[Conversion Surgery after Etoposide/Cisplatin(EP)Therapy for Huge Pancreatic Neuroendocrine Carcinoma - A Case Report].

Neuroendocrine carcinoma(NEC)is known as rapid tumor growth, high grade malignancy and poor prognosis. We report a case of huge pancreatic NEC successfully performed conversion surgery after EP therapy. A 70-year-old female, was presented to our hospital with appetite loss. CT scan revealed huge tumor, 15 cm in diameter, locating at the pancreas with possible involvement to liver, stomach, common hepatic artery, left gastric artery and gastroduodenal artery. Peritoneal dissemination and para-aortic lymph node metastasis were also suspected. EUS-FNA showed neuroendocrine carcinoma with almost 100%positive staining rate of Ki-67. We immediately started etoposide/cisplatin(EP)therapy. After 6 courses of EP, the tumor shrank remarkably and peritoneal disseminations were disappeared. Common hepatic artery and gastroduodenal artery became free from the tumor. However, after 7 courses of EP, CT and PET-CT revealed tumor re-growth. Also renal impairment could not afford to continue EP therapy. Therefore we decided to perform conversion surgery. In the guideline in Japan, there is no content specialized for surgical treatment for NEC. Moreover, second-line of chemotherapy for NEC has not been established. In the future, accumulation of NEC cases will contribute to develop effective multidisciplinary treatment.

[Resection for the Recurrence of Hepatocellular Carcinoma in the Inferior Vena Cava and Right Atrium under Deep Hypothermic Circulatory Arrest].

A 77-year-old man underwent extended right lobectomy of the liver for rupture of hepatocellular carcinoma. Recurrence in the inferior vena cava andright atrium was noted 30 months after surgery. We performedextirpation of this tumor thrombosis under retrograde cerebral perfusion during deep hypothermic circulatory arrest. The pericardium was cut through sternotomy, and cooling was initiated. After cardiac arrest at 20.4°C, the inferior vena cava was separated. An incision was made in the right atrium andthe tumor thrombus was extirpated. In the meantime, brain protection was maintainedby retrograde cerebral perfusion. The patient was discharged on day 12 without postoperative complications. He remains alive 6 months after surgery without recurrence. This procedure prevented pulmonary embolism due to tumor thrombosis release. It was also possible to perform the procedure with retrograde cerebral perfusion.

The Coexistence of Somatostatinoma and Gastrointestinal Stromal Tumor in the Duodenum of a Patient with Von Recklinghausen's Disease.

We herein describe a case of somatostatinoma coexisting with a gastrointestinal stromal tumor (GIST) in the duodenum of an 81-year-old woman with Von Recklinghausen's disease (VRD) and common bile duct stone who presented with diarrhea of three months in duration. Gastroduodenoscopy revealed an ulcer on the second part of the duodenum. A 2.1-cm enhancing tumor was observed to extend from the ulcer on an abdominal computed tomography scan. Subtotal stomach-preserving pancreaticoduodenectomy revealed a somatostatinoma on the papilla of the vater and duodenal GIST. There have been only eight reports on VRD associated with ampullary somatostatinoma and GIST. An awareness of this possibility in patients with gastrointestinal lesions is necessary for proper treatment and patient management.

[Strategy for Unresectable Pancreatic Cancer with Stenosis of the Duodenum - A Case Report].

Unresectable(UR)pancreatic cancer often causes duodenal obstruction. Case 1: A 58-year-old man was diagnosed with UR pancreatic cancer with obstruction of the 3rd duodenal portion. A duodenum 2nd portion jejunum bypass was performed, and FOLFIRINOX was introduced and continued over 6 months. Case 2: A 74-year-old man was diagnosed with UR pancreatic cancer with obstruction of the duodenum near the Treitz ligament. A duodenum 3rd portion jejunum bypass was performed, and gemcitabine plus nab-paclitaxel was introduced. After 8 courses of GN, adjuvant surgery was performed. Both patients resumed oral intake within a few days after bypass, their performance statuses(PS)were improved, and their body weights increased. Because a duodenal jejunum bypass is more physiological than a gastro-jejunum bypass and duodenal stent, stable ingestion is enabled, and they are stable enough for early initiation of chemotherapy.

[Primary Prophylactic Administration of Pegfilgrastim in FOLFIRINOX Therapy for Locally Advanced Pancreatic Carcinoma].

