[A Case of a Gastrointestinal Stromal Tumor of the Stomach with Lymph Node Metastasis].

A 87-year-old female was pointed out wall thickness in the upper part of gastric body for examination of anemia. The mass had a contrast effect, some of it protruded outside the wall, and the surrounding lymph nodes were enlarged. Upper endoscopy showed irregular ulcerative lesion with submucosal volume from posterior wall to the greater curvature in the upper part of gastric body. Biopsy was performed, and GIST of stomach was diagnosed. Surgery was performed for the GIST of the stomach. During open surgery, invasion of pancreatic tail was observed, therefore proximal gastrectomy with D1 lymph node dissection and distal pancreatectomy were performed. Pathologically, the tumor measured 95×78×65 mm with mitotic figures(38/50 high-power fields). Immunohistochemical analysis revealed that tumor cells expressed positive results for c-kit, α-SMA and CD34, and negative results for S-100 and desmin on the basis of the histology and immunostaining profile, the tumor was diagnosed as a GIST. The patient was classed as high risk according to Fletcher's risk classification. Tumor invades pancreatic tail, and lymph node metastasis was observed. She was discharged on the postoperative day 27 and alive without tumor recurrence at 6 months after surgery, not undergoing adjuvant chemotherapy.

[A Case of Resection of Leiomyosarcoma Suspected to Originate from the Right Ovarian Vein].

We present a 58-year-old female patient who underwent resection of a leiomyosarcoma arising from the right ovarian vein. She was referred to our hospital because of lower abdominal pain that had been present for 1 month prior to the visit. Ultrasound examination revealed a well-defined, smooth, lobulated, highly vascular mass(57 mm)adjacent to the distal portion of the duodenum. Contrast-enhanced computed tomography revealed the contrast enhancement mass (60 mm)located surround the right ovarian vein. In abdominal magnetic resonance image examination, the mass exhibited isointense signal on T1-weighted images, high signal on T2-weighted images, and restricted diffusion on diffusion- weighted images. We suspected primary leiomyosarcoma of the ovarian vein and proceeded with surgical intervention. On intraoperative findings, the mass was in contact with the duodenum and the inferior vena cava but dissection was easily achieved. We excised the mass together with the right ovarian vein. Pathological findings showed the mass was composed of proliferating spindle-shaped cells arranged in bundles. Some areas showed polygonal nuclear atypia and abnormal mitotic figures. Additional immunostaining showed positive for α-SMA, caldesmon, calponin, and negative for desmin, CD34, CKA1/AE3, S100. Based on the intraoperative findings, we diagnosed it as leiomyosarcoma arising of the right ovarian vein.

[A Case of Intussusception Caused by Ascending Colon Cancer That Underwent Elective Surgery without Repositioning].

An 81-year-old man visited his previous doctor with complaints of fever, vomiting, and diarrhea, and was transferred to our hospital on the 8th day after being admitted to the hospital for further treatment for a mass in the right side of his abdomen on CT. At the time of transfer, a fist-sized mass was palpable in the right side of the abdomen, but there was no tenderness, and blood tests showed that the white blood cell count was 10,700/µL and CRP 1.36 mg/dL, indicating a mild increase in inflammatory reaction. Contrast-enhanced CT showed an 8-cm diameter mass in the ascending colon with a stratified structure, and an antegrade intussusception with the mass as the advanced part. No intestinal obstruction was showed, and the contrast effect on the wall of the intestinal wall of the intussusception was favorable. Since the intussusception was already present on the previous CT scan, and since the patient also had severe aortic stenosis and a cardiothoracic examination was necessary, we decided on an elective operation. During the waiting period, lower gastrointestinal endoscopy revealed a type 1 tumor in the ascending colon, and biopsy revealed a highly differentiated tubular adenocarcinoma. The scope was not passed through to the oral side, and endoscopic repositioning was difficult. On the 19th day(12th day of admission), right hemicolectomy of the colon with D3 lymphadenectomy was performed. Since the descending duodenal wall was partially retracted into the area of the duplication, a partial resection of the duodenal wall was also performed, and the specimen was removed without releasing the duplication. Histopathologically, the tumor was diagnosed a 9.5×5.7 cm type 1 tumor with pT3, pN0, cM0, pStage Ⅱa(9th edition). The patient was discharged on the 9th postoperative day with good postoperative course.

