Introduction: Primary duodenal adenocarcinoma is a rare malignant gastrointestinal tract neoplasm. The unique anatomy of the duodenum makes many factors, such as treatment and surgical procedure, uncertain. Patients and Methods: We retrospectively analyzed clinical records of 27 patients who had undergone resection for primary duodenal adenocarcinoma between January 1992 and December 2007. Results: The rate of lymph node metastasis increased with tumor invasion depth. Most tumors invading beyond the serosal layer invaded the pancreas. Overall 5-year survival was 50.0%. Statistical analysis suggested that symptoms, serum CEA and CA19-9, tumor invasion depth, pancreatic invasion, and lymph node metastasis were significant prognostic factors. Discussion: Endoscopic or segmental resection is appropriate for patients whose tumor invasion depth is limited to the mucosal layer. Advanced cancer invading beyond the subserosa requires pancreaticoduodenectomy or pylorus-preserving pancreaticoduodenectomy with lymph node dissection in the pancreatoduodenal region, ceriac artery, and superior mesenteric artery. Rules and regulations must be determined for treating primary duodenal adenocarcinoma based on staging by numbers of lymph nodes involved in metastasis, tumor and pancreatic invasion depth, and lymph node grouping by tumor location.

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Introduction: We discussed postoperative-complication risk factors and evaluated the usefulness of Estimation of Physiologic Ability and Surgical Stress (E-PASS) in predicting postoperative complication in elderly patients with colorectal cancer. Methods: We studied clinicopathological features and postoperative complications in 82 patients over age 75 who underwent colorectal cancer surgery from 2002 to 2007. Based on E-PASS, preoperative risk score (PRS), surgical stress score (SSS), and comprehensive risk score (CRS), we calculated from physiological factors and operative severity, we evaluated the relationship between postoperative complications and E-PASS. Results: Patients were divided into Group A-46 patients without postoperative complications- and Group B-36 patients with postoperative complications. PRS, SSS, and CRS were higher in Group B than Group A. Patients with CRS≥0.5 numbered significantly more in Group B than in Group A, significantly more had perforation, undernutrition, PS of 2 to 3, ASA classification of 3 to 4. Multivariate analysis indicated that CRS≥0.5 was the most important risk factor for postoperative complications. Discussion: E-PASS appears to be useful in predicting postoperative risk in elderly patients with colorectal cancer.

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A 67-year-old man with erythema on the hands and face and adynamia of the extremities was found to be positive for anti Jo-1 antibody and diagnosed with dermatomyositis. Gastrointestinal endoscopy for cancer showed squamous cell carcinoma in the middle thoracic esophagus, necessitating esophagectomy with lymphadenectomy through right thoracotomy. Pathological examination of the resected specimen confirmed a poorly-differentiated squamous cell carcinoma, pStage IVa (pT3N4M0). He underwent adjuvant cisplatin and 5-fluorouracil chemotherapy, during the first course of which dermatomyositis skin symptoms such as pigmented discoloration and itching on both hands recurred. Symptoms were relieved by discontinuing chemotherapy. After esophageal cancer reccurence, dermatomyositis symptoms recurred. The man died 14 months after surgery. Dermatomyositis is often associated with malignant tumor, and its symptoms correlate with cancer progression.

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A 71-year-old man with calf edema and shortness of breath admitted for further examination was found to have a IgM-κ monoclonal protein (3,259 mg/dl) in serum and advanced gastric and rectal cancer. The histological diagnosis of bone marrow biopsy specimens was lymphoplasmacytic lymphoma (LPL), necessitating double filtration plasmapheresis preoperatively, followed by total gastrectomy and anterior rectal resection in May 2008. After an uneventful postoperative course and rituximab treatment 2 month after surgery, he attained a partial response. Lymphoplasmacytic lymphoma is comparatively rare and, to the best of our knowledge, no case combined with advanced gastric cancer synchronous with rectal cancer has been reported in the Japanese literature.

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We report two cases of benign biliary stricture in the hepatic hilum diagnosed as malignant biliary stricture. Both had obstructive jaundice and required biliary drainage. Computed tomography (CT) showed abdominal lymph node swelling, but cholangiography showed no primary sclerosing cholangitis. The bile duct in the hepatic hilum showed severe stenosis predominantly on the right side. Both cases were diagnosed as Bismuth type IIIa hilar cholangiocarcinoma. We conducted right hepatectomy and caudate lobectomy with extra hepatic bile duct resection. Pathological findings indicated extensive lymphoplasmacytic inflammatory infiltration under bile duct mucosa. The definitive diagnosis was chronic cholangitis with no finding of malignancy. When diagnosing a case of biliary stricture in the hepatic hilum, one should consider the possibility of benign biliary stricture other than hilar cholangiocarcinoma.

