Sarcoidosis with Pancreatic Mass, Endobronchial Nodules, and Miliary Opacities in the Lung.

Sarcoidosis affects multiple organs and rarely has unusual manifestations. A 78-year-old woman was referred to our hospital for coughing symptoms. A chest computed tomography (CT) scan revealed bilateral diffuse miliary patterns and right pleural effusion. Bronchoscopy showed multiple nodules in the carina and the bronchus intermedius. A CT scan of her abdomen revealed hypovascular lesions involving the pancreatic head and body. A transbronchial lung biopsy, bronchial mucosal biopsy, and endoscopic ultrasound-guided fine-needle aspiration of the pancreatic mass demonstrated non-caseating granulomas. We diagnosed the patient with sarcoidosis. She received no treatment for sarcoidosis and has been followed up for one year, during which no pulmonary disease progression had been observed and the pancreatic masses partially regressed.

E-cadherin-negative acinar cell carcinoma of the pancreas: report of a case showing a solid pseudopapillary growth pattern.

E-cadherin expression patterns in acinar cell carcinomas (ACCs) of the pancreas have not been well documented. Herein, we present a hitherto undescribed case of E-cadherin-negative ACC with a solid pseudopapillary growth pattern in a 65-year-old man. We used an antibody against the extracellular domain of E-cadherin. As a further unusual status in ACC, faint ß-catenin expression was observed in the cytoplasm of carcinoma cells. Morphological distinction from a solid pseudopapillary neoplasm (SPN) of the pancreas might be problematic in such a case, because of their similarities concerned with the growth pattern and E-cadherin negativity. Without nuclear accumulation of ß-catenin, a diagnosis of SPN was almost excluded. Immunoreactivity for trypsin and BCL10 made an accurate diagnosis of ACC to this case. The tumor recurred 10 months post-surgery as rapidly enlarging masses in the liver, presumably indicating the aggressiveness of the E-cadherin-negative phenotype among ACCs.

Myxoid epithelioid gastrointestinal stromal tumor harboring an unreported PDGFRA mutation: report of a case and review of the literature.

Activating mutations of platelet-derived growth factor receptor α (PDGFRA) are detected in a significant proportion of gastrointestinal stromal tumors (GISTs), in addition to the more frequent mutation in c-kit. GISTs with PDGFRA mutations have been found to have several characteristic morphological features, sometimes allowing to discriminate them from GISTs with c-kit mutations. Among these, epithelioid morphology in tumor cells and tumor-infiltrating mast cells are powerful predictors of PDGFRA mutations. Although myxoid stroma by itself is not so much a reliable predictor of PDGFRA mutation, myxoid stroma in conjunction with epithelioid morphology in tumor cells is a powerful predictor of mutations in this gene. GISTs showing either weak or negative immunoreactivity for c-kit and epithelioid cells with myxoid stroma are called myxoid epithelioid GISTs, which typically show PDGFRA mutation. Herein, we presented a case of a 59-year-old woman with myxoid epithelioid GIST of the stomach. A unique finding in this case was eosinophil infiltration, probably more numerous than mast cells; mast cell infiltration is known to be usually found in myxoid epithelioid GIST. The existence of a similar mechanism in eosinophil and mast cell recruitment via tumor-producing stem cell factor is speculated. Mutational analyses revealed a PDGFRA exon 18 mutation: D842_H845del, D846N. Combined deletion and substitution mutation has been reported in rare instances, but to the best of our knowledge, D846N has not been documented.

Intraductal papillary neoplasm of the bile duct, gastric type, arising in the intrapancreatic common bile duct could progress to colloid carcinoma: report of a case.

Intraductal papillary neoplasm of the bile duct (IPNB) exists in a pathway of multistep-carcinogenesis toward cholangiocarcinoma. Four subtypes are observed in IPNB, pancreatobiliary type, intestinal type, gastric type, and oncocytic type, similarly to the corresponding disease in the pancreas, intraductal papillary mucinous neoplasm (IPMN). IPNB can present with or without macroscopically visible mucin secretion. IPNB usually progresses to tubular adenocarcinoma. However, there are a limited number of well-described cases of gastric-type IPNB progressing not to tubular adenocarcinoma but to colloid carcinoma. Herein, we present a case of an 82-year-old female patient with gastric-type IPNB in the intrapancreatic common bile duct without macroscopically visible mucin secretion, which progressed to colloid carcinoma. As IPNB, especially without visible mucin secretion, is considered to be a heterogeneous group of diseases, such an unexpected association could occur.

Xanthogranulomatous gastritis associated with actinomycosis: report of a case presenting as a large submucosal mass.

Xanthogranulomatous gastritis (XGG) is a rarely encountered condition, and its causative mechanism is still unclear. Given that some types of xanthogranulomatous inflammation (XGI) are associated with pathogens, infection should be considered as a possible cause of XGG. Herein, we report a case of an 86-year-old woman presenting with a large, bleeding lesion resembling a submucosal tumor. Distal gastrectomy was performed, and the surgically resected specimen revealed a mass measuring 6 × 4.5 × 3 cm and appearing yellowish on the cut surface. Histopathological examination revealed a few Actinomyces "sulfur granules" and cellular composition characteristic of XGI, supporting a diagnosis of XGG associated with actinomycosis. Gastric actinomycosis is a rare condition and has not previously been reported in association with XGG, although rare cases of XGI associated with actinomycosis have been documented in other organs.