[A Case of Ascending Colon Cancer with a Tumor Thrombus in the Superior Mesenteric Vein].

The progression of intravenous tumor thrombus in colorectal cancer is rare and reports on its resection are limited. This study reports a case of ascending colon cancer with a tumor thrombus in the superior mesenteric vein(SMV). A 44-year-old woman was admitted to our hospital for right, lower abdominal pain. Dynamic CT revealed an enhanced mass in the ascending colon and a tumor thrombus in the SMV. She was diagnosed with ascending colon cancer and an SMV tumor thrombus. An extended right hemicolectomy was performed. The SMV tumor thrombus extended from the gastrocolic trunk (GCT)to the right gastroepiploic vein and the anterior superior pancreaticoduodenal vein. To remove the tumor thrombus, a wedge-shaped incision was made through the SMV. Pathological examination showed a moderately differentiated adenocarcinoma of the ascending colon with extra-regional lymph node metastasis(No. 6)and intrapancreatic venous invasion. The pathological staging was pT4b, pN0, pM1a, pStage Ⅳa(Japanese Classification 9th edition). The patient was discharged on day 13 postoperatively. After discharge, 14 courses of mFOLFOX6 plus bevacizumab chemotherapy were administered. The patient is currently alive with no recurrence 15 months postoperatively.

[A Resected Case of Intussusception Due to Undifferentiated Carcinoma of the Colon].

An 89-year-old woman was pointed out to have anemia for a routine blood examination by her family doctor and was referred to our gastroenterological department for further examination. Colonoscopy showed a type Ⅰ tumor in the transverse colon and insertion of fiber across the tumor was difficult. On contrast enema using gastrographin, a crab's claw-like appearance was found. CT after contrast enema revealed a tumor, 5 cm in diameter with pseudokidney sign near the hepatic flexure of the transverse colon. Pathological examination of biopsy specimen proved the tumor to be a poorly differentiated adenocarcinoma. Thus, she was diagnosed with intussusception due to transverse colon cancer and we performed partial resection of the transverse colon without regional lymphadenectomy. Final pathological diagnosis of the tumor was undifferentiated carcinoma and tumor stage was pT3pN0cM0, pStage Ⅱa. She was discharged 13 days after surgery and alive without tumor recurrence at 7 months after surgery, not undergoing adjuvant chemotherapy.

[A Case of Conversion Surgery following Chemotherapy for HER2-Positive Advanced Gastric Cancer].

A 73-year-old female was referred to the gastroenterological department of our hospital for examination because of anemia. CA19-9 increased to 1,392 U/mL, and upper endoscopy revealed type 2 gastric cancer extending from the anterior wall to the lesser curvature in the lower part of the gastric body. Biopsy specimens revealed well-differentiated tubular adenocarcinoma, and HER2 was positive immunohistologically. Contrast-enhanced CT revealed wall thickness of the lower part of the gastric body with marked lymph node swelling beside the lesser curvature and a low-density nodule measuring 10 mm in diameter with rim enhancement in segment 5 of the liver, indicative of liver metastasis. Thus, she was diagnosed with unresectable advanced gastric cancer(cT3cN2M1, HEP, cStage ⅣB), and combined chemotherapy with capecitabine, cisplatin, and trastuzumab was administered. After 7 courses, CA19-9 normalized, and upper endoscopy revealed a scar of the primary tumor, and cancer was not detected in the biopsy specimen. On contrast-enhanced CT, lymph nodes beside the lesser curvature shrank significantly, and liver nodules disappeared. She was referred to our department for conversion surgery, and distal gastrectomy with D2 lymph node dissection was performed 7 months after her first visit to our hospital. Pathologically, tumor invasion was restricted to the lamina propria, and lymph node metastasis was not observed(ypT1aN0M0, ypStage Ⅰ A). She was discharged on postoperative day 13 and is alive without tumor recurrence at 7 months after surgery. She is not currently undergoing adjuvant chemotherapy.

