A case of pancreatic PEComa with prominent inflammatory cell infiltration: the inflammatory subtype is a distinct histologic group of PEComa.

BACKGROUND: PEComa is a mesenchymal tumor that can occur in various organs including the uterus and soft tissues. PEComas are composed of perivascular epithelioid cells, and angiomyolipoma (AML), clear cell sugar tumor (CCST), and lymphangiomyomatosis (LAM) are considered lesions of the same lineage as tumors of the PEComa family. Histologically, a common PEComa shows solid or sheet-like proliferation of epithelioid cells. This is accompanied by an increase in the number of dilated blood vessels. Here, we report a case of pancreatic PEComa with marked inflammatory cell infiltration. CASE PRESENTATION: A 74-year-old male patient underwent an appendectomy for acute appendicitis. Postoperative computed tomography and magnetic resonance imaging revealed a 30 × 25 mm non-contrast-enhanced circular lesion in the tail of the pancreas. The imaging findings were consistent with a malignant tumor, and distal pancreatectomy was performed. Histologically, most area of the lesion was infiltrated with inflammatory cells. A few epithelioid cells with large, round nuclei, distinct nucleoli, and eosinophilic granular cytoplasm were observed. Spindle-shaped tumor cells were observed. Delicate and dilated blood vessels were observed around the tumor cells. Immunohistochemically, the atypical cells were positive for αSMA, Melan A, HMB-45, and TFE3. The cytological characteristics of the tumor cells and the results of immunohistochemical staining led to a diagnosis of pancreatic PEComa. CONCLUSIONS: A histological variant known as the inflammatory subtype has been defined for hepatic AML. A small number of tumor cells present with marked inflammatory cell infiltration, accounting for more than half of the lesions, and an inflammatory myofibroblastic tumor-like appearance. To our knowledge, this is the first report of pancreatic PEComa with severe inflammation. PEComa is also a generic term for tumors derived from perivascular epithelioid cells, such as AML, CCST, and LAM. Thus, this case is considered an inflammatory subtype of PEComa. It has a distinctive morphology that is not typical of PEComa. This histological phenotype should be widely recognized.

[A Case of Mucosal Gastric Cancer with Lymph Node Metastasis Successfully Treated with Chemotherapy].

A 64-year-old man was found to have an irregular region in the stomach by medical examinations. Upper gastrointestinal fiberscopy(GIF)revealed a reddish lesion in the lesser curvature of the upper stomach. Biopsy showed moderately differentiated adenocarcinoma. CT revealed swollen lymph nodes from the lesser curvature of the stomach to the para-aortic region. EUS-FNA was performed twice; however, histology revealed few atypical cells. A definitive diagnosis could not be obtained. Endoscopic findings revealed that the gastric cancer had invaded as far as the mucosa. Moreover, the swollen lymph nodes were considered to have originated from a different disease, such as lymphoma. The lesion of the stomach was an indication for ESD. On April 2016, ESD was performed, and histology revealed the following: Ⅱc, 31×23 mm, tub2, T1a(M), UL-, ly-, v-, VM0, and HM0. Incisional biopsy of the lymph nodes of the para-aorta was performed the followingmonth, and histology revealed poorly differentiated adenocarcinoma, which metastasized from gastric cancer. SOX therapy was performed in 10 courses. The para-aortic lymph nodes disappeared, and the number of lesser curvature lymph nodes decreased. On August 2018, follow-up GIF endoscopy was performed. A depressed mucosa was found in the lesser curvature of the gastric body, which was away from the ESD scar. Biopsy showed moderately differentiated adenocarcinoma. Total gastrectomy with lymph node dissection was performed on November 2018. Metastasis of the lesser curvature lymph nodes was positive; however, curative resection was performed histologically.

[A Case of Lung Metastasis Occurring Four Years after Pancreaticoduodenectomy for Pancreatic Cancer].

The patient was a 68-year-old man who underwent pancreaticoduodenectomy for pancreatic cancer in 2011(T3N1M0, Stage Ⅲ). Fifty-one months after surgery, 2tumors measuring about 8mm in diameter were detected in the right lung. Thus, partial resection of the right lung was performed. Histological examination of both tumors revealed a tubular adenocarcinoma, with positive staining for CA19-9 and cytokeratin 7 and negative staining for cytokeratin 20, TTF-1, and napsin A, which were diagnosed as metastasis of pancreatic cancer. Twenty-nine months after the lung operation, he died because of peritoneal dissemination. Recurrent pancreatic cancer with metastasis has a particularly poor prognosis; however, surgical resection of lung metastasis in pancreatic cancer might result in a better prognosis.

