Background/Aims: Previously rare malignant peritoneal mesothelioma appears to be increasing in incidence. We assessed the outcome of treatment for fatal malignant peritoneal mesothelioma cases in Japan Patients and methods: Among the 4,860 fatal mesothelioma cases extracted from the document “Vital Statistics of Japan” for 2003-2008, we obtained 805 cases from medical institutes with the permission of the families of the deceased and medical institutes. Among the 805 cases, we identified 114 cases with malignant peritoneal mesothelioma. Results: The 81 men and 33 women had a mean age of 64 years old. A past history of exposure to asbestos was confirmed in 61 of the 114 patients. A definitive diagnosis was obtained by open surgery and laparoscopy in 83 cases. Histologically 61 had epithelial, 10 biphasic, and 12 sarcomatoid mesothelioma. Chemotherapy had been conducted in 63, operation in 12, and chemotherapy + operation in 3. Overall median survival time was 4.8 months. With chemotherapy only, overall median survival time was 8.3 months. With operation, overall median survival was 4.7 months. In a multivariable analysis by the Cox proportional hazards model, age, gender, absence of abdominal pain, and histological type were prognostic factors. Chemotherapy and operation were not prognostic factors. Conclusion: There was a higher frequency of women and epithelial type in peritoneal mesothelioma than in pleural mesothelioma. Malignant peritoneal mesothelioma had lower populations of asbestos exposure, but an etiological relationship with highly concentrated asbestos exposure. Malignant peritoneal mesothelioma had a poor prognosis, and chemotherapy and operation were not found to be effective treatments based on this evaluation.

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Patient was a 60-year-old man who underwent an operation of gastric ulcer 2 years ago. He was transferred from another hospital for abdominal pain and hematemesis. Although vital signs were stable, physical examination demonstrated muscular rigidity and peritoneal signs in the abdomen. An abdominal CT showed massive air in the retroperitoneal space, portal vein, and intestinal wall besides free air under the diaphragm. Laparotomy was performed, expecting massive necrosis of the intestine. A large amount of turbid ascites was found and culture of the fluid later revealed candida albicans infection. The peritoneum was markedly inflamed, and the wall of the colon and mesocolon contained emphysematous cysts down to the rectum. Perforated gastric ulcer, 20 mm in size, was found at the anterior wall of the lower gastric body and was concealed by the transverse mesocolon. The ulcer was closed and abdominal cavity was drained. Massive air was thought to escape from the stomach into the surrounding loose connective tissue and portal venous system.

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A 55-year-old woman, who had previously undergone surgery for congenital biliary dilatation at the age of 8 years, had been suffering from recurrent right hypochondralgia with fever for the past year. Cholangitis was diagnosed and she was treated conservatively. However, her serum carcinoembryonic antigen level was elevated and computed tomography revealed a mass lesion in the pancreas head. She was therefore referred to our department for further evaluation and treatment. Percutaneous transhepatic biliary drainage was performed for obstructive jaundice, and a cholangiogram revealed bile duct cancer at the hepaticojejunostomy anastomotic site. She then underwent an internal drainage operation followed by pancreaticoduodenectomy. Macroscopically, an elevated lesion was observed in the inferior bile duct with invasion to the site of the hepaticojejunostomy and the parenchyma of the pancreas. A histological examination revealed moderately differentiated adenocarcinoma. Postoperative course was uneventful and she was discharged 30 days after the operation. She is doing well without any signs of recurrence at 4 years after the operation. We herein describe a rare case of bile duct cancer arising 47 years after an internal drainage operation for congenital biliary dilatation.

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A 79-year-old woman with obstructive jaundice was admitted to Akita Red Cross Hospital. Abdominal computed tomography showed a low density tumor in the head of the pancreas. Magnetic resonance imaging indicated tumors not only in the head but also in the tail of the pancreas. Magnetic resonance cholangiopancreatography revealed irregular narrowing in the tail of the pancreas. We diagnosed both tumors as pancreatic cancer. To retain glucose tolerance, pancreaticoduodenectomy, distal pancreatectomy and splenectomy were performed. Pathological examinations showed the pancreatic head tumor to be moderate differentiated tubular adenocarcinoma with invasion to the bile duct and the duodenum. The pancreatic tail tumor consisted of fibroinflammatory lesions with infiltration of lymphocytes and IgG4-positive plasma cells, and was diagnosed as IgG4-related autoimmune pancreatitis. Lower common bile duct was thickened with fibrosis and lymphoplasmacytic infiltration was observed. Obstructive jaundice was due to autoimmune pancreatitis related sclerosing cholangitis, not due to pancreatic cancer invasion. We could not definitely establish a preoperative diagnosis because cases of pancreatic cancer and autoimmune pancreatitis appearing as two different tumors are rare.