FOLFIRINOX therapy has a high response rate for pancreatic carcinoma, but has serious adverse effects. FOLFIRINOX therapy was administered to 11 patients with locally advanced pancreatic carcinoma at our hospital. We investigated the usefulness of primary prophylactic administration of pegfilgrastim(PegG). In the group receiving PegG, as well as with onset of neutropenia and thrombocytopenia, febrile neutropenia was reduced. Rates of anorexia and fatigue were also lower than in those who did not receive PegG. The PegG group maintained a high average relative dose intensity, as well as a high response rate. Primary prophylactic administration of PegG in FOLFIRINOX therapy is valid for pancreatic carcinoma.

Randomized controlled study of gemcitabine plus S-1 combination chemotherapy versus gemcitabine for unresectable pancreatic cancer.

PURPOSE: The aim of this study was to evaluate efficacy and safety of gemcitabine plus S-1 (GS) combination chemotherapy in patients with unresectable pancreatic cancer. METHODS: Patients were randomly assigned to receive GS (oral S-1 60 mg/m(2) daily on days 1-15 every 3 weeks and gemcitabine 1,000 mg/m(2) on days 8 and 15) or gemcitabine (1,000 mg/m(2) on days 1, 8, and 15 every 4 weeks). The primary endpoint was progression-free survival (PFS). RESULTS: One hundred and one patients were randomly assigned. PFS was significantly longer in the GS arm with an estimated hazard ratio (HR) of 0.65 (95 % CI 0.43-0.98; P = 0.039; median 5.3 vs 3.8 months). Objective response rate (ORR) was also better in the GS arm (21.6 vs 6 %, P = 0.048). Median survival was 8.6 months for GS and 8.6 months for GEM (HR 0.93; 95 % CI 0.61-1.41; P = 0.714). Grade 3-4 neutropenia (44 vs 19.6 %, P = 0.011) and thrombocytopenia (26 vs 8.7 %, P = 0.051) were more frequent in the GS arm. CONCLUSIONS: GS therapy improved PFS and ORR with acceptable toxicity profile in patients with unresectable pancreatic cancer.

Hemodynamic changes in the hepatic circulation after the modulation of the splenic circulation in an in vivo human experimental model.

Recent advances in liver surgery have highlighted the effects of the splenic circulation on the hepatic circulation with respect to the hepatic arterial buffer response (HABR). The aim of the present study was to investigate the actual hemodynamic effects of splenic artery embolization/ligation and splenectomy on the hepatic circulation in patients who underwent pancreaticoduodenectomy through in vivo experimental models. In vivo models of splenic artery embolization/ligation (only splenic artery clamping) and splenectomy (simultaneous clamping of both the splenic artery and the splenic vein) were created in 40 patients who underwent pancreaticoduodenectomy for various reasons. The portal venous flow velocity, the portal venous flow volume, the hepatic arterial flow velocity, and the hepatic arterial resistance index were measured with color Doppler ultrasonography. Clamping of the splenic artery induced an immediate and significant increase (16%) in the hepatic artery velocity (P < 0.001), and the portal venous flow also decreased significantly (10%, P = 0.03). Fifteen minutes after the clamping of the splenic artery, the hepatic artery velocity remained significantly increased at the level of the initial clamping, and the portal venous flow significantly decreased (16%, P < 0.001). Clamping of the splenic vein, which was performed after the clamping of the splenic artery, resulted in an immediate and significant decrease (30%) in the portal venous flow (P < 0.001), but the hepatic arterial flow was not affected. Fifteen minutes after the clamping of the splenic vein, there was no change in the portal flow, which remained significantly lower (28%) than the flow in controls, whereas the hepatic arterial flow further significantly increased (31%, P < 0.001). In conclusion, our findings indicate that both splenic artery embolization/ligation and splenectomy are effective for increasing hepatic arterial flow and decreasing portal flow, with splenectomy providing a greater advantage. The HABR underlies these hemodynamic changes.

[A case of inflammatory myofibroblastic tumor with repeated recurrence and metastasis].

A 37-year-old woman presented with an asymptomatic pulmonary nodule. A pulmonary S8 segmentectomy was performed. Recurrence and metastasis were noted every 6 months after surgery; repeat surgeries were performed at 18, 24, and 36 months. On histopathological examination, the tumor showed spindle cell proliferation with infiltration of various inflammatory cells, and was diagnosed as inflammatory myofibroblastic tumor (IMT). An IMT is defined as a tumor of intermediate biological potential, which may sometimes metastasize. It is necessary to note the potential for metastasis in the future, especially in cases with anaplastic lymphoma kinase (ALK)-negative immunohistological staining, where the tumor grade can be high.

[A case of neuroendocrine small cell carcinoma of the common bile duct].