Combined imaging with multi-detector row computed tomography and diffusion-weighted imaging in the detection of pancreatic cancer.

Pancreatic cancer is one of the most aggressive malignant tumor types. Its prognosis is extremely poor without early detection, and an accurate diagnosis with imaging techniques is vital for any chance of long-term survival after treatment. Despite the great technical advances that have been made with various kinds of imaging equipment, detection of pancreatic cancer is unsatisfactory, and new modalities are required. Diffusion-weighted imaging (DWI) on magnetic resonance imaging (MRI) has high sensitivity and specificity for the detection of pancreatic cancer; however, it does not provide anatomic information. Quite often, the necessary spatial correlation of a high-intensity area with anatomic structures is constrained. If it becomes possible to combine the imaging technique of DWI with multi-detector row computed tomography (MDCT), such as is done with positron emission tomography (PET) and computed tomography (CT), it is expected that the early diagnostic capability will improve. The objective of this study was to introduce combined imaging with DWI and CT into clinical practice to improve the diagnosis of pancreatic cancer. In the current report, we demonstrate a clinical attempt to combine DWI on MRI with the anatomical accuracy of MDCT for two patients of pancreatic adenocarcinoma. Analysis of these two patients revealed that the combined images corresponded precisely to the operative findings. Thus, the combined imaging with DWI and MDCT is useful for the detection of the pancreatic cancer.

Diffusion-weighted imaging in the detection of lymph node metastasis in colorectal cancer.

Diffusion-weighted imaging (DWI) is a functional technique based on the ability to depict movement of water molecules. The magnitude of water molecule movement is expressed as apparent diffusion coefficient (ADC) value. Its usefulness in the diagnosis of malignant tumors has gained interest. The purpose of this study was to evaluate the usefulness of DWI in detecting lymph node metastases of colorectal cancer. The subjects were 46 consecutive patients (mean age 71.4 +/- 8.7 years) with colorectal cancer, treated by radical surgery from 2006 to 2008. The size of metastatic lymph nodes on DWI was significantly larger than non-metastatic lymph nodes (10.3 vs. 7.6 mm). The mean ADC value was significantly lower for metastatic lymph nodes than non-metastatic lymph nodes (1.36 vs. 1.85 x 10(-3) mm(2)/sec). In addition, for evaluation of lymph node metastasis that reflects the primary tumor characteristics, the LN/T ratio (defined as the ratio of lymph node ADC value to the primary tumor ADC value) was calculated. It was significantly lower for metastatic lymph nodes than non-metastatic lymph nodes (1.41 vs. 1.59). Receiver operating characteristic curve analysis revealed that the best performing cutoffs were 8.5 mm for lymph node size, 1.44 x 10(-3) mm(2)/sec for ADC value, and 1.495 for LN/T ratio. Accuracy was significantly greater for lymph nodes with LN/T ratio (78.5%) than for lymph node size (62.0%) or ADC value (74.8%). In conclusion, preoperative DWI, especially the LN/T ratio, is recommended for evaluation of lymph node metastasis in patients with colorectal cancer.

Small cell undifferentiated carcinoma of the ascending colon with rapid enlargement after resection: report of a case and review of the literature.

Although adenocarcinoma represents the vast majority of neoplasms of the large intestine, small cell undifferentiated carcinoma (SCUC) also arises from the colorectum. SCUC of the colorectum is highly malignant and shares the similarities in histologic characteristics, behavior, and histochemistry with SCUC of the lung. We report herein a case of SCUC in the ascending colon with rapid enlargement after resection. A 70-year-old male, who presented to a nearby physician with chief complaints of pain in the right lower quadrant, was referred to our hospital. We found a tumor mass that was approximately 15 cm in size in the right lower quadrant. Computed tomography (CT) images showed an irregularly shaped tumor, located inferior to the lower border of the right kidney and in the area of the ascending colon. By colonoscopy, we found a circumferential tumor of the ascending colon. A biopsy indicated it to be SCUC. The patient underwent right hemicolectomy. Two weeks after the resection, we palpated a tumor mass in the same area. The abdominal CT images showed a tumor mass that was approximately 10 cm in size. The tumor rapidly enlarged, and the patient died of multiple organ failure. SCUC is a tumor with a high malignant potential. Radical treatment cannot be achieved by surgical therapy alone and hence further studies of effective adjuvant therapy would be required.