[Case of Resected Retroperitoneal Dedifferentiated Liposarcoma Extending to the Posterior Mediastinum].

A 71-year-old woman visited our hospital for the examination and treatment of retroperitoneal tumor. CT showed a retroperitoneal tumor extending to the posterior mediastinum; the tumor pressed the IVC and widely abutted the aorta. On MRI, the tumor showed low intensity on T1WI and high intensity on T2WI and DWI. However, the tumor did not show signal reduction on an ADC map. PET-CT showed high accumulation at the tumor. The patient was diagnosed with sarcoma arising from the retroperitoneum. The tumor located on a part of the diaphragm was resected. Histological examination revealed spindle cells with atypical nuclear and multinuclear cells. There were no lesions of well-differentiated liposarcoma. Both CDK4 and MDM2 tested positive on immunohistological staining. Histopathologically, the tumor was diagnosed as dedifferentiated liposarcoma without any well-differentiated liposarcoma component. The postoperative course was uneventful, and she was discharged on the 13th day after surgery. Two months after surgery, no recurrence has been detected.

Cholecystohepatic duct detected during laparoscopic cholecystectomy: a case report.

BACKGROUND: The cholecystohepatic duct is a rare form of an aberrant hepatic duct that connects to the gallbladder. Although cholecystohepatic duct is reported to be a very rare anomaly, injury of cholecystohepatic duct during cholecystectomy may result in serious complications. Herein, we present a case of cholecystohepatic duct in the ventral branch of the right posterior inferior segmental bile duct detected during laparoscopic cholecystectomy. CASE PRESENTATION: A 77-year-old woman with cholecystolithiasis had been referred to our hospital for surgery. Drip infusion cholecystocholangiography-computed tomography revealed a bile duct branch without communication between the intra- and extrabiliary systems, although the existence of this aberrant hepatic duct was not suspected preoperatively. A 4-port laparoscopic cholecystectomy was performed. After critical view of safety was confirmed, the cystic artery and duct were divided after double clipping. During antegrade mobilization of the gallbladder from the gallbladder bed, a thin, white cord-like material connecting the gallbladder neck and bed was detected. After clipping and dividing it, a cholecystohepatic duct injury was recognized through rechecking the results of the preoperative examinations. Biliary reconstruction was considered unnecessary because of the lesion's small drainage area. The postoperative course was uneventful, and an enhanced computed tomography performed 6 months after the surgery revealed a dilation in the ventral branch of the right posterior inferior segmental bile duct. The patient's liver function remained normal, and she had no symptoms of cholangitis 42 months after the surgery. CONCLUSIONS: Although cholecystohepatic duct is a rare anomaly compared to other aberrant hepatic ducts, surgeons performing cholecystectomy should always keep its existence in mind to avoid serious postoperative complications. Ideally, preoperative detection of cholecystohepatic duct is preferable, but even if it is detected during surgery, the appropriate management according to the drainage area is also important.

[Two Cases of Gastric Cancer Diagnosed after Omental Patch Repair for Gastric Perforation].

Case 1: A 62-year-old man was urgently admitted to our hospital because of left lower abdominal pain. Abdominal CT showed gastric perforation and we performed omental patch repair emergently. Postoperative upper gastrointestinal endoscope revealed an ulcerative lesion in the lesser curve of upper area of gastric body, which proved to be a poorly differentiated adenocarcinoma by biopsy. Thus, we performed total gastrectomy with D2 lymph node dissection. Resected specimen revealed type 2 gastric cancer in the perforated area. Pathological stage was pT3pN0M0, pStage ⅡA. Adjuvant chemotherapy by S-1 was performed and he is alive without tumor recurrence 12 months after the first operation. Case 2: A 71-year- old man was urgently admitted to our hospital because of upper abdominal pain after dinner. Abdominal CT suggested gastric perforation and we performed emergent laparoscopic operation. Perforated lesion about 8 mm in diameter was found in the anterior wall of gastric body. After debridement, perforated lesion was closed with a running suture and additionally omental patch repair was performed. Pathologically, well-differentiated adenocarcinoma was detected in the debridement tissue, and he was diagnosed with perforated gastric cancer. Thus, we performed total gastrectomy with D2 lymph node dissection 28 days after surgery. Pathological stage was pT3pN0M0, pStage ⅡA. Adjuvant chemotherapy by CapeOX was performed and he is alive without tumor recurrence 12 months after the first operation.

