Purpose: To evaluate the efficacy, significance and safety of a ratings-based stepwise surgical training program to teach pancreaticoduodenectomy (PD). Materials and Methods: The target group consisted of twelve trainee doctors and three senior surgeons. Their surgical skills were assessed using a rating system based on structured objective assessment forms that evaluated basic surgical techniques and operative ability in stepwise PD. This stepwise PD comprised three stages: A - basic processes such as laparotomy, B - processes common to surgical procedures for gastric and colonic cancer and C - processes specific to PD. The trainees were ranked on their performance into one of four levels (1–4). Those ranked at level 3 or above were qualified to perform PD, and those at level 4 were qualified to perform PD in pancreatic cancer patients who require vascular resection. These levels were determined by the combined score of A+B or the score for C. The assessment process examined the following factors: 1. Change in a trainee’s total score over time and the number of completed operations, 2. Comparison of surgical outcome between the trainee and senior surgeon groups, and 3. Analysis of questionnaire results. Results: The total score for A+B increased with each operation in the trainee group once the four doctors at level 2 or below were excluded, while the score for C varied. The number of completed operations also increased over time. The mean operative time was significantly shorter in the senior surgeon group than in the trainee group (P<0.05). However, no differences were found with respect to mean blood loss, incidence of complications or postoperative death. The questionnaire results indicated that the surgical procedures and staging need some improvement. Conclusion: The ratings-based stepwise surgical training program in PD was efficient in improving processes common to surgical procedures for gastric and colonic cancer, but it was not sufficient on its own to train doctors in processes specific to PD. There was no risk to patient safety in this program, regardless of the surgeon’s skill level.

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A 42-year-old man presenting with dysphagia and who underwent esophagogastroduodenoscopy was found to have a tumor in the lower thoracic esophagus with hiatus hernia and a submucosal gastric tumor. We suspected he had Barrett’s esophagus because endoscopy revealed that the columnar epithelium existed at the proximal side of the oral margin of the longitudinal folds along the greater curvature of the stomach. Histologically, biopsy specimens of the esophageal tumor indicated adenocarcinoma but gastric tumor specimens suggested no malignancy. CT showed swelling of several abdominal lymph nodes. He was treated with subtotal esophagectomy and two-field lymph node dissection with right thoracotomy followed by a reconstruction procedure using a gastric tube. The submucosal tumor of the stomach was resected at the preparation of the esophageal substitution. He had complications of left recurrent laryngeal nerve palsy, but the postoperative course was uneventful. Therefore, he was discharged on postoperative day 16. Histopathological examination of the resected specimen showed adenocarcinoma in the submucosal layer of the stomach and esophageal gland beneath the overlying columnar epithelium. A final diagnosis of Barrett’s esophageal adenocarcinoma with intramural metastasis to the stomach was given. Because of 16 lymph node metastases from the upper mediastinum to the abdomen, he received chemotherapy with S-1 and cisplatin followed by S-1 for one year. The patient has been well without recurrence for 41 months after esophagectomy.

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An 82-year-old man was transported to our emergency room with hematemesis, melena, and dyspnea. He became hemodynamically unstable during the examination. The blood test revealed increased inflammatory response and endotoxin positivity. We diagnosed septic shock and he was admitted. After hospitalization, he had signs of peritonitis and we performed emergency laparotomy. Operative findings revealed that there were segmental necrosis of the jejunum and duodenum. We diagnosed non-occlusive mesenteric ischemia (NOMI). Due to his hemodynamic instability, we decided to resect the necrotic jejunum and duodenum without anastomosis and wound closure. We performed the second operation on day 3. The duodenal necrosis extended to the 3rd portion, so we added partial duodenectomy and performed duodeno-jejuno anastomosis. Here, we report a rare case of NOMI with duodenal necrosis.

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A 26-year-old man suffering from right hypochondriac pain was referred by a local physician due to a mass with heterogeneous internal echoes (size, 80 mm) in the right lobe of the liver shown by abdominal US. Our hospital performed a CT scan and a low-density area (size, 100 mm) was observed. The tumor was inhomogeneously hypointense on T1-weighted images and hyperintense on T2-weighted images. T1- and T2-weighted MRI showed a high-intensity lesion in part of the mass, considered to be due to blood components. According to selective angiography, the lesion was found to be hypovascular. A malignant tumor was suspected, and a right lobectomy was performed. Based on histopathological findings, a malignant fibrous histiocytoma (MFH) was diagnosed. Eight months later, a similar tumor was detected in the right diaphragm by CT scan, and this was resected after laparotomy. Six months after the operation, a single recurrent mass was observed in S4 of the liver, and a partial hepatectomy was performed. At present, 5 years after the first operation, the patient is alive and has not experienced recurrence. According to the best of our knowledge, only 56 patients with primary hepatic MFH have been reported, and there were few patients for whom surgery could be selected for recurrence. We report this case along with a review of the literature.

