A Case of Atypical Familial Mediterranean Fever With Pseudopolyposis-Like Mucosal Changes in the Ileum.

A 15-year-old male patient presented with recurrent fever. Three months prior, he experienced repeated fevers of 38°C, headaches, and malaise for three days. He experienced repeated fevers over 38°C for >72 hours two weeks prior to the current visit. A computed tomography scan showed enlarged lymph nodes around the ileum, suggesting familial Mediterranean fever (FMF) or inflammatory bowel disease. Endoscopic examination revealed a deformed Bauhin valve and inflammatory changes in the ileum, making inflammatory bowel disease unlikely. Thus, FMF was suspected, and after a thorough explanation, the patient was treated with colchicine, and his symptoms improved. Genetic testing revealed a mutation in the MEFV gene P369S-R408Q, and atypical FMF was diagnosed.

Takayasu's arteritis associated with Crohn's disease treated with infliximab.

A 23-year-old woman presented with fever, diarrhea, bloody stools, and arthralgia that did not improve despite previous treatments and was diagnosed with Crohn's disease. Remission was achieved after the introduction of infliximab, nutritional therapy, and 5-aminosalicylic acid treatment. However, the patient's blood sedimentation rate remained elevated without symptom recurrence, except for abdominal pain in the following year. Aortic wall thickening in the thoracic descending aorta was also observed on computed tomography. Accumulation in the thoracic descending aorta and abdominal aorta was confirmed using positron emission tomography-computed tomography. The patient was diagnosed with Takayasu's arteritis. The patient's abdominal symptoms resolved, and her blood sedimentation rate normalized after steroid administration.

A Case of Pancreaticobiliary Maljunction with Repeated Pancreatitis Due to Protein Plugs in a Short Period.

A 70-year-old woman was admitted to the hospital 1 month prior to presentation with acute pancreatitis due to pancreaticobiliary maljunction. After discharge, she was referred for elevated hepatobiliary enzyme levels. She was diagnosed with an acute pancreatitis flare-up. Computed tomography revealed dilation of the common duct compared to the previous admission. Considering the protein plug formation as the cause, endoscopic retrograde cholangiopancreatography (ERCP) was performed after improvement. ERCP revealed a defect in the duct, suspected to be caused by protein plugs, which were removed using a balloon after endoscopic papillary balloon dilatation. An analysis revealed that this component was a protein. No recurrence of pancreatitis was observed after the treatment.

Gastrocolic fistula caused by transverse colon cancer: a case report.

BACKGROUND: A gastrocolic fistula is an unusual communication between the colon and the stomach. Although colon cancer is the most common malignant cause of gastrocolic fistula in the Western world, the incidence of gastrocolic fistula due to colon cancer is 0.3% in operated cases. CASE PRESENTATION: A 68-year-old man presented with anorexia, general malaise, weight loss, and vomiting of fecal matter. Investigations revealed that the patient had a large nonmetastatic splenic flexure tumor that was diagnosed as colon cancer and had invaded the stomach and pancreas. An upper gastrointestinal series confirmed a gastrocolic fistula. Left hemicolectomy, distal gastrectomy, distal pancreatectomy, and splenectomy were performed. Histology revealed transverse colon cancer, which was UICC stage (8th edition) pT4bN1bcM0 pStage IIIC. Adjuvant chemotherapy was not performed. There was no recurrence or metastasis one year after surgery. We reviewed 17 cases including our case of a gastrocolic fistula caused by colon cancer. Neoadjuvant chemotherapy was not given to any of the patients, and en bloc resections were conducted in all cases. Adjuvant chemotherapy was given to almost all of the patients. There was no recurrence or metastasis. CONCLUSIONS: For gastrocolic fistula caused by advanced colon cancer, secure en bloc surgical resection was the initial treatment in all 17 reported cases including the present case, and adjuvant chemotherapy may contribute to a better prognosis.

Mesalazine-Induced Diffuse Lung Injury in a Patient Without Respiratory Signs and Symptoms.