[Resected Cases of Primary Duodenal Carcinoma-A Ten-Year Experience].

Primary duodenal carcinoma excluding tumors of the ampulla of Vaterare are rare, thus, therapeutic strategy has not been established. In this study, we investigated the treatment outcome of 7 cases of duodenal carcinoma resected in our hospital between January 2010 and December 2019. The tumor locations were the duodenal bulb in 5(71%), the descending part and the transverse part in 1, respectively. Distal gastrectomy was performed in 4 out of 5 bulbous cases, and pancreatoduodenectomy was performed in the other 3 cases. The pathological stage by the 8th edition of the UICC TNM classification was Stage Ⅰ(T1a/T2, N0)in 3 cases, ⅡA(T3, N0)in 1, ⅢA(N1)in 2, and ⅢB(N2)in 1. R0 resection was achieved in all cases. Adjuvant chemotherapy with S-1 was performed in 3 of 4 patients with Stage Ⅱ or more advanced Stage. There were no tumor recurrences in 4 patients with Stage Ⅰ and Stage ⅡA, but recurrence was occurred in 2 of 3 patients with Stage ⅢA or more. The surgical outcome for duodenal carcinoma without lymph node metastasis were good. On the other hand, the prognosis for advanced cases with lymph node metastasis were poor. Thus, the development of effective adjuvant chemotherapy is strongly expected.

Isolated pancreatic metastasis from malignant melanoma: a case report and literature review.

Isolated pancreatic metastasis from malignant melanoma is rare. Pancreatic metastasis is difficult to diagnose in patients with unknown primary malignant melanoma. Endoscopic ultrasound-guided fine-needle aspiration plays an important role in confirming the diagnosis. A 67-year-old woman was referred to our institution because of a mass in her pancreas. Computed tomography and magnetic resonance imaging revealed a 35-mm mass localized on the pancreatic tail, with low attenuation, surrounded by a high-attenuation rim. Endoscopic ultrasonography revealed a hypoechoic mass with central anechoic areas. Endoscopic ultrasound-guided fine-needle aspiration of the mass was performed, and the pathological diagnosis was malignant melanoma. Intense fluorodeoxyglucose uptake was observed in the pancreatic tail on positron emission tomography-computed tomography. No other malignant melanoma was found. Distal pancreatectomy was performed. Six months postoperatively, positron emission tomography-computed tomography revealed high uptake in the left nasal cavity, and biopsy revealed the mass to be a malignant melanoma, indicating that the primary site of the malignant melanoma was the left nasal cavity and that the pancreatic mass and peritoneal lesion were metastases. The patient had survived > 2 years after the distal pancreatectomy. Pancreatic resection of isolated pancreatic metastasis can possibly prolong survival; however, metastatic melanoma usually has poor prognosis.

[Two Cases of Complete Response to Trastuzumab Combined with Chemotherapy for Recurrence of HER2-Positive Gastric Cancer].

Case 1: A 67-year-old male underwent distal gastrectomy for advanced gastric cancer. Postoperative histopathological examination indicated pT2a, pN2, M0, pStage ⅢA. He received 4 courses of TS-1 with paclitaxel chemotherapy and TS-1 chemotherapy for 2 years. Three years and 5 months after surgery, computed tomography suggested lymph node metastasis of the mediastinum, so TS-1 with cisplatin(CDDP)therapy was administered. Five years and 10 months after surgery, recurrence occurred and docetaxel and CPT-11 were administered with no response. Since HER2 was overexpressed in the primary tumor, he was treated with capecitabine, CDDP, and trastuzumab(XPT)therapy. After 1 year and 6 months, the patient was considered to have achieved a complete response(CR), and after further trastuzumab therapy for half a year, CR was maintained for 12 years and 3 months after surgery. Case 2: A 59-year-old female underwent total gastrectomy for advanced gastric cancer. Postoperative histopathological examination indicated pT3, pN3a, M0, pStageⅢB. She received TS-1 chemotherapy for 1 year and 8 months. Computed tomography suggested paraaortic lymph node metastasis, and XPT therapy was administered. The patients responded well, and alternate administration of XPT and capecitabine and docetaxel(XT) was performed. Three years and 5 months after surgery, recurrence of lymphadenopathy occurred and intensity-modulated radiation therapy in addition to XPT/XT alternate therapy was introduced, leading to a CR 5 years and 8months after surgery. XT therapy was continued afterward, and CR was maintained for 9 years and 2 months after surgery.

