[Six cases of anaplastic pancreatic carcinoma diagnosed by endoscopic ultrasound-guided fine needle aspiration].

Anaplastic pancreatic carcinoma is a rare form of pancreatic cancer with an extremely poor prognosis. Its diagnosis is often based on surgical specimens and few reports have described the use of endoscopic ultrasound-guided fine needle aspiration (EUS-FNA) for diagnosis. In this study, we examined six patients (mean age, 70.5 years;sex ratio, 1:1) who were diagnosed with anaplastic pancreatic carcinoma using EUS-FNA. The carcinomas were located in the pancreatic head, body, and tail in one, three, and two patients, respectively. The mean tumor diameter was 49.2mm. Five patients opted for best supportive care due to poor performance status and one underwent chemotherapy (GEM+nab-PTX). The median survival was 40.5 (14-98) days. The characteristic imaging findings of anaplastic pancreatic carcinoma, including central necrosis, marginal contrast enhancement, cystic findings, and internal calcification, were frequently observed in the patients. Anaplastic pancreatic carcinoma can also be diagnosed using biopsy tissue;however, a pathologist's consultation is required to differentiate the disease based on imaging findings for an accurate diagnosis.

Severe refractory Clostridium difficile infection with good response to fecal microbiota transplantation: a case report.

A 49-year-old woman diagnosed with pseudomembranous enterocolitis was transferred to our hospital for medical treatment. She responded poorly to treatment with vancomycin hydrochloride and metronidazole, so she underwent fecal microbiota transplantation. Treatment effects were observed the next day, and the diarrhea disappeared within 3 days. Colonoscopy 4 days later revealed the resolution of pseudomembranes, and no recurrences were reported within the first year after discharge.

A Case of Liver Metastasis from Small Intestinal Gastrointestinal Stromal Tumor 25 Years after Surgery including Autopsy Findings.

Gastrointestinal stromal tumor (GIST) is the most common mesenchymal tumor in the digestive tract. Recurrences may occur even after radical resection; however, recurrence later than 10 years after surgery is rare. We report a case of GIST with recurrence of liver metastasis 25 years after surgery. A 56-year-old man complained of sudden epigastric pain and was transferred to the emergency department. He had undergone partial resection of the small intestine for leiomyosarcoma 25 years previously. Abdominal computed tomography showed multiple liver tumors with massive hemorrhage. Ultrasound-guided percutaneous biopsy was performed for the 15-mm hepatic tumor in segment 2. Pathological findings revealed proliferation of spindle-shaped atypical cells, and immunostaining for c-kit and CD34 was both positive; the patient was therefore diagnosed with GIST. He then underwent chemotherapy for 7 years but died of multiple organ failure due to GIST. Autopsy revealed GIST occupying the entire liver with peritoneal dissemination, and minute lung metastases that could not be identified by CT were also detected. This case is interesting in considering the recurrence of GIST, and we will report it together with the literature review.

A case of primary pancreatic schwannoma diagnosed by endoscopic ultrasound-fine needle aspiration.

Pancreatic schwannoma is difficult to diagnose preoperatively. A 79-year-old man was found to have a 9-mm pancreatic mass on abdominal ultrasonography. On EUS, there was a 9-mm, clearly demarcated, round, solid, hypo-echoic mass in the pancreatic body. The differential diagnosis included a pancreatic neuroendocrine tumor, a solid-pseudopapillary neoplasm, and an atypical pancreatic cancer. EUS-FNA was performed with a 22G needle. On pathology examination, spindle-shaped tumor cells were seen proliferating in bundles. On immunostaining, the lesion was negative for c-kit, CD34, and α-SMA but positive for S-100 protein. The MIB-1 index was < 2%. Based on the above findings, the lesion was diagnosed as a benign pancreatic schwannoma. We, therefore, decided to follow the patient with careful observation rather than resecting the lesion surgically. The tumor has not changed significantly after 3 years of follow-up. EUS-FNA is useful for the diagnosis of pancreatic schwannoma. If the tumor can be determined to be benign preoperatively, unnecessary surgery can be avoided. EUS-FNA should be actively implemented for pancreatic tumors that are difficult to diagnose definitively on imaging.