A resected case of a small hepatocellular carcinoma developing within the bile duct.

We experienced a resected case of a small hepatocellular carcinoma, which required differential diagnosis from intrahepatic cholangiocellular carcinoma. The patient was a 76-year-old man. While his course had been being observed because of hepatitis C antibody-positive liver cirrhosis, ultrasonographic examination of the abdomen revealed dilation of biliary branches in the anterior segment of the liver and a hyperechoic mass 10 mm in diameter at the origin of the branch. A dynamic computed tomography scan showed a high-density tumor in the early phase. After embolization of the right branch of the portal vein, resection of the right lobe of the liver and the extrahepatic bile duct was performed. A resected specimen showed a white-colored mass 8 mm in diameter at the origin of the anterior segmental biliary branch. In the pathological findings, the diagnosis was a poorly differentiated hepatocellular carcinoma with strong nuclear atypia; the tumor filled the bile duct, forming a trabecular structure. The immunohistological stains of the tumor were positive for cytokeratin (CK) 8, CK18, and HepParl and negative for alpha-fetoprotein, carcinoembryonic antigen, CA19-9, CK7, CK19, and CK20. There was atypia in the biliary lining epithelium adjacent to the tumor, and the hepatocellular carcinoma may have developed from the biliary epithelium.

Laparoscopic right hemihepatectomy for a case of polycystic liver disease with right predominance.

BACKGROUND/PURPOSE: A case of polycystic liver disease with right predominance treated with laparoscopic right hemihepatectomy is described. PATIENT: A 43-year-old woman complaining of right upper abdominal pain came in for consultation. Abdominal ultrasonography and computed tomography studies showed multiple liver cysts occupying mainly the right lobe, renal cysts, and splenomegaly. METHODS: Four trocars were used. A 12-mm trocar placed under the umbilicus was used for abdominal exploration. The other three trocars, two 12-mm trocars and one 5-mm trocar, were used as working ports. The liver was transected with ultrasound scissors and LigaSure. Major vessels such as the right portal vein, the right bile duct, and the hepatic vein were divided with a vascular endostapler. Operation time was 320 min, intraoperative blood loss was 120 ml, and postoperative oral intake occurred on day 3. RESULTS: No complication was observed during the perioperative period. CONCLUSIONS: Laparoscopic right hemihepatectomy is generally considered to cause excessive intraoperative bleeding and a long operation time. For our patient with multiple liver cysts, the procedure was a safe and minimally invasive option because little hepatic parenchymal resection was necessary for the multiple cysts.A case of polycystic liver disease with right predominance treated with laparoscopic right hemihepatectomy is described.

Enhanced proliferation of hepatic progenitor cells in rats after portal branch occlusion.

It is known that hepatic progenitor cells increase in number after liver injury caused by carcinogens, but this injury cannot be reproduced in humans. In order to create a practical source of hepatic progenitor cells, changes in the number of liver epithelial cells (LECs), a type of hepatic progenitor cell, were examined following partial interruption of the portal flow. Efficiency in this isolation procedure was investigated, and isolated LECs were transplanted into livers to demonstrate their differentiation into hepatocytes in vivo.A volume of 70% of Sprague-Dawley rat's livers was exposed to portal vein ligation. LECs, identified as alpha-fetoprotein (AFP)-positive and albumin-negative cells, were counted and LECs isolated from the portal vein ligated-lobe were characterized by immunostaining and Western blotting. Isolated cells were subjected to a 1-week-culture, and the number of colonies formed on dishes was counted. The cells were then transplanted to the livers of genetic analbuminemic rats and identified by immunohistochemistry. The number of LECs in the portal ligated-lobes on day 7 was 14.7 +/- 6.5 cells/1,000 hepatocytes: 18 times higher than numbers in a normal liver. A significant increase was noted from day 3 until day 28. Isolated LECs were AFP-positive, albumin-negative, and cytokeratin-19-positive cells. The number of colonies on the 7th day following portal vein ligation was 42 times higher than in a normal liver. After transplantation of the LECs to the analbuminemic rat, a cluster of albumin-producing cells was present until day 56, suggesting that they differentiate into hepatocytes. We conclude that after portal vein occlusion, the liver can be a good source of hepatic progenitor cell. These results open up the possibility of cellular transplantation for liver functional support in clinical settings.