A 75-year-old woman admitted for jaundice was found to have a tumor occupying the lumen of the middle bile duct on diagnostic imaging. Treatment with endoscopic retrograde bile drainage (ERBD) improved the jaundice. Bile cytology was defined as class III. We diagnosed the condition as middle bile duct cancer with a replaced right hepatic artery, and therefore, curative resection was possible. Pancreatoduodenectomy was performed and the replaced right hepatic artery was resected. Histological examination using hematoxylin and eosin( HE) staining and immunological staining with chromogranin A, synaptophysin, and CD56 revealed small cell carcinoma. Small cell carcinoma of the bile duct is a highly malignant disease. Fortunately, it is rare and only 22 cases have been reported in the Japanese literature.

[Colon cancer with portal vein tumor thrombosis-a case report and review of the literature].

Colon cancer with portal vein tumor thrombosis has a poor prognosis. However, little is known about the clinicopathological characteristics of these patients. In this study, we attempted to clarify the clinicopathological characteristics of such patients reported in the Japanese literature, including our own case. This case concerns a 48-year-old female patient diagnosed as having transverse colon cancer with severe portal vein tumor thrombosis. Despite curative resection, the patient was found to have multiple liver metastases six months later, and chemotherapy did not prove to be adequately effective; she died 18 months after surgery. A search of the relevant literature revealed 9 reports of similar patients. The patients consisted of 4 males and 6 synchronous cases, with a median age of 70 years. Portal vein tumor thrombosis was observed in 6 patients. While the portal vein was the most frequent site of thrombosis, other patients showed tumor thrombosis of the superior and inferior mesenteric veins. Despite curative resection, 3 patients eventually developed liver metastases after the operation. The median disease-free survival of the patients who had undergone curative resection was 300 days, and the overall median survival of the patients was 420 days. Thus, for the portal vein tumor thrombosis, we need to adopt adjuvant chemotherapy in consideration of a high risk for the liver metastases.

One-step percutaneous transhepatic insertion of a balloon-expanding metallic stent for obstructive jaundice.

BACKGROUND AND AIM: The aim of this study was to evaluate the efficacy and safety of one-step percutaneous transhepatic insertion of the Express LD stent, a balloon-expanding stainless steel stent used for the management of distal artery stenosis in the treatment of obstructive jaundice caused by various inoperable malignancies. METHODS: Seventy-one consecutive patients with unresectable malignant biliary obstruction who underwent Express LD stent placement between 2007 and 2010 at our institute were reviewed. RESULTS: Mean stent patency was 165 ± 144 days and mean patient survival was 180 ± 156 days, while the cumulative stent patency rate and patient survival rate at 6 and 12 months were 79% and 65%, and 38% and 16%, respectively. Stents were successfully placed in all cases without any stent migration or misplacement. Stent failure occurred in 14 patients (20%), and 16 complications were observed, including 12 cholangitis (17%), two cholecysitis (3%), and two pancreatitis (3%). Y-configuration stenting for hilar bile duct obstruction was the only independent prognostic factor for stent failure. CONCLUSIONS: One-step percutaneous transhepatic insertion of the Express LD stent is effective and safe for the management of obstructive jaundice caused by inoperable malignancies.

Left hepatic trisectionectomy for hilar cholangiocarcinoma presenting with an aberrant biliary duct of segment 5: a case report.

INTRODUCTION: Management of the biliary ducts during liver resection is one of the most important challenges for hepatobiliary surgeons. Here, we report the case of a left hepatic trisectionectomy for hilar cholangiocarcinoma with a rare aberrant biliary duct of segment 5, which, to the best of our knowledge, has never been reported in previous literature. CASE PRESENTATION: A 56-year-old Asian female initially presented with intrahepatic bile duct dilatation in the left lateral sector, left paramedian sector, and right paramedian sector. Simultaneous cholangiography from a percutaneous transhepatic biliary drainage tube in biliary duct of segment 8 and endoscopic nasobiliary drainage tube in biliary duct of segment 3 revealed drainage of the right lateral sectoral branch into the common hepatic duct and the aberrant drainage of segment 5 into the right lateral sectoral branch. The left hepatic duct, right paramedian sectoral duct, and the confluence of the right lateral sectoral duct were narrowed. Left hepatic trisectionectomy was successfully performed with careful dissection and division of the aberrant biliary duct of segment 5. CONCLUSION: For safe liver resection, it is important to perform a detailed anatomic evaluation of the intrahepatic ducts, both preoperatively and intraoperatively.

Giant liver hemangioma resected by trisectorectomy after efficient volume reduction by transcatheter arterial embolization: a case report.