A Case of Ileocecal IgG4-Related Sclerosing Mesenteritis Diagnosed by Endoscopic Ultrasound-Guided Fine Needle Aspiration using Forward-Viewing Linear Echoendoscope.

A 25-year-old woman had undergone removal of a cryptogenic tumor in the left maxillary sinus 1 year prior to presentation. The patient experienced abdominal pain for 4 days with repeated vomiting episodes; therefore, she was transferred to our hospital by an ambulance. Contrast-enhanced computed tomography revealed a 3-cm tumor in the ileocecal region, which caused small bowel obstruction. Contrast imaging of the ileus tube showed extrinsic compression of the ileocecal region. Forward-viewing linear echoendoscope revealed an irregular hypoechoic tumor measuring 3 cm outside the gastrointestinal tract. Using a 25G needle, endoscopic ultrasound-guided fine needle aspiration (EUS-FNA) was performed. Pathological finding was an inflammatory fibrous tissue with diffuse lymphoplasmacytic infiltration, with more than 10 IgG4-positive cells detected in a high-power field. Re-examination of a pathology specimen of the maxillary sinus tumor provided by the previous attending physician revealed that the inflammatory tissue had diffuse lymphoplasmacytic infiltration, which were accompanied by storiform fibrosis and obliterative phlebitis. Immunostaining revealed more than 50 IgG4-positive cells in a high-power field, a finding suggestive of IgG4-related disease. The serum IgG4 level was 21 mg/dl, which was within the normal range. Treatment was initiated with prednisolone at a dose of 50 mg/day, and the dose was later tapered off. CT and MRI performed 2 months later showed complete disappearance of the ileocecal tumor. The final diagnosis was asynchronously occurring IgG4-related maxillary sinusitis and sclerosing mesenteritis.

Serum and histological IgG4-negative type 1 autoimmune pancreatitis.

A 66-year-old man who was on oral medication for type 2 diabetes experienced a rapid decline in glycemic control (increase in glycosylated hemoglobin level from 7.7 to 10.2% over 3 months). Abdominal ultrasonography revealed a 20-mm hypoechoic mass in the pancreatic tail. Serum tumor marker carbohydrate antigen 19-9 and DUPAN2 levels were within the respective normal ranges; serum IgG4 level was also normal at 21.8 mg/dL. Abdominal contrast computed tomography revealed a 26-mm tumor in the pancreatic tail. Magnetic resonance cholangiopancreatography revealed disruption of the main pancreatic duct and dilation of the caudal pancreatic duct. Endoscopic ultrasonography revealed a near-round-shaped hypoechoic mass with interspersed hyperechoic areas. Endoscopic ultrasonography-guided fine needle aspiration was performed using a 22-G needle, but no malignant findings were observed. There were no signs of sialadenitis, retroperitoneal fibrosis, nephropathy, or other conditions associated with IgG4-related diseases. Distal pancreatectomy was performed; a 23-mm white mass was resected from the pancreatic tail. A histopathological examination showed advanced inflammatory cell infiltration mainly involving lymphocytes/plasma cells along with storiform fibrosis and obliterative phlebitis. No more than five IgG4-positive cells were observed per high-power field. These were level 1 pathological findings, and a definitive diagnosis of type 1 autoimmune pancreatitis (AIP) was made according to the International Consensus Diagnostic Criteria. Type 1 AIP associated with normal serum IgG4 levels and absence of IgG4-positive cells on histological examination is a rare clinical entity, which is very difficult to distinguish from pancreatic cancer. Here we report such a case and present a review of the relevant literature.

Optimal timing for precutting in cases with difficult biliary cannulation.

