Background : Congenital biliary dilatation is a rare disease. Although the possibility of refractory cholangitis and/or the frequency of malignant tumours legitimize hepatobiliary surgery, repeated cholangitis and biliary obstruction result in secondary liver cirrhosis even after polysurgery. There are no definitive guidelines of liver transplantation for congenital biliary dilatation patients. Patients : A total of 1,101 liver transplantation recipients were enrolled in this study. Eleven congenital biliary dilatation patients including 5 Caroli's disease patients were retrospectively analyzed in detail. Results : Nine of eleven patients underwent initial surgeries before liver transplantation, while two Caroli's disease patients received liver transplantation as initial surgery with good outcomes. All patients had intractable symptoms caused by liver cirrhosis, and growth reduction was considerable in patients aged <20 years. Histopathological analysis of the native liver revealed hepatic fibrosis (≥F2). One patient with ABO incompatibility finally died. Though one Caroli's disease patient accompanied with intrahepatic carcinoma, this patient survives 11.8 years after liver transplantation without any recurrences. Conclusions : The congenital biliary dilatation patients with refractory symptoms and complications secondary to liver failure are appropriate candidates for liver transplantation. We suggested that liver transplantation become an effective therapeutic option for congenital biliary dilatation patients, under the thoughtful considerations of many supportive factors, such as clinical course, growth reduction, image findings and histopathological analysis.

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Purpose : We clarified the prognostic factors of primary colorectal carcinoma with peritoneal dissemination. Patients and methods : A total of 71 cases of operated primary colorectal carcinoma with peritoneal dissemination between 2002 and 2010 were reviewed. Results : Nineteen cases were categorized as P1 (for localized dissemination into adjacent peritoneum), 20 cases were P2 (for slight dissemination into remote areas of the peritoneum) and 32 cases were P3 (for marked dissemination into remote areas of the peritoneum). The 3-year overall survival rate of the patients with P1 and that of the patients with P2 were significantly better than that of P3 (50.0%, 48.2% and 9.6%, respectively P<0.01, P<0.05). Multivariate analysis showed that curativity (residual tumor classification) (HR, 3.91: P<0.05) was a significant prognostic factor affecting the overall survival rate. The 3-year overall survival rates of the patients with curativity B (Cur B: No macroscopic residual tumors) and curativity C (Cur C: with macroscopic residual tumors) were 78.4% and 15.9%, respectively (P<0.0001). In the patients with Cur B, the relapse-free survival rates and overall survival rates of the patients with P1 and those of the patients with P2 showed no significant differences (P=0.37, P=0.82). Conclusions : These findings suggest that the complete resection of primary tumor and metastatic lesions may prolong survival in patients with colorectal carcinoma accompanied by peritoneal dissemination.

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A 66-year-old man presented with epigastralgia and dysphagia. An irregular protruding lesion with an ulcer at the esophagogastric junction was seen endoscopically and on an upper gastrointestinal series. Abdominal CT scan showed enlarged lymph nodes near the left gastric artery and cardia, but we did not recognize malignancy in the biopsy specimen. The patient's condition deteriorated due to esophageal stenosis, therefore we performed total gastrectomy and lower esophagectomy. Macroscopicically, the resected specimen showed a protruding lesion with an irregular surface, which was covered with white epithelium. Histopathologically, the tumor consisted of squamous epithelium with little atypia, invading the deeper layer of the muscular layer of the esophagus. Furthermore the basal cell layer was stained by Ki-67 immunostaining, we finally diagnosed verrucous carcinoma of the esophagus. Verrucous caricinoma is a variant of well-differentiated squamous cell carcinoma and is very rare in esophageal cancer. We discussed our case based on a review of the literature.

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A 68-year-old man with esophageal cancer underwent a subtotal esophagectomy followed by a three-field lymphadenectomy and reconstruction using the left hemicolon. Nine days postoperatively, abdominal pain and tachypnea suddenly appeared. Enhanced abdominal computed tomography (CT) revealed hepatic portal venous gas and bowel pneumatosis. Emergency surgery was performed under a diagnosis of intestinal necrosis. At surgery, we found ischemic, edematous jejunum 20 to 120 cm from the ligament of Treitz, affecting the ileum 90 cm in an oral direction from the cecum. We resected this necrotic bowel and performed an ileostomy without anastomosis. Four days later, CT revealed that the intrahepatic portal venous gas had disappeared and there was no evidence of intestinal necrosis. We closed the colostomy, including the anastomosis, 12 days after the emergency operation. The patient had a relatively uneventful postoperative course. Hepatic portal venous gas is a rare complication in cases of ileus and bowel necrosis and is associated with a poor prognosis. Here, we report a case of hepatic portal venous gas associated with non-occlusive mesenteric ischemia in a patient after surgery for esophageal cancer.