Clinical guidelines for primary sclerosing cholangitis 2017.

BACKGROUND: Primary sclerosing cholangitis (PSC) is relatively rare disease and pathogenesis and methods of treatments were still not established. Then, we had conducted the making clinical guidelines to manage patients with PSC based on the literature review and expert opinions. These clinical guidelines were made for the medical doctors on the management of PSC, except child case of PSC. METHODS: We had employed modified Delphi method. The production committee decided guidelines, strength of recommendations and evidence level after reviewed literatures systematically, and The Expert panel evaluated those. The Scientific Committee of the Japan Biliary Association (JBA) evaluated revised guidelines, and the Public comments were collected on web site of JBA. RESULTS: We had made 16 guidelines about epidemiology/pathophysiology, diagnostics, therapy and prognosis. Also, we had made both diagnostic and therapeutic flow chart. CONCLUSIONS: We hope that these guidelines will contribute to the improvement and development of the medical care of PSC.

[A Resected Case of UR-LA Pancreatic Tail Cancer with Aortic Invasion after Chemoradiotherapy].

A 70-year-old male was referred to our hospital because of weight loss and epigastric discomfort. CT showed an irregularshaped, low-density tumor, 12 cm in diameter in the tail of the pancreas. This tumor widely invaded to the left kidney and to the anterior and left lateral sides of the aorta in spite of no involvement of celiac and superior mesenteric arteries. Moreover, it closely contacted with the stomach and the spleen. EUS-fine-needle aspiration biopsy of the tumor detected adenocarcinoma. Thus, he was diagnosed with UR-LA pancreatic cancer with aortic invasion. He received combination chemotherapy(S-1 plus gemcitabine[GEM])and 50.4 Gy 3-dimensional conformal radiation therapy, but this therapy had no expected effect. We changed the regimen to GEM plus nab-PTX. After 1 course of changed regimen, the tumor ruptured into the stomach and endoscopic debridement of the necrotic tissue was performed. Twenty-six days later, We performed distal pancreatectomy with splenectomy, total gastrectomy, left nephrectomy, left adrenalectomy, and segmental resection of the colon. The tumor was detached from the aorta as much as possible. The final diagnosis was pT3N0M0, pStage II A. Fifty-nine days after operation, we restarted GEM plus nab-PTX therapy. However, a cerebral infarction suddenly occurred, and we discontinued the chemotherapy. Five months after the operation, he died of cancerous peritonitis.

[A Case of Small Intestinal Neuroendocrine Tumor Detected One Year after Resection of Liver Metastasis].

A 49-year-old woman received a detailed examination for a myoma uteri, and a hepatic tumor was detected incidentally. A CT scan showed a tumor 6 cm in diameter in the posterior segment, which was irregularly enhanced. The tumor showed a low signal intensity on T1WI MRI and a slightly high intensity with high-density spots on T2WI. The tumor showed a low signal intensity in the hepatobiliary phase of the EOB-enhanced MRI. Percutaneous liver biopsy proved that this tumor was a grade 1 neuroendocrine tumor(NET G1). We examined her whole body in detail but found no primary lesions. Therefore, we made a diagnosis of primary hepatic NET or hepatic metastasis of an unknown origin and performed right hepatectomy. A year after the operation, a tumor was found in the jejunum. We made a diagnosis of NET by using endoscopic biopsy and performed partial intestinal resection. Histological findings showed NET G2(Ki-67 labeling index: 3.5%), which had venous invasion and one lymph node metastasis, suggesting that the jejunum was a primary lesion of NET. Three years and 2 months after the first operation, multiple liver metastases were found, and bland TAE was performed three times. Four years and 6 months after the first operation, we started sustained-release somatostatin analogues for tumor progression. She is still alive 5 years and 6 months after the first operation.