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A 55-year-old man underwent right nephrectomy for right renal cell carcinoma in 1996. Abdominal computed tomography revealed a mass in the spleen, which had not been detected one year previously in August 2010. He complained of no symptoms and had no history of trauma or inflammatory diseases. Ultrasound revealed a solitary, hypoechogenic nodule 2 cm in diameter with slight blood flow and a clear margin, in the mid-spleen. The possibility of a neoplastic lesion involving hemorrhage was suggested by magnetic resonance imaging. We attempted enhanced computed tomography and fluorodeoxyglucose positron emission tomography/computed tomography, but did not reach a definitive diagnosis. The patient underwent splenectomy in September 2010 because of the possibility of malignancy. The pathological diagnosis using immunohistochemistry for CD31, CD34 and CD8 was sclerosing angiomatoid nodular transformation. This disease is a rare tumor-forming disorder of the spleen. We found only 5 other case reports in Japan. We report this case along with a clinical analysis of the 6 case reports.

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A 49-year-old man underwent upper gastrointestinal series during a medical checkup in February 2009. Two days later he had abdominal pain with fever, and visited our hospital on the following day. He was found to have diffuse tenderness and muscular guarding upon abdominal physical examination and an elevated inflammatory response in blood tests. Abdominal X-ray and computed tomography showed free air, ascites and outlined collection of barium in the lower abdomen. Emergency operation revealed turbid ascites due to the perforated Meckel’s diverticulum located 55 cm oral from the terminal ileum. The diverticulum formed a cystiform shape in the distal half to the connecting pedicle with constriction on the boundary between them. Diverticulectomy was performed. Pathological examination showed the mural necrosis of the cystiform portion of the diverticulum, with no evidence of ectopic mucosa. The perforation of the diverticulum in our case was thought to be caused by increased internal pressure and chemical stimulation due to the stagnated barium collection in the cystiform portion, due to constriction and stenosis on the boundary between the two portions.

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We present a very rare case of a patient that caused delayed and multiple stenoses of the jejunum and sigmoid colon after abdominal injury. A 20-year-old man, who had suffered abdominal injury in a traffic accident, developed bowel obstruction 12 days later. We started treating this patient conservatively via an ileus tube, but no improvement was obtained. Therefore, we performed operation 15 days after the ileus tube had been inserted. During the laparotomy, we found three sites of stenosis in the jejunum at 120 cm on the anal side from the ligament of Treitz and also stenosis in the sigmoid colon. We therefore performed partial resections of both the jejunum and sigmoid colon. Though laparoscopic surgery was considered, we selected open laparotomy in this case to fully investigate the intra-abdominal findings. When we treat patients with delayed bowel obstruction after abdominal injury, it is necessary to fully recognize the possibility of irreversible bowel stenosis, and also the possibility of the presence of multiple stenoses.

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A 46-year-old man was admitted with dizziness and abdominal pain. Laboratory tests showed severe anemia. Computed tomography (CT) scan showed a large heterogeneous mass and fluid collection in the abdominal cavity. Under a diagnosis of intra-abdominal hemorrhage from the intra-abdominal mass, we performed an emergency operation. Laparotomy revealed a 12×10×6 cm solid tumor of the ileum with bleeding at about 150 cm distant from the ligament of Treitz, accompanied by peritoneal metastases. Partial resection of the ileum and coagulation of peritoneal metastases by electric knife were performed. Pathological examination showed interlaced bundles of large bizarre spindle-like tumor cells, high cellularity and 12 mitoses per 50 high-power fields. Immunohistochemical staining showed positive responses for c-kit and CD34, but negative responses for α-smooth muscle actin (SMA) and S-100 protein. Based on the above findings, the tumor was diagnosed as a high risk malignant gastrointestinal stromal tumor (GIST) of the small intestine. C-kit mutation analysis showed tumor had mutation at exon 11 of the c-kit gene. His postoperative course was uneventful. On the 14th postoperative day, oral administration of imatinib mesylate at 400 mg/day was started. The patient has been followed up for 5 years with no evidence of recurrence.

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An 81-year-old woman was admitted to our hospital for appetite loss and weight loss. She was diagnosed as having a carcinoma of the ascending colon, accompanied by para aortic lymph nodes, multiple lung metastases, and disseminated lesions. To evade intestional obstruction, we performed right hemicolectomy. After the operation, low grade fever and leukocytosis continued without any evidence of infection. Elevated serum Granulocyte-colony stimulating factor (G-CSF) level (124.0 pg/ml) was noted. An immunohistochemical study showed positive staining for G-CSF in the tumor cells. We diagnosed that the tumor was a G-CSF producing tumor. Postoperative FOLFOX4 therapy reduced para aortic lymph nodes and lung metastases, although ascites increased. The patient died 7 months after the operation. FOLFOX4 might be a beneficial therapeutic option for G-CSF producing tumors.