Resection of a solitary pancreatic metastasis from renal cell carcinoma with a gallbladder carcinoma: report of a case.

Most metastatic pancreatic tumors are detected at an advanced stage and are not considered suitable for surgery; however, resection is sometimes indicated for a solitary pancreatic metastasis from renal cell carcinoma (RCC) and improves the prognosis. We report such a case, in which the hilar liver was resected with lymph node dissection and distal pancreatectomy. Histological examination revealed regional lymph node metastasis of gallbladder carcinoma (GBC), but all the surgical margins were free of cancer. Postoperative extra-beam radiation therapy was delivered to the hepatic portal lesion to prevent GBC recurrence. The patient remains disease-free 14 months after the completion of radiation therapy. Thus, if all affected areas can be resected, the prognosis associated with pancreatic metastasis from RCC may be favorable.

Cholangiocarcinoma coincident with schistosomiasis japonica.

The relationship of parasitic liver disease to cholangiocarcinoma has long been debated, and it has been reported that cholangiocarcinoma is associated with opisthorchiasis viverrini. We report herein a rare case of cholangiocarcinoma associated with schistosomiasis japonica. A 76-year-old Japanese man with jaundice was diagnosed with cholangiocarcinoma. Radical resection was not done because of hepatic arterial and portal vein invasion. Biliary microwave tissue coagulation therapy was performed with placement of a metallic stent endoprosthesis. Twenty-two months after the treatment, however, the patient died from hematemesis. Autopsy findings revealed that there was no distant metastasis, even in the area of regional lymph node metastasis. The primary tumor in the hepatic hilar region had been replaced by necrotic debris resulting from the microwave therapy, and an expandable metallic stent was located in the center of the debris. Histological findings showed schistosome eggs, which were old and microcalcified, in veins in the colonic submucosa. Glisson's fibrosis around the cancer lesion suggested that schistosomiasis japonica and cholangiocarcinoma can occur together with severe chronic inflammation of the portal vein.

Outcomes and indications of segmental pancreatectomy. Comparison with distal pancreatectomy.

BACKGROUND/AIM: To preserve pancreatic function, segmental pancreatectomy has been proposed for benign or low-malignancy tumors in the pancreatic body. Indications for the procedure, however, are still controversial. METHODS: In this study, we investigated the advantages and disadvantages of segmental pancreatectomy compared with distal pancreatectomy and subsequently determined indications for segmental pancreatectomy. RESULTS: The distal pancreatectomy patients had shorter operation times, lower incidence of operative complications, and shorter hospital stays compared to segmental pancreatectomy patients. Endocrine function in distal pancreatectomy patients deteriorated compared to that of segmental pancreatectomy patients. The postoperative 75-gram oral glucose tolerance test showed a diabetic pattern in 3 of 7 distal pancreatectomy patients, whereas none of the segmental pancreatectomy patients became diabetic after surgery. The relation between the length of the removed pancreas and plasma glucose at 2 h after the 75-gram glucose intake showed a significant correlation. CONCLUSION: According to our results, if the length of removed pancreas is longer than 12 cm, the patients will have a risk of acquiring diabetes. In those cases, the segmental pancreatectomy should be considered.

Treatment for postoperative liver failure after major hepatectomy under hepatic total vascular exclusion.

Hepatic total vascular exclusion (HTVE) with clamping of the portal triad and the inferior vena cava below and above the liver is a useful technique in the resection of major hepatic lesions situated close to the hepatic veins and inferior vena cava. From 1996 to 2000, five patients underwent major hepatectomy under HTVE; among these, liver failure occurred in two patients because of liver cirrhosis or hepatic artery interruption. In the former case, apheresis therapy (plasma exchange: 9 times), continuous prostaglandin E, (PGE,) infusion via the hepatic artery(0.01 tg/kg/min) for 7 days, and hyperbaric oxygen therapy (3 times: 2 ATA, 60 min) were applied. In the latter case, apheresis therapy (plasma exchange: 9 times, continuous hemodiafiltration: 12 days) and continuous PGE, infusion via the superior mesenteric artery for 7 days were applied. With these treatment modalities, both cases were cured of postoperative liver failure.