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A 78-year-old woman was admitted to our hospital for abdominal pain and nausea at midnight. She had undergone total gastrectomy (antecolic Roux-en-Y reconstruction with splenectomy) for gastric cancer 9 years previously. Enhanced CT scan showed a severely dilated afferent loop with a beak sign and concentration of the mesentery, dilated intestinal loops without Kerckring folds, and a large amount of ascitic fluid. We made a diagnosis of strangulated ileus and acute afferent loop obstruction due to internal hernia involving the jejunojejunal end-to-side anastomosis created at total gastrectomy (Y anastomosis), and performed an emergency operation. At laparotomy, we found that the elongated reconstructed jejunum at total gastrectomy (Y arm) had adhered to the left diaphragm and formed an intestinal loop. A loop of the small intestine distal to the Y anastomosis had encircled the loop of the Y arm and formed a knot. The involvement of the Y anastomosis in this knot resulted in afferent loop obstruction. The operative diagnosis was an intestinal knot. After untwisting the knot, the blood supply to the congested Y arm and small intestine was improved, and a perforation at the posterior wall of the afferent loop was revealed. The perforated site was repaired, the adhesion of the Y arm to the diaphragm was dissected to straighten the Y arm, and a tube duodenostomy was created. Although minor leakage at the site of tube duodenostomy occurred, her postoperative clinical course was satisfactory, and she was discharged 27 days after surgery.

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A 46-year-old woman was admitted to our hospital because of epigastric pain. Esophagogastroduodenoscopy revealed a type 3 tumor in the anterior wall of the gastric fundus. Biopsy showed poorly differentiated adenocarcinoma. Ultrasonography and computed tomography of the abdomen demonstrated a tumor in segment 6 of the liver. Total gastrectomy with D2 lymph node dissection and right hepatectomy was performed. The resected specimens showed a type 3 tumor, 7.5×4.5 cm in size, in the anterior wall of the upper stomach and a 6.5×4.0 cm tumor in the right lobe of the liver. The tumor of the stomach demonstrated proliferation of atypical cells in large solid acinous formation from mucosal to subserosal layers. The tumor was diagnosed as a well-differentiated endocrine carcinoma (atypical carcinoid tumor). It contained many mitoses and the Ki-67 index was 32%. These data indicated high grade malignancy. The hepatic tumor was also diagnosed as a metastatic endocrine carcinoma from the stomach. The pathological classifications were pT3 (SS) pN0 pM1 pStage IV (Japanese Classification of Gastric Carcinoma The 14th Edition). Adjuvant chemotherapy including intravenous systemic infusion and intra-arterial hepatic infusion was performed. She is doing well without any recurrence 10 years after surgery. We report this case of long survival for 10 years after surgery for gastric atypical carcinoid tumor and simultaneous hepatic metastasis and review the relevant literature.

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A 54-year-old woman was admitted to our hospital with vomiting and jaundice. Abdominal enhanced CT showed wall thickening of the common bile duct and middle bile duct cancer was suspected. Pancreatoduodenectomy was performed, but histopathological findings showed only inflammatory changes without any signs of malignancy. Four years after the surgery, she suffered perforation of the ascending colon. Colostomy and drainage of a retroperitoneal abscess were performed. Three months after the colostomy, a right hemicolectomy was performed on the suspicion of colon cancer. The resected specimen of the transverse colon showed stenosis and wall thickening, but no evidence of malignancy. Two years later, partial resection of the ileum was performed due to ileal stricture. No malignant cells were observed. We then performed histopathological studies of all of the previous surgical specimens and found lymphoplasmacytic infiltration and fibrosis. On immunohistochemical examination, all specimens were confirmed to contain IgG4-positive plasma cells. This case was considered to be a rare case of sclerosing cholangitis and gastrointestinal strictures caused by IgG4-related sclerosing disease. After establishment of this diagnosis, steroid treatment was attempted for stenosis of the jejunum. However, because of the limited efficacy of the steroid treatment, gastrojejunostomy was performed. This patient is still alive 8 years after the first surgery and requires long-term follow-up for relapse of sclerosis.