Mesalazine is a drug used to treat ulcerative colitis and Crohn's disease, and is known to rarely cause lung injury. We show herein a unique case who developed this drug-induced injury. A 17-year-old boy presented with fever and anorexia after administration of mesalazine. Computed tomography showed extensive ground-glass opacities with peripheral distribution in both lungs. He had general weakness, but had no respiratory symptoms such as cough and dyspnea. With prednisolone, which is primarily aimed at controlling ulcerative colitis, the extensive opacity in both lungs were improved. All patients with this drug-induced lung injury reported to date have had respiratory symptoms, but this patient had no subjective respiratory symptoms and had no abnormalities in respiratory rate and oxyhaemoglobin saturation. Although very rare, we do believe that this clinical course will provide some suggestive information on treatment for patients with similar course in the future.

Aspiration pneumonia associated with achalasia.

In this case, a chest computed tomography scan of a young patient with pneumonia revealed esophageal obstruction, which led to the diagnosis of aspiration pneumonia due to achalasia. This report highlights achalasia and other gastrointestinal obstructions as one of the causes of aspiration pneumonia.

Serotonin-positive neuroendocrine neoplasm with pancreatic ductal dilation in which cytological immunostaining contributed to the diagnosis.

A 52-year-old man was transported via an ambulance because of syncope and the passage of tarry stools, which had been noted the previous day. He was diagnosed with upper gastrointestinal bleeding from a gastric ulcer and underwent endoscopic hemostasis. Prior to endoscopy, abdominal computerized tomography performed for gastrointestinal bleeding revealed pancreatic duct dilation. After discharge, abdominal imaging revealed a strongly enhancing tumor (5 mm) with caudal pancreatic duct dilation. Endoscopic retrograde pancreatography revealed that the main pancreatic duct was interrupted at the body. Pancreatic juice cytology was class III, and additional immunostaining were positive for chromogranin A, synaptophysin, and serotonin, suggesting a pancreatic neuroendocrine neoplasm (NEN). Distal pancreatectomy was performed and a yellowish-white solid lesion was found in the pancreatic duct. Pathological examination revealed narrowing of the pancreatic duct, extensive stromal fibrosis, and proliferation of tumor cells with small round nuclei and eosinophilic vesicles. Furthermore, the immunostaining findings of the resected specimen corresponded with those of the cytology. A diagnosis of NEN G1 (WHO classification) with Ki-67 index < 1% was made. Imaging of the pancreatic duct tend to be normal or show no involvement of the duct in pancreatic neuroendocrine neoplasms; however, there have been a few reports of stenosis due to fibrosis around the pancreatic duct. Serotonin positivity was previously documented to be significantly higher in patients with fibrosis. In lesions with pancreatic ductal stenosis, the addition of immunostaining to pancreatic juice cytology was thought to be useful in differentiating pancreatic cancer from pNEN.

Spontaneous retroperitoneal bleeding secondary to celiac artery compression syndrome.

Clinicians should consider celiac artery compression syndrome as the cause of ruptured visceral aneurysm and dissection and ask patients for unexplained chronic abdominal symptoms. Endovascular embolization with metallic coil placement is the first-line treatment, and surgery can be avoided in some cases.

Small bowel obstruction of barium for gastric cancer screening.

We report a case of small bowel obstruction without perforation and peritonitis caused by inspissated barium sulfate for gastric cancer screening and surgically treated 26 hours after the onset of abdominal pain.

Pancreatic panniculitis in a patient with pancreatic adenosquamous carcinoma.