[Biliary stenting for hepatobiliary-pancreatic disease].

Since 1990, expandable metallic stents (EMS) have been used in biliary obstruction, which are thinner than the plastic endoprosthesis and can secure sufficient biliary tract. EMS treatment improves the prognosis of patients with unresectable malignant biliary obstruction. Several types of stent are available, and each has its own characteristics of expansion, flexibility, visibility, shorting, and size variation. Those characteristics must be taken into account when selecting a stent for individual patients. In the case of hepatic hilar obstruction, more than one EMS is needed and the position of stent placement is important. For bile duct cancer, stents should be placed in a side-by-side or end-to-side position, because in these cases the tumor affects in-growth through the stent, so repeat biliary drainage and stenting would be needed. EMS treatment is sometimes used for stenosis of the portal vein, but stent placement is not effective for chronic stenosis with collateral circulation. With the development of stenting instruments, biliary endoprosthesis has become safer and easier. But we must not forget that stent treatment is a palliative treatment and consider the indications for and selection of the stent carefully.

Two cases of hepatic artery interruption after hepatopancreatobiliary surgery treated by prostaglandin E(1) infusion via the superior mesenteric artery.

In two cases of hepatic arterial flow interruption after hepatopancreatic surgery, continuous PGE(1) infusion from the superior mesenteric artery (SMA) was applied to oxygenate the liver through the portal vein. Case 1 was a 69-year-old woman with a non-functioning islet cell tumor of the pancreas. She underwent pancreatic resection following hepatic arterial infusion of anticancer drugs. Serum alanine aminotransferase (ALT) was elevated to 5500 IU/l on postoperative day (POD) 2; angiography revealed complete celiac artery obstruction. Continuous PGE(1) was administered from SMA at a rate of 0.01 &mgr;g/kg/min for 7 days. Serum ALT was normalized within 2 weeks and the peak level of serum total bilirubin (T. Bil) was 4.5 mg/dl. Case 2 was a 66-year-old man suffering from metastatic liver cancer. Complete obstruction of the proper hepatic artery was noted at the time of liver resection after hepatic arterial chemotherapy. Serum ALT was elevated to 2930 IU/l on POD 1, and PGE(1) infusion from SMA was done for the succeeding 7 days. Necrotic area was so vast that serum T. Bil rose to 19 mg/dl. However, it decreased with time. Both cases required 3 months for necrotic liver shrinkage. Doppler ultrasonography revealed that PGE(1) infusion actually increased portal blood flow. In conclusion, based on the preceding experimental backgrounds and clinical experiences, continuous PGE(1) infusion via the SMA can be a useful measure to prevent severe liver damage after hepatic arterial flow interruption through portal blood oxygenation.

Jejunal varix with extrahepatic portal obstruction treated by embolization using interventional radiology: report of a case.

We report a case of relapsing jejunal varix with extrahepatic portal obstruction, which was successfully treated by embolization using interventional radiology. A 79-year-old woman suffered repeated episodes of tarry stools 2 years after undergoing jejunal resection for a jejunal varix. The bleeding point was inferred to be in the small intestine, and abdominal angiography revealed extrahepatic portal obstruction and the development of a jejunal varix around the hepaticojejunostomy. Because surgical obliteration of the varices or a shunt operation for portal decompression may have been very invasive due to severe adhesions, the jejunal varix was embolized with anhydrous ethanol and interlocking detachable coils. There were no changes in liver enzymes, the clearance rate of indocyanine green, or portal pressure, and there has been no sign of rebleeding for 13 months. Our experience shows that hemostasis can last, as long as the embolization can be done without aggravating portal hypertension. In conclusion, embolization using interventional radiology is a safe and useful method of treating intestinal varices.

Uptake of indocyanine green by hepatocytes under inflow occlusion of the liver.