BACKGROUND AND STUDY AIMS: In endoscopic retrograde cholangiopancreatography (ERCP), precutting is widely used when achieving biliary cannulation is difficult. However, no consensus has been reached with regard to the best time to initiate precutting. PATIENTS AND METHODS: We retrospectively examined 63 patients who underwent precutting for naïve papilla with difficulty in biliary cannulation between 2009 and 2016. The outcomes of the early precut group (≤ 20 min from cannulation until initiating precutting) and the late precut group (> 20 min) were compared. RESULTS: Of the 63 patients, 17 (27 %) were in the early precut group and 46 (73 %) were in the late precut group; median time until the initiating precutting was 28 minutes (7 - 50). No significant difference was observed between the two groups in terms of clinical features (age, sex, and indication for ERCP), precutting method, and rate of pancreatic duct stent placement. Significantly higher rates of successful biliary cannulation were observed in the early precut group (16/17; 94 %) than in the late precut group (32/46; 70 %) ( P  < 0.05). In 13 patients in whom precutting was commenced after 40 minutes, the rate of successful biliary cannulation was very low at 53 % (7/13). No significant difference was found between the two groups in terms of incidence of complications (pancreatitis in 5 patients and bleeding in 1 patient). CONCLUSION: In actual clinical practice, precutting is commenced approximately 30 minutes after cannulation; however, to successfully achieve biliary cannulation, precutting is recommended to be performed within 20 minutes. Precutting is effective when little inflammation and swelling of the ampulla of Vater is observed. This study was limited in that it was single-center, retrospective and had a small subject sample.

Life-threatening emphysematous liver abscess associated with poorly controlled diabetes mellitus: a case report.

BACKGROUND: Emphysematous liver abscesses are defined as liver abscesses accompanied by gas formation. The fatality rate is extremely high at 27%, necessitating prompt intensive care. CASE PRESENTATION: The patient was a 69-year-old Japanese man with type 2 diabetes. He visited the emergency outpatient department for fever and general malaise that had been ongoing for 2 weeks. Abdominal computed tomography revealed an abscess 5 cm in diameter accompanied by gas formation in the right hepatic lobe. Markedly impaired glucose tolerance was observed with a blood sugar level of 571 mg/dL and a glycated hemoglobin level of 14.6%. The patient underwent emergency percutaneous abscess drainage, and intensive care was subsequently initiated. Klebsiella pneumoniae was detected in both the abscess cavity and blood cultures. The drain was removed 3 weeks later, and the patient was discharged. CONCLUSION: Emphysematous liver abscesses are often observed in patients with poorly controlled diabetes, and the fatality rate is extremely high. Fever and malaise occasionally mask life-threatening infections in diabetic patients, necessitating careful examination.

A resected case of two branch duct-type intraductal papillary mucinous neoplasms showing different clinical courses after a two-year follow-up.

The patient was a 60-year-old man without any particular complaints, but he underwent abdominal computed tomography (CT) and magnetic resonance cholangiopancreatography (MRCP) due to a fatty liver, which revealed two similar cystic lesions regarded as branch duct-type intraductal papillary mucinous neoplasm (BD-IPMN) in the pancreatic body [BD-IPMN (b), 16 mm in size] and tail [BD-IPMN (t), 13 mm in size] without a "high-risk stigmata" or "worrisome features". He subsequently received follow-up by MRCP every 6 months. Two years later, MRCP showed prominent dilation of the main pancreatic duct (MPD) and mural nodule formation within the dilated MPD adjacent to the BD-IPMN (b). Distal pancreatectomy specimens revealed that the BD-IPMN (b) was lined by low-papillary gastric mucinous epithelium with low-to-intermediate-grade dysplasia and involved the MPD, forming a malignant mural nodule showing pancreatobiliary-type IPMN. In contrast, the BD-IPMN (t) was lined by flat, monolayer columnar gastric mucinous epithelium without atypia, which suggested the possibility of a "simple mucinous cyst". A genetic analysis showed KRAS mutation only in BD-IPMN (b). Differences in the histological and genetic findings between two similar BD-IPMNs in the present case may suggest what kinds of examinations should be performed in patients with BD-IPMNs without any worrisome features.

Perforation of the Papilla of Vater in Wire-Guided Cannulation.