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A 70-year-old man complained of hematochezia. Colonoscopy showed a type-2 tumor in the transverse colon. CT showed that the transverse colon tumor directly invaded the greater curvature of the stomach. Esophagogastroduodenoscopy showed a submucosal tumor in the greater curvature of the middle stomach which was thought to be extramural gastric invasion on endoscopic ultrasonography. At laparotomy, we observed the colon tumor directly invading the greater curvature of the stomach. Transverse colectomy combined with partial gastrectomy was performed. However, frozen section showed cancer cells with vascular invasion at the resected stump of the gastric wall, so distal gastrectomy was added. On the cut surface of the resected specimen, the colon tumor invaded the gastric wall showing extramural progression. Microscopically, the tumor cells budded from moderately to poorly differentiated adenocarcinoma in the colon, and then poorly differentiated adenocarcinoma cells diffusely infiltrated the submucosal layer of the stomach and lymphatic vessels of the gastric wall. Although nodular metastases in the transverse mesocolon were observed, a single node at the lesser curvature of the middle of the stomach (the gastric angle) showed metastasis histologically. In conclusion, we reported a case of transverse colon cancer directly invading the stomach with node metastasis at the gastric angle, although metastases of regional lymph nodes of invaded organs are usually rare.

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An 84-year-old man, who had taken aspirin for cerebral infarction, visited our hospital with abdominal pain. His abdomen was hard and board-like. Computed tomography showed a low dense inhomogeneous mass, ascites, and free air in the pelvic cavity. Emergency laparotomy revealed a subserous hematoma, elastic soft mass, and pus in the sigmoid colon, indicating a perforation of the intestine. Partial resection of the sigmoid colon was performed. Macroscopic findings of the resected specimen showed a submucosal tumor 3 cm in diameter with small central erosion. Histological examination disclosed a mucosal defect, a hematoma within the inner muscular layer, and disruption of the wall structure. The perforation was considered to have taken place due to the disruption of the intestinal wall by mechanical compression from the hematoma caused by coagulopathy with aspirin. There have been 15 reports of idiopathic intramural hematoma of the colorectum in Japanese literature, however, this case is the first associated with perforation. Generally, idiopathic hematoma of the intestine is treated conservatively; however, further examination may be required to detect possible perforation.

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A 61-year-old man was referred to our hospital on the suspicion of hepatocellular carcinoma just under the right diaphragm. An enhanced abdominal computed tomography conducted previously at another hospital revealed a 40 mm hypervascular tumor. T1-weighted magnetic resonance imaging (MRI) showed a slightly lower intensity lesion than around the liver parenchyma, which revealed clearly high intensity by T2-weighted MRI. Arteriography showed the tumor to be fed by the right inferior phrenic artery, and was enhanced in both the early and delayed phase, but no enhancement was seen by hepatic arteriography. Despite these imaging examinations, we could not determine the tumor location. The malignant potential of the tumor could not be denied, so we performed the resection of the tumor for diagnosis and cure. Intraoperatively, the tumor was located in the middle of the right diaphragm, which was not invaded and not adherent to the liver or the lung. By pathological examination, the tumor was diagnosed to be paraganglioma. Paraganglioma often occurs below the diaphragm and distributes along the paravertebral and para-aortic axes, especially between the origin of the inferior mesenteric artery and the aortic bifurcation, called the organ of Zuckerkandle. In our investigation, there were only two reports about diaphragmatic paraganglioma, but one was a recurrent case of pheochromocytoma occurring on the diaphragm, so our case was the second report of primary diaphragmatic paraganglioma.

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Inferior lumbar hernia is an uncommon abdominal wall hernia, and an incarcerated case is quite rare. We present a case of incarcerated inferior lumbar hernia, leading to small bowel obstruction. A 74-year-old woman visited our emergency department, complaining of a tender mass on her back and vomiting. Physical examination and abdominal CT revealed hernia of the small intestine through the right inferior lumbar triangle. Emergency operation was performed and a diagnosis of incarcerated inferior lumbar hernia was given. Hernia contents were not strangulated. The hernia orifice was closed by direct approximation and by placing a light-weight polypropylene mesh through the anterior approach.

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A 20-year-old woman consulted the department of gynecology in our hospital because of abdominal pain. US and MRI revealed a cystic tumor in the presacral region and she was refered for surgery. Subsequent CT and PET suggested a congenital cystic tumor which did not invade the colon or rectum. The tumor was removed by the trans-sacral approach and diagnosed as a dermoid cyst, with no malignancy. Masses in the presacral region are rare in adults, and this is only the 14th case of dermoid cyst reported in Japan.

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Myxofibrosarcoma (MFS) is also known as malignant fibrous histiocytoma (MFH) myxoid type. It was classified into groups by the revised WHO classification of malignant fibrous tumors in 2002. In general, MFH has a poor prognosis, but the myxoid type shows a 60-70% 5-year survival rate, and it has a relatively good prognosis. MFS appears more commonly in the limbs, and subcutaneously and intramuscularly in the trunk of elderly people, but rarely occurs in the retroperitoneum. The response rate to chemotherapy and radiation therapy is low, and surgical excision is the only treatment for radical cure. Only 4 cases of primary retroperitoneal MFS have been reported in Japan. The prognosis is unclear because only short-term observation is available, but recurrent cases have been considered difficult to treat. Here we present a case of primary retroperitoneal MFS which recurred and was resected 4 times after the initial surgery, and showed a long-term recurrence-free survival.

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