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A 76-year-old man with general fatigue visited our hospital. The laboratory date showed marked leukocytosis. Dynamic CT showed a liver mass in the right lobe, 8 cm in diameter, slightly enhanced only in the peripheral region. Although it was difficult to determine whether or not the liver mass with central necrosis was a malignant tumor or benign abscess, the tumor was considered to be malignant because symptoms including fever, leukocytosis and elevation of CRP did not improve by medical treatment such as antibiotic administration and drainage. Furthermore, we diagnosed this granulocyte-colony stimulating factor (G-CSF) producing tumor as causing marked leukocytosis (71,000/μl) and elevation of serum G-CSF level (800 pg/ml). Right hepatectomy with resection of the right lower lobe of the lung was performed. Immunohistochemical analysis of the resected tumor with anti-G-CSF antibody revealed positive staining in the cellular cytoplasm of the tumor cells. After surgery, although the leukocyte count normalized once, it rapidly began to elevate again. The tumor recurred locally, his physical condition gradually deteriorated, and he died on postoperative day 32. So far, only 15 cases of G-CSF producing liver tumor have been reported, and the characteristics of this rare tumor are discussed in this paper.

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A 46-year-old man with a past history of gastrointestinal bypass operation was admitted to our hospital because of epigastric pain and jaundice. CT showed not only stones in both the gallbladder and common bile duct, but also anatomical anomalies, with the stomach and liver in the normal position, and the horizontal part of the duodenum running from left to right behind the superior mesenteric artery and vein, resulting in partial situs inversus distal to the duodenum. Right-sided round ligament, annular pancreas, and polysplenia were also apparent. The patient was given a diagnosis of cholangitis. We first tried biliary drainage and choledocholithotomy endoscopically, but due to difficulty approaching the papilla of Vater, this proved unsuccessful. We therefore performed cholecystectomy and choledocholithotomy as soon as possible. The gallbladder was on the left side of the round ligament and the cystic duct joined the common bile duct from the left side. No stones were found in the common bile duct, but a retrograde transhepatic biliary drainage (RTBD) tube was placed preparatory to choledocholithotomy for the remaining stones. The postoperative course was uneventful. The RTBD tube was removed 1 month postoperatively.

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A 77-year-old woman visited a nearby hospital with abdominal pain in June 2009. Calculus cholecystitis was suspected from the findings of abdominal US and CT; thus, the patient was referred to our department. The enlarged gallbladder was palpable in the right hypochondrium, but the abdomen was soft and there was no tenderness. Abdominal US, CT, and MRI showed significant enlargement and wall thickness of the gallbladder. Gallstones were noted in the gallbladder neck, and retention of inspissated bile or biliary sludge was suspected; however, no evident tumorous lesions were identified. A diagnosis of calculus cholecystitis was made and cholecystectomy was performed. Histopathological examination of the resected specimen revealed findings consistent with mixed adenoneuroendocrine carcinoma, and additional regional lymph node dissection for gallbladder cancer was performed. The regional lymph nodes showed evidence of metastasis, and the final clinical staging was stage IIIB. Adjuvant chemotherapy with gemcitabine hydrochloride was administered; however, CT and PET after the completion of chemotherapy revealed metastases in segment 8 of the liver and abdominal para-aortic lymph nodes. CPT-11 monotherapy was started based on the treatment strategy for recurrent small cell lung cancer, resulting in the disappearance of the metastatic foci. To the best of our knowledge, this is the first case report in which complete remission was achieved by CPT-11 for recurrent mixed adenoendocrine carcinoma. The present case suggests the usefulness of CPT-11.

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A 64-year-old man visited our hospital because of jaundice, and was given a diagnosis of pancreatic head cancer. A preoperative screening examination revealed prolonged prothrombin time (17.7 sec.) and activated partial thromboplastin time (63.1 sec.). The value of factor V was 5%. We found the patient had pancreatic head cancer with ‍congenital factor V deficiency, so we transfused fresh frozen plasma (FFP) preoperatively in order to improve ‍prothrombin time and activated partial thromboplastin time. Under FFP transfusion, we conducted pancreatoduodenectomy successfully. We transfused 4 units of FFP for 4 days postoperatively. There was no serious tendency to hemorrhage during the operation or in the postoperative period.

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We report a case of a ruptured small cystiform gastrointestinal stromal tumor (GIST) of the small intestine related to the peritonitis. An 87-year-old woman complaining of abdominal fullness and lower abdominal pain was brought by an ambulance. CT scan showed a cystiform lesion with fluid collection just attached to the ileum in the pelvis, accompanied by ascites and peritonitis (high density CT value of the mesentery). Then emergency laparotomy was performed, and the operative findings showed bloody ascites and a ruptured cystiform tumor (about 5 cm in diameter) arising from the ileum. We performed partial resection of the ileum, intraperitoneal irrigation and drainage procedure. Histologically, section shows a proliferation of spindle cells and epithelioid cells with sheets or interlacing pattern and immunohistohemically, tumor cells are positive for c-kit. Consequently the tumor was diagnosed as GIST (gastrointestinal stromal tumor) of the small intestine. The patient has survived for a year disease-free after the operation.