Background. WGC in ERCP is considered a safe technique, although rare complications can occur. One unique complication of WGC is the perforation of the papilla of Vater by the guidewire. Subjects and Methods. Of 2032 patients who underwent ERCP at our department between January 2010 and December 2014, we selected 208 patients who underwent WGC for naïve papilla as subjects. A detailed examination of patients in whom a perforation occurred was conducted, and risk factors for perforations were investigated. Results. The perforation was observed in 7 of 208 patients (3.4%). All patients recovered with conservative treatment without the need for surgery. The perforation rate was significantly higher in the patients with juxtapapillary duodenal diverticula than those without diverticula (12.5% versus 0.6%, p < 0.001). Cannulation of the bile duct was ultimately achieved in 5 of 7 patients; PSP was performed for 4 of these patients. Conclusion. Caution must be exercised when dealing with patients who have a juxtapapillary duodenal diverticula because they are at higher risk of perforations. Because these are small perforations made by a wire, most of them heal with conservative treatment. However, perforations can make cannulation difficult, and PSP may be useful for deep cannulation.

A case of concurrent pancreatic intraepithelial neoplasia and type 1 autoimmune pancreatitis with marked pancreatic duct dilatation.

The case patient was a previously healthy 82-year-old male. Abdominal ultrasound during a medical check-up revealed a dilatation of the main pancreatic duct, and the patient was referred to our hospital for closer examination. Contrast-enhanced computed tomography (CT) revealed a low-density mass of 20 mm in the pancreatic head-body transitional area. Magnetic resonance cholangiopancreatography (MRCP) revealed marked dilatation of the main pancreatic duct and branches in the body-tail. On endoscopic ultrasonography (EUS), a hypoechoic mass with irregular shape was detected, which was consistent with the area of pancreatic duct stenosis. Endoscopic ultrasound-guided fine needle aspiration (EUS-FNA) and pancreatic juice cytology were performed; however, there were no malignant findings. Serum IgG4 levels had increased to 299 mg/dL. Cancer of the pancreatic head was suspected and a pancreaticoduodenectomy was thus performed. Macroscopic findings included a white mass with indistinct border in the constricted part of the pancreatic duct and mottled fatty replacement of the pancreatic head. Pathologically, a large amount of IgG4-positive plasma cells was found in the white mass, with storiform fibrosis and obstructive phlebitis, which led to the diagnosis of type 1 autoimmune pancreatitis (AIP). Furthermore, scattered low-high grade pancreatic intraepithelial neoplasia lesions were observed throughout the pancreatic head, separately from the AIP lesion. This is an interesting case that suggests an association between AIP and pancreatic cancer. We report the case with a review of relevant literature.

Clinical features of gallstone impaction at the ampulla of Vater and the effectiveness of endoscopic biliary drainage without papillotomy.

BACKGROUND AND STUDY AIMS: Gallstone impaction at the ampulla of Vater is a critical condition, and the standard treatment is endoscopic papillotomy. However, the clinical features remain largely unclear, and some patients are reluctant to undergo papillotomy because of a bleeding tendency. The aim of this study was to clarify the clinical features of gallstone impaction at the ampulla of Vater and to examine the effectiveness of endoscopic biliary drainage without papillotomy. PATIENTS AND METHODS: We retrospectively examined 30 patients who had undergone endoscopic treatment for gallstone impaction at the ampulla of Vater between 2010 and 2015. RESULTS: According to the severity classification for acute cholangitis in the Tokyo Guidelines (TG13), the condition was mild in 8 patients, moderate in 14, and severe in only 8 (27 %), despite the stone impaction at the ampulla of Vater. Hyperamylasemia was observed in 18 patients (60 %); computed tomography (CT) revealed clear pancreatitis in 5 cases (17 %). Patients were classified into Group A (13 patients who received biliary drainage with papillotomy) and Group B (17 patients receiving biliary drainage without papillotomy). All patients in Group B had 1 or more types of hemorrhage risk. There were no differences between the 2 groups in the success rate of biliary drainage, the rate of elevated serum amylase the following day, or procedure-related complications. Serum amylase levels decreased the following day in all patients, even in Group B (including the 10 patients with hyperamylasemia and the 3 patients with clear pancreatitis on CT). CONCLUSIONS: Even with stone impaction at the ampulla of Vater, typical clinical features of cholangitis and pancreatitis are relatively rare. Biliary drainage without papillotomy is acceptable for gallstone impaction at the ampulla of Vater.

Prolapse into the bile duct and expansive growth is characteristic behavior of mucinous cystic neoplasm of the liver: report of two cases and review of the literature.