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An 80-year-old woman diagnosed with extrahepatic bile duct cancer was found in preoperative imaging to have the lower bile duct obstructed by a tumor and underwent pancreaticoduodenectomy. The tumor was 4×3.5×3 cm in size and showed a flat type with an infiltrating growth pattern macroscopically. Histopatholgically, the tumor consisted mainly of spindle-shaped cells and giant neoplastic cells showed in immunohistochemical staining to be diffusely positive for AE1/AE3, CK7, and vimentin, yielding a diagnosis of undifferentiated spindle and giant cell carcinoma arising from the lower bile duct. Pathological diagnosis was pT4, pN2, M0, fStageIVb. The MIB-1 labeling index was 80%, suggesting high tumor proliferation and a dismal prognosis. CT scan 21 days postoperatively showed multiple liver metastases and lymph node swelling around the superior mesenteric artery. She died of peritonitis and pleuritis carcinomatosa 39 days after surgery. Undifferentiated carcinoma of the common bile duct is extremely rare, and its biological features remain to be clarified beyond the facts of high malignancy and a dismal prognosis.

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We report herein a case of carcinoma in the intrapancreatic bile duct developing 13 years after initial choledochal cyst surgery without pancreatobiliary maljunction. A 41-year-old woman admitted for epigastralgia and weight loss. Thirteen years earlier, she had undergone choledochal cyst surgery (Alonso-Lej type Ia without pancreatobiliary maljunction), which involved choledochal cyst excision with Roux-en-Y hepaticojejunostomy. Upper gastrointestinal fiberscopy showed a severe pyloric stenosis and computed tomography showed a mass 25 mm in diameter at the head of the pancreas with severe duodenal bulb invasion. PET showed FDG accumulation at the pancreatic head, corresponding to the mass found in CT. Under a diagnosis of intrapancreatic remnant bile duct cancer, we conducted substomach-preserving pancreaticoduodenectomy. Histopathologic examination showed that tubular adenocarcinoma had developed in the intrapancreatic remnant bile duct with severe invasion to the duodenum and pancreas. Adjuvant chemotherapy with TS-1 (100 mg/body) was started 4 weeks postoperatively, and no evidence of recurrence or distant metastasis has been detected 6 months after the operation.

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A 45-year-old man who had had surgery for cerebellar hemangioblastoma at age 31 and having no family history of von Hippel-Lindau disease (VHL disease) was found in abdominal ultrasonography to have a nonfunctional pancreatic-head tumor. Following pylorus-preserving pancreaticoduodenectomy, the pancreatic tumor was diagnosed as neuroendocrine based on inmmunohistochemical staining. This case was not diagnosed as VHL disease due to absence of a family history of it. Fourteen years later, however, pancreatic tumor surgery confirmed VHL disease. Disease associated with VHL disease thus requires lomg-term follow-up to definitively confirm family VHL disease.

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A 66-year-old man admitted for abdominal pain and vomiting was found in abdominal contrast-enhanced computed tomography to have a thrombus of the superior mesenteric vein and thickened small-intestine walls. Laboratory test showed low protein S activity and protein S antigen, leading to a diagnosis of superior mesenteric vein thrombosis caused by protein S deficiency. Anticoagulation therapy using heparin relieved symptoms, but one month later, he was referred for intestinal obstruction. When his condition failed to improve with conservative therapy, we conducted laparotomy, finding ileal stenosis 120 cm distal from the Treitz ligament and inflammatory adhesion around the stenotic ileum. We resected about 10 cm of the stenotic lesion with end-to-end anastomosis, then conducted anticoagulation and antiplatelet therapy using warfarin and aspirin. The postoperative course was uneventful. Superior mesenteric vein thrombosis caused by protein S deficiency is rare, with only 12 cases reported to our knowledge in Japan.