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Lynch syndrome, one of the most well-documented hereditary cancer predisposition syndromes, is associated with mutations in mismatch repair genes such as MLH1, MSH2, MSH6, and PMS2. We report here the case of a 25-year-old woman with transverse colon cancer associated with MSH6 mutation. The patient underwent curative segmental colectomy. Since the patient met the standards of the revised guidelines, we performed microsatellite instability (MSI) profiling and immunohistochemical analysis. The tumors were found to be MSI-H, and MSH6 expression was not detected; based on these results, Lynch syndrome was suspected. Germ line mutation analysis of MSH6 by direct sequencing revealed a frame-shift mutation in codon 3261 of exon 5; this mutation has been reported to be pathogenic. Our findings suggest that screening cancer patients for Lynch syndrome would help predict the risk of their family members developing cancer.

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The patient was an 84-year-old man with a history of anal fistula since the age of 60 years. He underwent radical surgery for anal fistula three times at a local clinic. Since the symptoms did not improve, he was referred to our department. CT and MRI revealed a 30-mm, septated cystic lesion on the dorsal side of the anal canal. Biopsies from the lesion showed no evidence of malignancy. The lesion, which tended to enlarge during follow-up, was resected transanally. Histopathological examination of the resected specimen showed no evidence of malignancy. The cystic lesion recurred 6 months after surgery, and tended to enlarge. Fine-needle aspiration cytology of the inracystic mucus revealed no evidence of malignancy, and PET-CT showed no significant uptake. However, the serum CEA level was slightly elevated at 10.0 ng/ml, and the CEA level in the intracystic mucus was abnormally high. Therefore, abdominoperineal resection of the rectum with D2 lymphadenectomy was performed, and the lesion was histopathologically diagnosed as anal fistula cancer. It is often difficult to diagnose this condition. In the present case, anal fistula cancer could not be diagnosed by imaging or repeated biopsies, and immunochromatography of the intracystic mucus was helpful in the diagnosis of this condition, leading to radical surgery.

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Enterocolic lymphocytic phlebitis (ELP) is a disease entity characterized by lymphocytic inflammation of the bowel wall and mesenteric veins and venules that cause intestinal ischemia. We report two cases of ELP with a review of the literature. Our first case was of a 26-year-old man with a diagnosis of acute peritonitis with gastrointestinal perforation, in which emergency laparotomy was performed. Multiple wall thinness and perforations at the ascending colon were seen and he underwent a right hemicolectomy. The second case was of a 32-year-old woman who presented with a 5-day history of cramping, abdominal pain, and diarrhea. Clinical findings and CT findings of the segmental wall thickness of the upper jejunum and markedly dilated changes of the oral side of the intestine with associated ascites roused the suspicion of strangulation obstruction, and emergency laparotomy was performed. Partial jejunectomy was performed due to findings of segmental redness and wall thickness of the jejunum and markedly dilated changes on the oral side of the intestine. Both cases were diagnosed as ELP from their histopathological findings of artery-sparing phlebitis and associated ischemic changes.

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Dihydropyrimidinase (DHP) is a key enzyme in the catabolism of 5-fluorouracil (5-FU). DHP deficiency is a very rare autosomal recessive disease. To the best of our knowledge, only 28 patients have been reported so far. We report a case of a 56-year-old woman who underwent ileocecal resection for cecal cancer. She was pathologically, a poorly differentiated adenocarcinoma, of pathological stage IIIa (pT4a, pN1, pM0), was diagnosed. She was subsequently treated with capecitabine chemotherapy in the adjuvant setting starting on postoperative day 37. However, she had to discontinue treatment 10 days later due to severe oral mucositis. The patient also suffered from diarrhea, vomiting and high fever, and then developed severe leukocytopenia, disseminated intravascular coagulation (DIC), and disturbance of consciousness. Eventually, her condition worsened and became critical. We performed transfusion with granulocyte-colony stimulating factor and hemodialysis, and administrated antibiotics and treatment for DIC. Leukocytopenia started to improve 13 days after interruption of chemotherapy. Over time, the patient recovered and was discharged on day 65. Urinary pyrimidine analysis and DHP gene analysis by Sanger’s direct sequencing method revealed that she had compound heterozygous mutations, 1001A>G (Q334R) and 1393C>T (R465X), in the DHP gene. To our knowledge, this is the first report of DHP deficiency with severe 5-FU-associated toxicity in a Japanese patient.

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