BACKGROUND: It is not clear that hepatic venous backflow actually contributes to hepatic tissue oxygenation under inflow occlusion of the liver. In order to prove that substances delivered via the hepatic vein can be utilized and/or metabolized in hepatocytes during inflow occlusion, hepatic uptake in bile and excretion of indocyanine green (ICG) were investigated in pigs. MATERIALS AND METHODS: Animals were divided into two groups: an inflow occlusion (IO) group (N = 6) and a total hepatic vascular exclusion (THVE) group (N = 3) using a bypass. One milligram of ICG per kilogram body weight was administered at the beginning of blood flow occlusion, the retention rate in the blood (ICG R) measured, and the ICG in the hepatic tissue measured by near-infrared (NIR) spectroscopy. Furthermore, the ICG concentration was measured in bile excreted by intermittent perfusion of the liver. RESULTS: ICG R declined with time in both groups; however, ICG R in the IO group decreased much faster than in the THVE group. There were significant differences between the two groups after 30 min of occlusion (P < 0.05). ICG in the hepatic tissue could be detected as a peak at 805 nm 10 min after ICG injection, and the peak became steeper with time. On the other hand, ICG was not detected at all in the hepatic tissue after 180 min in the THVE group. ICG was excreted in the bile after 60 min under IO and increased with time. On the contrary, ICG was not excreted in the bile at all under THVE. There were significant differences between the two groups after 90 min (P < 0.05). CONCLUSION: These results indicate that ICG can be extracted in hepatocytes and excreted in bile under IO of the liver. Consequently, substances such as oxygen and drugs, which are delivered via the hepatic vein, can be utilized and/or metabolized in hepatocytes under IO.

Acute emphysematous cholecystitis preceded by symptoms of ileus: report of a case.

We herein describe a case of acute emphysematous cholecystitis in which the patient presented with symptoms of ileus. The patient was a 72-year-old man with no history of diabetes mellitus. He presented with epigastric pain, vomiting, and low-grade fever. Plain abdominal radiography showed some intestinal gas and niveau, and he was admitted to our hospital with a diagnosis of ileus. The next day, the abdominal pain increased and was accompanied by muscular defense. Plain radiography and computed tomography of the abdomen were carried out, and an emergency laparotomy was performed under a diagnosis of panperitonitis due to a perforation of the gallbladder caused by acute emphysematous cholecystitis. The patient made favorable progress after the operation and was discharged on postoperative day 14. Percutaneous transhepatic gallbladder drainage has been increasingly performed for the treatment of acute emphysematous cholecystitis. but when a perforation of the gallbladder is suspected, a laparotomy first should be considered.

Continuous arterial infusion of prostaglandin E(1) via the superior mesenteric artery in the treatment of postoperative liver failure.

Impaired hepatic blood flow is one of the causative factors in postoperative liver failure. To restore the hepatic blood flow in case of hepatic artery interruption (HAI), the effect of continuous arterial infusion of prostaglandin E(1) (PGE(1)), which has a strong vasodilatory effect on vascular smooth muscles, was assessed experimentally and clinically. Twelve pigs underwent ligation and division of the hepatic artery and were divided into 2 groups. In the control group, saline was infused in the superior mesenteric artery (SMA), and in the PGE(1) group, 0.02 microg/kg/min of PGE(1) was infused continuously in the SMA. Hepatic oxygen delivery (HDO(2)) in the control group was 87.8 +/- 8.9 ml/min before HAI and decreased to 43.1 +/- 2.6 ml/min at 60 min after HAI, showing 50.9% decrease by HAI. On the contrary, HDO(2) in the PGE(1) group was 86.7 +/- 9.1 ml/min before HAI and was 76.6 +/- 12.2 ml/min at 60 min after HAI, showing only 11.6% decrease by HAI. Clinically, a 65-year-old female suffering from cholangiocellular carcinoma underwent extended left hepatic lobectomy. At operation, the branch of the hepatic artery to the anterior segment of the liver was ligated, and the right branch of the portal vein became stenotic unavoidably. Postoperatively, severe liver dysfunction developed so that continuous PGE1 infusion in the SMA was initiated at a rate of 0.01 microg/kg/min on the eighth postoperative day and continued for 9 days. Plasma exchange was performed twice concomitantly. Portal venous flow increased from 612 ml/min to 1,192 ml/min, and bile flow from external biliary drainage tube doubled by the PGE(1) infusion. The liver function was ameliorated after PGE(1) infusion.