Pancreatic panniculitis is a rare complication of pancreatic diseases. We aimed to evaluate a case of pancreatic panniculitis. A 58-year-old woman was referred to our hospital with complaints of painful cutaneous nodules on her limbs. Various diagnostic tests confirmed pancreatic panniculitis and pancreatic adenosquamous carcinoma. We diagnosed pancreatic panniculitis by a skin nodule biopsy that revealed fine basophilic material within anucleate cells and neutrophil infiltration. Abdominal imaging detected a tumor with necrosis on the pancreas and endoscopic ultrasound-guided fine-needle aspiration revealed it as an adenocarcinoma. The patient underwent pancreatoduodenectomy after neoadjuvant chemotherapy. The tumor was composed of differentiated adenocarcinoma and squamous cell carcinoma and diagnosed as adenosquamous carcinoma. This is the first report of pancreatic panniculitis in a patient with adenosquamous cell carcinoma of the pancreas.

Ceftriaxone pseudolithiasis detected by computed tomography and followed up until resolution.

Introduction: Ceftriaxone is a third-generation cephalosporin antibiotic that has been widely used to treat various infectious diseases. We report a case of ceftriaxone pseudolithiasis that was detected by computed tomography (CT) and followed up until it was resolved. Case: A 76-year-old woman with diabetes mellitus and renal impairment, but no history of gallstones, was diagnosed with septic shock due to renal and lung abscesses and treated with ceftriaxone. On day 22 after admission, abdominal CT revealed a gallstone, which increased in size up to day 50. Ceftriaxone was stopped on day 50, and the gallstone resolved completely after 10 weeks. Conclusion: Ceftriaxone pseudolithiasis should be cautiously considered, specifically in a patient with renal impairment and a prolonged treatment period.

Balloon-Occluded Trans-Arterial Chemo-Embolization Technique with Repeated Alternate Infusion of Cisplatin Solution and Sparse Gelatin Slurry (RAIB-TACE) for Large Hepatocellular Carcinoma Nodules More than 7 cm in Diameter.

OBJECTIVE: It is sometimes difficult to obtain complete/partial response of large hepatocellular carcinoma (HCC) nodules by trans-arterial chemoembolization/embolization (TACE/TAE). The aim is retrospective investigation of tumor response of large HCC nodules (>7 cm) treated by the new TACE technique, repeated alternate infusion of cisplatin solution, and sparse gelatin slurry under balloon occlusion (RAIB-TACE). Materials and Methods. A microballoon catheter was placed at a proximal portion of the hepatic artery (subsegmental to the lobar level hepatic artery), and alternate infusion of cisplatin solution and sparse gelatin slurry were repeated under balloon occlusion until stasis of gelatin slurry beyond the catheter was seen. RAIB-TACE of multiple proximal hepatic and extrahepatic collateral arteries were performed to treat hemi-lobe or more of the liver while avoiding infusion into gastric and cystic arteries for 19 large nodules (>7 cm) in 19 patients without portal venous invasion. All patients underwent dynamic CT/MRI 1-3 months after RAIB-TACE, and tumor response of each large nodule was evaluated based on modified RECIST criteria. RESULTS: CR, PR, SD, and PD were achieved in 11, 8, 0, and 0 nodules, respectively. CR and PR were considered as success, and the ratio of success was 100%. Major complications were abscess formation in the necrotic nodule (n = 1) which was treated by drainage tube placement, and subsegmental level liver infarction (n = 1) which was treated by drainage tube placement, and subsegmental level liver infarction (. CONCLUSION: New TACE technique, RAIB-TACE, was useful to achieve successful response of large HCC nodules.

Preoperative histological subtype classification of intraductal papillary mucinous neoplasms (IPMN) by pancreatic juice cytology with MUC stain.