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We report two cases of early signet-ring cell carcinoma of the colon. Case 1: A 61-year-old man undergoing colonoscopy and found to have a flat elevated lesion with a central depression in the cecum was diagnosed pathologically with adenocarcinoma, necessitating right hemicolectomy with lymph node dissection. Histologically, signet-ring cell carcinoma had infiltrated into the submucosal layer. The man remains healthy without tumor recurrence nine years after surgery. Case 2: An 80-year-old man undergoing postoperative colonoscopy after sigmoid colon cancer surgery was found to have a sessile cecal polyp resected endoscopically, histologically to be differentiated adenocarcinoma with signet-ring cell carcinoma massively infiltrating the submucosal layer, necessitating additional laparoscopic ileocecal resection. Histologically, no residual carcinoma or nodal metastasis was found. He was died two years postoperatively of septicemia without tumor relapse. Patients with early signet ring cell carcinoma of the colon thus have a favorable outcome when undergoing curative surgery with postoperative adjuvant chemotherapy.

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We report a case of endometrioid adenocarcinoma arising from rectal endometriosis 17 years after endometriosis surgery. A 54-year-old woman who underwent hysterectomy and left salpingo-oophorectomy for endometriosis at 37 years of age and unopposed estrogen therapy for 16 years developed lower abdominal pain after eating. Colonoscopy and barium enema showed a submucosal tumor in the left anterior wall of the middle third portion of the rectum that led to rectal narrowing. Positron emission tomography with fluorodeoxyglucose (FDG) showed only FDG accumulation in the same region. A biopsy specimen showed well-differentiated adenocarcinoma in which carcinoma cells were positive for cytokeratin 7 but negative for cytokeratin 20, yielding a definitive diagnosis of endometriosis-associated intestinal tumor (EAIT) necessitating low anterior resection. The histological diagnosis was endometrioid adenocarcinoma, and endometrial glands were seen in an adjacent tissue, together with regional lymph node metastases. Adjuvant paclitaxel and carboplatin therapy was then done. An intestinal tumor in a woman with a history of treatment for endometriosis and unopposed estrogen therapy should include the consideration of a differential EAIT diagnosis.

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A 43-year-old woman with neurofibromatosis type 1 (NF1) reporting hematochezia was found in colonoscopy to have a few small submucosal nodules in the lower rectum, confirmed from a locally resected specimen to be carcinoid tumor. Abdominal computed tomography (CT) showing a 3 cm mass in the rectal posterior wall and a 3.5 cm mass in the ileocolic mesentery, necessitated low anterior rectum resection and ileocolic tumor excision. Histological diagnosis showed both tumors to be plexiform neurofibromas. Ten carcinoids 1 to 6 mm in diameter were found in the resected rectum with lymph-node metastasis. In patients with NF1, neurofibromas may be present in the abdominal cavity. Carcinoid tumors may arise in the periampullary area but rarely in the rectum. We present a case with NF1 accompanying abdominal plexiform neurofibromas and multiple rectal carcinoids.

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We report a case of intraperitoneal angiosarcoma with hemorrhaging. A 66-year-old man admitted for significant anemia and gastromegaly was found in. A computed tomography (CT) and abdominocentesis showed to have a hemoperitoneal mass, but the hemorrhaging point was not clear. Emergency surgery to control hemorrhaging showed numerous dark red, individually bleeding nodules a few millimeters in size on the mesentery, together with multiple 2-3 centimeters nodes in the greater omentum regarded as the main hemorrhaging source. Omentectomy did not provide complete hemostasis. Examination of the abdominal organs showed no primary tumor. Histopathological findings suggested angiosarcoma, but an additional workup did not determine the primary tumor. The definitive diagnosis was angiosarcoma of the greater omentum or mesentery-extremely rare conditions with an overall dismal prognosis. Our patient died 33 days postoperatively.

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A 76-year-old man who had undergone proximal gastrectomy and was then diagnosed with severe pyloric ring stenosis from inoperable remnant gastric cancer due to massive local infiltration was treated using gastric and jejunal tubes inserted through a jejunostomy for gastric decompression. On postoperative day (POD) 18, TS-1 was administered as first-line chemotherapy through the jejunal tube. TS-1 pharmacokinetics were then monitored from drug administration on day (DAD) 1. Plasma tegafur (FT), 5-FU, gimeracil (CDHP), and oteracil potassium (Oxo) levels were then measured and adverse effects monitored. The highest plasma FT concentration was 1,920 ng/ml at 30 minutes, of 5-FU 89.2 mg/ml at 2 hours, of CDHP 162.2 mg/ml at 1 hour, and of Oxo 119.6 mg/ml also at 1 hour after TS-1 administration. No grade 3 or 4 adverse events were observed. The primary tumor began shrinking after 2 cycles, and the man has since undergone four regimen cycles. This case suggests that TS-1 administration through a jejunostomy can be continued long-term without adverse events and may be effective in patients unable to ingest oral medication.

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