INTRODUCTION: Liver hemangiomas are the most common benign liver tumors, usually small in size and requiring no treatment. Giant hemangiomas complicated with consumptive coagulopathy (Kasabach-Merritt syndrome) or causing severe incapacitating symptoms, however, are generally considered an absolute indication for surgical resection. Here, we present the case of a giant hemangioma, which was, to the best of our knowledge, one of the largest ever reported. CASE PRESENTATION: A 38-year-old Asian man was referred to our hospital with complaints of severe abdominal distension and pancytopenia. Examinations at the first visit revealed a right liver hemangioma occupying the abdominal cavity, protruding into the right diaphragm up to the right thoracic cavity and extending down to the pelvic cavity, with a maximum diameter of 43 cm, complicated with "asymptomatic" Kasabach-Merritt syndrome. Based on the tumor size and the anatomic relationship between the tumor and hepatic vena cava, primary resection seemed difficult and dangerous, leading us to first perform transcatheter arterial embolization to reduce the tumor volume and to ensure the safety of future resection. The tumor volume was significantly decreased by two successive transcatheter arterial embolizations, and a conventional right trisectorectomy was then performed without difficulty to resect the tumor. CONCLUSIONS: To date, there have been several reports of aggressive surgical treatments, including extra-corporeal hepatic resection and liver transplantation, for huge hemangiomas like the present case, but because of its benign nature, every effort should be made to avoid life-threatening surgical stress for patients. Our experience demonstrates that a pre-operative arterial embolization may effectively enable the resection of large hemangiomas.

Risk factors for postoperative pancreatic fistula after pancreaticoduodenectomy: the significance of the ratio of the main pancreatic duct to the pancreas body as a predictor of leakage.

OBJECTIVE: Postoperative pancreatic fistula (POPF) is a severe and frequent complication after pancreaticoduodenectomy (PD). The aim of this study was to identify an independent predictor of POPF and to assess the efficacy of preoperative multidetector row computed tomography (MDCT) images as an indicator for POPF. METHODS: A total of 122 patients who underwent PD with an end-to-side, duct-to-mucosa pancreaticojejunostomy between January 2005 and May 2009 were retrospectively reviewed. The diameter of the main pancreatic duct (MPD), the diameter of the short axis of the pancreas body, and the ratio of the MPD to the pancreas body (MPD index) were digitally measured based on the curved reformatted images of preoperative MDCT. RESULTS: Postoperative pancreatic fistula occurred in 33 patients (27%). The operative mortality rate was 3.3% (4 patients). All four patients had grade C POPF. Three died because of hemorrhage from a pseudoaneurysm of the gastroduodenal artery stump, and one died because of sepsis due to major leakage from the pancreaticojejunostomy. In a multivariate analysis, the intraoperative blood loss (/100 ml) [odds ratio (OR), 1.1; 95% confidence interval (CI), 1.05-1.17] and MPD index (<0.2) (OR 50; 95% CI 6-41) proved to be independent predictors of POPF. In patients with an MPD index of <0.2, the incidence of POPF was 45%, and the mortality rate was 7.5%. CONCLUSION: The MPD index obtained from preoperative MDCT can be a reliable predictor of POPF after PD.

Modified liver-hanging maneuver designed to minimize blood loss during hepatic parenchymal transection in hemihepatectomy.

PURPOSE: We evaluated the efficiency of a modified liver-hanging technique for minimizing intraoperative blood loss during right and left hemihepatectomy. METHODS: The lower end of the hanging tape was repositioned between the parenchyma of the left paramedian sector and the hilar plate. The upper end of the tape was positioned between the right hepatic vein and middle hepatic vein for right hepatectomy (Belghiti), and between the middle hepatic vein and left hepatic vein for left hepatectomy. The tape was positioned prior to the parenchymal transection. We compared the results of this operative technique, performed in 15 recent patients, with those of conventional hemihepatectomy performed in 14 earlier patients. RESULTS: There were no intergroup differences in baseline characteristics or postoperative outcomes. Intraoperative blood loss (P = 0.02), especially blood loss during the parenchymal transection (P = 0.005), was significantly less in patients undergoing the modified technique. Multivariate analysis revealed that this modified liver-hanging technique offered a significant advantage in blood-loss reduction during parenchymal transection over the conventional techniques (P = 0.005). CONCLUSION: Using the liver-hanging technique during hemihepatectomy could be crucial for liver surgeons.

Diagnostic accuracy of multidetector-row computed tomography for hilar cholangiocarcinoma.