Mucinous cystic neoplasm of the liver (MCN-L) is a very rare tumor whose detailed behavior is still unknown. We describe two cases of MCN-L that exhibited extremely interesting growth patterns, and discuss the characteristics of MCN-Ls. Both cases exhibited MCN-L that originated from the left hepatic lobe (Segment 4) and then prolapsed into the left hepatic duct and common bile duct, resulting in obstructive jaundice due to expansive growth. Endoscopic retrograde cholangiopancreatographies showed the characteristic oval-shaped filling defects in the bile ducts. Endoscopic ultrasound and intraductal ultrasound were useful for differentiating the tumors from stones, since multiple septal formations were observed inside the tumors. A literature search revealed that, over the past 10 years, 15 cases of MCN-L (biliary cystadenomas with ovarian-like stroma) that showed expansive growth in the bile duct had been reported. Prolapse into the bile duct and expansive growth appear to be characteristic behavior of MCN-L. In the future, additional data on more cases needs to be collected to further elucidate MCN-L pathophysiology.

Liver abscessation and multiple septic pulmonary emboli associated with Lemierre's syndrome: a case report.

BACKGROUND: In Lemierre's syndrome, patients first exhibit pharyngitis and peritonsillar abscessation, followed by the development of anaerobic bacterial (usually Fusobacterium necrophorum) septicemia and metastatic infections throughout the body. However, these infections rarely affect the liver. We describe a case of Lemierre's syndrome, in which the first disease manifestation was liver abscess, for drawing attention of emergency physicians to this rare but fatal disease. CASE PRESENTATION: A 28-year-old Asian ethnicity Filipino male, who was previously healthy, entered the emergency department presenting with fever and pharyngeal pain that had persisted for 5 days. Contrast-enhanced abdominal computed tomography revealed a 3-cm area of low density in segment 6 of the liver, consistent with an abscess. Chest computed tomography also revealed that multiple nodes in both lungs were enlarged, and septic emboli were suspected. The patient was hospitalized and antibiotic treatment was initiated. On hospital day 6, blood culture results confirmed Fusobacterium necrophorum septicemia. The patient was diagnosed with Lemierre's syndrome, as pharyngitis developed into bacteremia associated with hepatic and pulmonary lesions. The patient's condition improved with antibiotics and he was discharged following three weeks of treatment in the hospital. CONCLUSION: With the widespread use of antibiotics, Lemierre's syndrome is rarely encountered anymore, but it can be fatal if not properly diagnosed. It is a crucial differential diagnosis in young patients exhibiting septicemia or multiple metastatic infection of unknown origin.

Successful conservative treatment of emphysematous gastritis.

Emphysematous gastritis is an extremely rare disease with an unfavorable prognosis. To date, very few studies have been conducted regarding the intragastric recovery process based on endoscopic findings. We herein report a case of emphysematous gastritis that improved with long-term (five months) conservative treatment in which we were able to observe the intragastric recovery process endoscopically. In cases in which emphysematous gastritis is suspected, it is important to provide prompt diagnostic imaging (including CT) and early appropriate treatment in order to improve the prognosis.

Small intestinal obstruction caused by anisakiasis.

Small intestinal anisakiasis is a rare disease that is very difficult to diagnose, and its initial diagnosis is often surgical. However, it is typically a benign disease that resolves with conservative treatment, and unnecessary surgery can be avoided if it is appropriately diagnosed. This case report is an example of small intestinal obstruction caused by anisakiasis that resolved with conservative treatment. A 63-year-old man admitted to our department with acute abdominal pain. A history of raw fish (sushi) ingestion was recorded. Abdominal CT demonstrated small intestinal dilatation with wall thickening and contrast enhancement. Ascitic fluid was found on the liver surface and in the Douglas pouch. His IgE (RIST) was elevated, and he tested positive for the anti-Anisakis antibodies IgG and IgA. Small intestinal obstruction by anisakiasis was highly suspected and conservative treatment was performed, ileus tube, fasting, and fluid replacement. Symptoms quickly resolved, and he was discharged on the seventh day of admission. Small intestinal anisakiasis is a relatively uncommon disease, the diagnosis of which may be difficult. Because it is a self-limiting disease that usually resolves in 1-2 weeks, a conservative approach is advisable to avoid unnecessary surgery.