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An unusual dilatation of the portal vein in a 57-year-old man on a screening examination. Enhanced CT showed an iso-enhanced tumor measuring 5 cm in diameter in the tail of the pancreas, accompanied by portal vein tumor thrombus (PVTT). We suspected tumor other than invasive ductal carcinoma and strongly considered non-functioning endocrine carcinoma with PVTT. The patient underwent distal pancreatectomy and extraction of the PVTT. Pathologically, tumor cells demonstrated ellipsoidal eccentric nuclei and eosinophilic cytoplasm in a rosette-like arrangement and a trabecular pattern. There were 0-1 mitoses/10 HPF. Metastasis to lymph nodes around the splenic artery and a marked vascular invasion were observed. On immunohistochemical analysis, Chromogranin, Synaptophysin and CD56 showed positive staining, while staining for pancreatic hormones was negative. The Ki-67 labelling index was less than 2%. These findings yielded a definitive diagnosis of well-differentiated non-functioning endocrine carcinoma according to the WHO classification. Surgery has resulted in survival without recurrence for 24 months to date. Non-functioning endocrine carcinoma with PVTT is very rare and only 4 cases had been reported previously. Three cases of well-differentiated carcinoma among those 4 cases showed good outcome due to curative resection. Surgical resection should be considered as the primary option for non-functioning endocrine carcinoma with PVTT.

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Tuberculosis (TB) morbidity has recently decreased in Japan, but in some categories it has increased, such the elderly and those vulnerable to opportunistic infections. The symptoms of intestinal tuberculosis (TB) are caused mainly by stenosis but rarely by perforation. We encountered a case of intestinal TB perforation developing in a 75-year-old woman who had been treated for Parkinson disease. During hospitalization for appetite loss gastro-intestinal perforation occurred. On emergency laparotomy we found 8 ulcers in the terminal ileum, and 2 of them had caused perforations. We resected 60 cm of the ileum, including the ulcerated sections and made an ileal stoma with an oral side ileum, designating the anal side ileum as the blind edge. Pathological findings of the ileum showed Langhans large cells and acid-fast staining positive bacterial bodies. A cavity lesion with a thick wall was seen in the left lung. The sputum culture revealed a Gaffky score of 5 number and polymerase chain reaction for TB was positive. We diagnosed intestinal perforation with secondary intestinal TB, and we referred her to an indstitution spializing in TB. Administration of 3 kinds of anti-TB drugs converted her TB culture to negative and she went to another rehabilitation facility 9 months after surgery.

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We report a case of small-intestinal sarcomatoid carcinoma in a 74-year-old man admitted with severe anemia. X-ray examination of the small intestine failed to show the lower ileum, and abdominal computed tomography (CT) showed lymph node swelling at the base of the mesenterium. A tumor of the small bowel was revealed 70 cm from the ileocecal junction, along with lymph node swelling around the mesenteric artery. A partial ileectomy was performed with tumor and mesenteric lymph node resection. On histological examination and immunohistochemical staining, pleomorphic/sarcomatoid carcinoma was diagnosed. The tumor cells showed positive reaction for epithelial markers AE1/AE3, CAM 5.2, and EMA, and the stromal marker vimentin. Staining for both CEA and squamous differentiation were negative. The patient died of multiple liver metastasis on post-operative day 40. Sarcomatoid carcinoma is a rare form of primitive carcinoma of the small intestine. This is the 22nd case reported in the literature to date.

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A 45-year-old man suffering intermittent melena for several years was referred to our hospital for detailed examinations. A capsule endoscopy and a double-balloon enteroscopy revealed a tumor with a depression on the smooth surface, at approximately 150 cm proximal to the ileocecal valve. We made a diagnosis of ileal carcinoma by biopsy, and a partial ileal resection with lymph node dissection was performed. The resected specimen showed a yellowish tumor measuring 1.2×0.8 cm in size with a depression on the smooth surface. Based on the histological examination and immunohistochemical staining we diagnosed ileal carcinoid tumor. The patient is doing well 2 years after the surgery without any signs of recurrence.

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We report a case of segmental ileal lipomatosis, which penetrated the mesentery. A 21-year-old man with lower abdominal pain showed right lower abdominal tenderness with slight rebound tenderness and guarding. Computed tomography showed ileocecal intussusception and some air had escaped from intestinal tract. We conducted laparotomy, finding ileocecal intussusception and pus on theileal mesentery. There was 20 cm of diffuse adipose tissue infiltration in ileum segmentally. It had caused an ulcer and penetrated the mesentery. Ileal lipomatosis is extremely rare, and this is the first report of diffuse infiltrative segmental type in Japan.