BACKGROUND AND AIM: The aim of this study was to investigate the diagnostic reliability of multidetector-row computed tomography (MDCT) for the evaluation of tumor spread in hilar cholangiocarcinoma. METHODS: Images obtained from a 16-detector row scanner of 22 patients were interpreted. The diagnostic accuracy of longitudinal ductal spread, vertical invasion (including hepatic parenchyma), and lymph node metastasis was assessed with reference to histopathological findings. RESULTS: The location of the tumor was correctly diagnosed in 95% of cases (21/22), but in five of these cases, the cut end of the intrahepatic bile duct was positive, resulting in 77% diagnostic accuracy for longitudinal spread. Among the patients with a negative bile duct surgical margin, there was a significant difference in the measurement of tumor spread between MDCT and microscopic investigation (P < 0.001). For vertical invasion, the sensitivity, specificity, positive predictive value (PPV), and negative predictive value (NPV) of MDCT were 69%, 100%, 100%, and 69% for the liver parenchyma, respectively. The sensitivity, specificity, PPV, and NPV of MDCT for lymph node metastasis were 50%, 75%, 43%, and 80%, respectively. CONCLUSIONS: The diagnostic accuracy of MDCT for tumor location and vertical invasion was satisfactory, but ductal spread was underestimated in comparison with microscopic measurements.

[Switching to direct intrahepatic arterial infusion of 5-FU after a progression of liver metastases from colorectal carcinoma treated with systemic infusion of FOLFOX].

Direct intrahepatic arterial infusion of 5-FU produced a significantly higher response rate than systemic infusion of FOLFOX in the treatment of hepatic metastases from colorectal carcinoma. Fourteen patients switched over from systemic FOLFOX therapy to intrahepatic protracted 5-FU infusion after a progression of liver metastases treated with systemic therapy. Of the 14 patients whose tumors had initially failed to respond to systemic FOLFOX therapy, 12 (85%) had a partial response, and 13 (93%) had a reduction in their tumor marker (CEA, CA19-9, TPA) when the treatment was switched to intrahepatic 5-FU therapy. Traditional chemotherapy toxicity, such as myelosuppression, nausea, vomiting and neurotoxicity did not occur in the intrahepatic group. Three out of 14 patients survived more than a year, and the longest was 18 months. A better survival rate can be achieved with the use of hepatic artery infusion therapy.

Preoperative evaluation of the longitudinal spread of extrahepatic bile duct cancer using multidetector computed tomography.

BACKGROUND/PURPOSE: The aim of this study was to compare the diagnostic accuracy of multidetector computed tomography (MDCT) and direct cholangiography in evaluating the longitudinal spread of extrahepatic bile duct cancer. METHODS: Images obtained from a 16-detector row scanner (MDCT) and from direct cholangiography (via either endoscopic naso-biliary drainage or percutaneous transhepatic biliary drainage) of 47 patients with histopathologically proven extrahepatic bile duct cancer were retrospectively interpreted. Differences between measures of longitudinal tumor spread determined by each modality and measures of macroscopic spread in resected specimens were assessed and compared. RESULTS: Assessments carried out using MDCT differed significantly less from the macroscopic measurements than those made using direct cholangiography (P < 0.0001). Provided the diagnosis was defined as being accurate, based on a diagnostic difference of within +/-5 mm, the diagnostic accuracy of MDCT (96%) was significantly higher than that of direct cholangiography (70%) (P = 0.028). Preoperative evaluation with direct cholangiography resulted in a 30% underestimation of the incidence. CONCLUSION: MDCT is superior to direct cholangiography for evaluating the preoperative longitudinal extent of bile duct cancer. Consequently, the utility of MDCT for preoperative evaluation of extrahepatic bile duct cancer warrants further examination.

[Usefulness of F-18-fluorodeoxyglucose positron emission tomography for evaluating the efficacy of transient hepatic arterial chemoembolization for liver metastases of rectal cancer--a case report].

It is pointed out that there can be a discrepancy between the effect diagnosed by radiographic imaging and that by histological examination, when we treat patients with liver metastases of colorectal cancer by a transient hepatic arterial chemoembolization. We report a case of liver metastases of rectal cancer in which F-18-fluorodeoxyglucose positron emission tomography (FDG-PET) was useful for evaluating the therapeutic efficacy for transient hepatic arterial chemoembolization. A 58-year-old man with synchronous liver metastases (H2) of rectal cancer underwent a low anterior resection, a partial hepatectomy, cholecystectomy, and ligation of the gastroduodenal artery. After these operations, the patient received 6 hepatic arterial injections with degradable starch microspheres (300-600 mg), adriamycin (30 mg), and mitomycin C (10 mg) for the remaining metastatic lesion (S7). Although abdominal CT scan revealed a partial response, FDG-PET did not show any abnormal deposits. Hepatic posterior segmentectomy was performed 7 months after the first operation. Histological examination did not show any viable tumor cells in the resected specimen.