OBJECTIVE: To prospectively evaluate the diagnostic value of preoperative histological subtyping of intraductal papillary mucinous neoplasms (IPMNs) by pancreatic juice cytology (PJC) with mucin (MUC) stain. BACKGROUND: IPMNs are classified into four subtypes based on their histomorphology and mucin phenotype, and varied degrees of malignant nature and prognosis among these subtypes have been shown. METHODS: The subjects were 36 patients with surgically confirmed IPMNs, who underwent PJC preoperatively by endoscopic retrograde cholangiopancreatography. Histological subtyping of cytological samples with or without MUC stain (MUC1, MUC2, and MUC5AC) was compared with that of resected specimens. RESULTS: Histologically, low-grade dysplasia was found in 4 patients, intermediate in 10, high grade in 11, and invasive carcinoma in 11. Gastric, intestinal, pancreatobiliary, and oncocytic subtypes corresponded to 16, 14, 5, and 1 patient, respectively. The rate of high-grade dysplasia (HGD) and/or invasive IPMNs was 25% for gastric subtype, 85.7% for intestinal subtype, and 100% for both pancreatobiliary and oncocytic subtypes, showing a significant correlation between histological subtype and rate of HGD and/or invasive IPMN (P < 0.01 for gastric vs nongastric).Histological subtype was successfully diagnosed by PJC in 42% (15/36) without MUC stain, and the rate was significantly improved to 89% (32/36) with MUC stain (P < 0.01). The sensitivity, specificity, and overall accuracy of PJC with MUC stain were 86%, 100%, and 94% for intestinal subtype, respectively. When cytological grade was combined with MUC stain, the diagnosis of HGD/invasive IPMN showed 77.2% sensitivity, 85.7% specificity, and 80.5% accuracy. CONCLUSIONS: Preoperative PJC with MUC stain proved to be highly reliable for identifying the histological subtype of IPMN and may provide useful information for deciding surgical indication.

Extensive endothelial replacement by tumor cells in the portal system: an autopsy case of intrahepatic cholangiocarcinoma.

An autopsy case of intrahepatic cholangiocarcinoma (ICC) with a peculiar form of extensive portal invasion is reported here. A 76-year-old woman presented with anorexia and abdominal discomfort. A high level of serum carbohydrate antigen 19-9 and endoscopically detected esophageal varices were found. Obvious mass lesion was not identified on CT scan and no portal blood flow was found. The patient died 6 months after admission. At autopsy multiple irregular shaped tumors in the liver were found. The size of the largest one was 3 x 2 cm. These tumors were well-differentiated adenocarcinomas with partial mucinous carcinoma morphology. Surprisingly, portal veins contained mucinous fluid and the inner surface was lined with a single layer of tumor cells but not endothelial cells. Invasion of carcinoma into the tissue outside the blood vessels was hardly observed in organs other than the liver. This form of extensive invasion of the tumor, termed intimal carcinoma spreading, caused complete obstruction of the portal system. To our knowledge there has been no report on this type of portal invasion of ICC.

[Examination of the shapes of the end of the common bile ducts after endoscopic sphincterotomy (EST)].

At the treatment of bile duct stones using EST, we sometimes experienced the difficult cases, even if the stones were small. For these cases, we tried endoscopic papillary balloon dilatation, and as a result knoches were made. And after the disappearance of the knoch, we could easily remove the stone. So we prospectively examined how often and at what cases, the knoches were made immediately after EST. As a result, knoches were present for 25% (13/52) of patients even after EST. Narrow distal segments of knoch-present patients were longer than that of knoch-absent patients, significantly. As a result, some function of sphincter was shown to be remained for a quarter of patients even after EST, and for these cases, the treatment of the stones may be difficult.

Spindle and giant cell type undifferentiated carcinoma arising in the common bile duct: a case report.

We report on a 61-year-old Japanese male with a pedunculated tumor in the common bile duct. The tumor consisted of two types of neoplastic cells. The majority showed atypical spindle- and giant-shaped features and proliferated densely in an inflammatory stroma, revealing a sarcomatous pattern. They expressed vimentin, KL-1, and CAM5.2. The remaining minority showed glandular and tubular features, occupied only less than 5%, located only in the tumor surface, and expressed wide spectrum keratin, KL-1, CAM5.2, epithelial membrane antigen, AE1/AE3, and carcinoembryonic antigen. CD68-positive osteoclast-like giant cells were also observed. Therefore, the patient was diagnosed as having an undifferentiated carcinoma, spindle and giant cell type.