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A 53-year-old man with anemia was found to have a tumor in the ascending colon by colonoscopy. Endoscopic biopsy revealed the possibility of either leiomyoma or gastrointestinal stromal tumor (GIST), while CT revealed a tumor of the caudate lobe of the liver. Based on a suspected diagnosis of GIST in the ascending colon and a solitary liver metastasis, we performed ileocecal resection and caudate lobectomy. Leiomyosarcoma in the ascending colon and a liver metastasis were confirmed by histopathology, immunohistology, and gene analysis. The definition and treatment of GIST have undergone changes in recent times. Almost all cases formerly diagnosed as leiomyosarcoma of the gastrointestinal tract are now classified as GIST and are treated with molecular targeted agents such as imatinib. As a result, cases of true leiomyosarcoma of the gastrointestinal tract are few and treated solely by surgical resection. In this case, gene analysis was crucial for diagnosis and to determine the treatment course.

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A 65-year-old man underwent sigmoidectomy for colon cancer in 1997. Ten years after the first operation his serum CEA level increased, and FDG-PET showed abnormal accumulation in the left adrenal gland and the right lung. Abdominal and chest CT scan demonstrated a tumor in the same lesion of the adrenal gland and the lung. From these findings, adrenal and lung metastasis of colon cancer was suspected, therefore a partial resection of the upper lobe of the right lung was performed for diagnostic purposes. Pathological diagnosis of the tumor revealed metastatic adenocarcinoma of colon cancer. Then left adrenalectomy was performed successfully. Pathological findings again showed metastatic adrenal tumor of colon cancer. Serum CEA levels decreased gradually after the operation. However, 5 months after the surgery, FDG-PET disclosed abnormal accumulation only in the right adrenal gland, whereupon right adrenalectomy was performed additionally. In Japan, only 37 cases with resection of adrenal metastasis from colorectal cancer have been reported, probably because of non-resectability, and only 3 with bilateral adrenalectomy have been reported. We report a patient with colon cancer who had bilateral adrenal and lung metastases 10 years after the first operation, and review the relevamt literature.

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We report a case of solitary fibrous tumor (SFT) in the pelvic space. A 45-year-old woman who had been found to have a left ovarian tumor in a medical check-up underwent surgery. At laparotomy, a large smooth mass 7 cm in diameter was found in the left side of the pelvic space, and a needle biopsy was performed. Pathologically, the mass was diagnosed as a spindle cell tumor, suspected of being a low-grade malignant soft-tissue tumor. CT showed a large, round, heterogeneously enhanced, smooth-surfaced mass compressing the rectum and uterus. No invasion was detected. Colonoscopy showed that the lower rectum was compressed from the left side but that the mucosa appeared normal. Then the abdominosacral excision was performed. Macroscopic findings of the resected specimen showed an encapsulated yellowish-white elastic soft tumor sized 7.0×5.5×5.1 cm, colored. Histological examination demonstrated the tumor to be composed of cellular proliferation of spindle cells with a "patternless pattern" arrangement. Hypercellularity and pleomorphism were remarkable; however, mitosis was not seen. Immunohistochemical staining was positive for α-SMA, partially positive for vimentin and CD34, and negative for desmin, EMA, and S-100. These findings suggested a diagnosis of SFT in the pelvic space. The tumor had invaded the gluteus maximus muscle. The patient is alive without recurrence 7 years after the excision.

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A 21-year-old woman visited another facility, complaining of pain in the coccygeal region. CT scans and MRI revealed a tumor on the posterior side of therectum. The woman was referred to our hospital in November 2009. Surgical resection was performed on the basis of a preoperative diagnosis of pelvic retroperitoneal cystic mass of about 7 cm in maximum dimension. For cosmetic reasons, the operation was carried out by single-incision laparoscopic surgery and with a transperineal approach. The resected tumor was histopathologically diagnosed as mature teratoma. The postoperative course was uneventful, and the surgical wound became almost invisible one year after surgery. To date, no report has been published on transperineal resection of sacral teratoma in adults via single-incision laparoscopic surgery. We report one such case, with reference to the literature.

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We performed laparoscopic tension-free surgery for a case of spigelian hernia (SH) using composite mesh. A 88-year-old woman was referred to our hospital for evaluation of a painful palpable mass over her right lower abdomen. Abdominal ultrasonography and CT revealed a loop of the small intestine at the lateral edge of rectus abdominal muscle, and the hernial orifice was about 2 cm in diameter. This case was diagnosed as SH preoperatively, and laparoscopic repair of the hernia was performed. The hernial defect was located in the spigelian aponeurosis outside of the right rectus abdominal muscle, and this case was definitively diagnosed as SH under direct visual observation. Composite mesh was used to cover the hernia defect, and the edge of the mesh was fixed to the abdominal wall with 22 tacks. Laparoscopic surgery using composite mesh is suitable for SH, especially in aged patients, and it allows intraoperative diagnosis, less invasive surgery, and less mesh-related complications.

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