Evidence-based clinical practice guidelines for cholelithiasis 2016.

Cholelithiasis is one of the commonest diseases in gastroenterology. Remarkable improvements in therapeutic modalities for cholelithiasis and its complications are evident. The Japanese Society of Gastroenterology has revised the evidence-based clinical practice guidelines for cholelithiasis. Forty-three clinical questions, for four categories-epidemiology and pathogenesis, diagnosis, treatments, and prognosis and complications-were selected, and a literature search was performed for the clinical questions with use of the MEDLINE, Cochrane, and Igaku Chuo Zasshi databases for the period between 1983 and June 2012. The guidelines were developed with use of the Grading of Recommendations Assessment, Development, and Evaluation (GRADE) system. This article preferentially describes the clinical management of cholelithiasis and its complications. Following description of the diagnosis performed stepwise through imaging modalities, treatments of cholecystolithiasis, choledocholithiasis, and hepatolithiasis are introduced along with a flowchart. Since there have been remarkable improvements in endoscopic treatments and surgical techniques, the guidelines ensure flexibility in choices according to the actual clinical environment. The revised clinical practice guidelines are appropriate for use by clinicians in their daily practice.

[A Case of Primary Peritoneal Cancer Successfully Treated with Debulking Surgery and Postoperative Chemotherapy].

A 52-year-old woman with abdominal pain and a feeling of incomplete evacuation visited a local clinic. Enlargement of the right ovary was detected, and the patient was referred to the gynecological department of our hospital. CT and MRI revealed a round-shaped mass, 8 cm in diameter, with cystic and solid components in the Douglas pouch. The patient underwent a laparotomy under the diagnosis of ovarian cancer. Intraoperatively, both the ovaries appeared normal and the tumor strongly adhered to the rectum and uterus. An exploratory laparotomy was performed; the tumor was identified as unresectable, and the patient was referred to our department after the surgery. PET-CT revealed nodules in the liver and peritoneum, in addition to the main tumor. Gastrointestinal endoscopy and immunohistochemical examination of a needle biopsy of the main tumor did not lead to the identification of the primary lesion. Thus, debulking surgery was performed to alleviate the patient's complaints. Histologically, the tumor was diagnosed as a primary peritoneal clear cell carcinoma. One month after surgery, multiple liver metastases and swelling of the peritoneal lymph nodes occurred. Six courses of dose-dense TC therapy were administered, and the patient achieved a complete response. At 8 months after surgery, the patient is still alive without tumor recurrence.

Clinical practice guidelines for the management of biliary tract cancers 2015: the 2nd English edition.

BACKGROUND: The Japanese Society of Hepato-Biliary-Pancreatic Surgery launched the clinical practice guidelines for the management of biliary tract and ampullary carcinomas in 2008. Novel treatment modalities and handling of clinical issues have been proposed after the publication. New approaches for editing clinical guidelines, such as the Grading of Recommendations Assessment, Development and Evaluation (GRADE) system, also have been introduced for better and clearer grading of recommendations. METHODS: Clinical questions (CQs) were proposed in seven topics. Recommendation, grade of recommendation and statement for each CQ were discussed and finalized by evidence-based approach. Recommendation was graded to grade 1 (strong) and 2 (weak) according to the concept of GRADE system. RESULTS: The 29 CQs covered seven topics: (1) prophylactic treatment, (2) diagnosis, (3) biliary drainage, (4) surgical treatment, (5) chemotherapy, (6) radiation therapy, and (7) pathology. In 27 CQs, 19 recommendations were rated strong and 11 recommendations weak. Each CQ included the statement of how the recommendation was graded. CONCLUSIONS: This guideline provides recommendation for important clinical aspects based on evidence. Future collaboration with cancer registry will be a key for assessment of the guidelines and establishment of new evidence. Free full-text articles and a mobile application of this guideline are available via http://www.jshbps.jp/en/guideline/biliary-tract2.html.