Successful transarterial embolization for recurrent pseudoaneurysm of the right hepatic artery with acute cholecystitis.

Pseudoaneurysm of the right hepatic artery is an extremely rare complication of acute cholecystitis. We report a patient with a right hepatic artery pseudoaneurysm associated with acute cholecystitis who was treated successfully by transarterial embolization. We also review the literature on right hepatic artery pseudoaneurysm secondary to acute cholecystitis. A 50-year-old male visited Fujieda General Municipal Hospital with an episode of sudden headache. He was diagnosed with a subarachnoid hemorrhage and treated successfully by microcoil embolization on hospital day 4. On hospital day 54, he developed fever and right upper quadrant tenderness. Abdominal ultrasonography revealed acute cholecystitis, while color Doppler imaging showed a low-echogenic mass with a pulsatile wave pattern inside the gallbladder. Contrast-enhanced computed tomography (CE-CT) demonstrated a pseudoaneurysm in the gallbladder, and angiography disclosed a right hepatic artery pseudoaneurysm. Selective transarterial embolization (TAE) was then performed using a steel coil. Abdominal pain and fever continued after TAE, with CE-CT showing re-bleeding from the previous pseudoaneurysm. Selective angiography identified extravasation at the same place as the previous pseudoaneurysm from the posterior superior pancreaticoduodenal artery and the inferior pancreaticoduodenal artery via the epicholedochal arterial plexus. TAE was performed resulting in successful occlusion of the pseudoaneurysm.

Looking for endoscopic features of cytomegalovirus colitis: a study of 187 patients with active ulcerative colitis, positive and negative for cytomegalovirus.

BACKGROUND: Cytomegalovirus (CMV) is frequently detected in ulcerative colitis (UC) lesions of steroid-refractory patients. This has led to the suspicion that CMV might cause colitis and steroid refractoriness. METHODS: During 2003 and 2011, 187 consecutive patients were divided into group I (n = 105), corticosteroid-free and thiopurine-free in the past 6 months, and group II (n = 82), all corticosteroid refractory. The combination of serum CMV immunoglobulin (Ig)M, CMV IgG, CMV antigenemia (Ag), and real-time polymerase chain reaction assays were performed to identify CMV(+) patients. RESULTS: In group I, 79 patients were CMV IgG(+) and 26 patients were CMV IgG(-) and CMV IgM(-). In group II, 61 patients were CMV IgG(+), 1 CMV IgM(+), and 20 CMV IgG(-) and CMV IgM(-). All CMV IgG(+) patients were screened for CMV Ag. In group I, 6 of the 79 CMV IgG(+) patients were CMV Ag(+). In group II, 27 patients were CMV Ag(+). Colonoscopy was performed in all patients before screening for CMV. Similar colonoscopic features including punched out ulcers, geographic ulcers, and irregular ulcers were found in both CMV(+) and CMV(-) patients, without any striking difference between the 2 groups. CONCLUSIONS: CMV reactivation might be encouraged by immunosuppressive drugs, like corticosteroids, immunomodulators, and therefore, patients with UC are at a high risk of CMV reactivation, potentially exacerbating UC. However, this study of 187 patients, CMV(+) and CMV(-), could not find colonoscopic features unique to CMV, except that CMV might be one factor for steroid refractoriness, and UC exacerbation.

[Esophageal involvement in a case of eosinophilic gastroenteritis].

A 43-year-old woman presented with severe diarrhea and vomiting in addition to abdominal distension and dysphagia which had persisted for a month. Endoscopic biopsies revealed a significant eosinophilic infiltration in the mucosal layer of the esophagus, duodenum and ileum, yielding a diagnosis of eosinophilic gastroenteritis (EG) with esophageal involvement. The endoscopic examination of esophageal lesion in this case showed linear fissures and concentric rings, both of which are characteristic findings observed in eosinophilic esophagitis (EE). This suggests that these characteristic endoscopic findings in EE can also be applied to the results of endoscopic examinations for esophageal involvement in EG.

Long-term prognosis of patients after endoscopic sphincterotomy for choledocholithiasis.

INTRODUCTION: The aims of the present study were to clarify the long-term prognosis of endoscopic sphincterotomy (EST) for choledocholithiasis and to evaluate the need for cholecystectomy after EST. METHODS: Between 1993 and 2007, 262 patients who underwent successful EST for choledocholithiasis were followed up for more than 6 months). Eighteen patients had previously undergone cholecystectomy (Group A), 129 had a calculous gallbladder (GB) and underwent cholecystectomy after EST (Group B), 46 had a calculous GB in situ (Group C), and 69 had an acalculous GB in situ (Group D). Late complications, including recurrence of choledocholithiasis, acute cholecystitis and biliary carcinoma, were evaluated. RESULTS: Of the 262 patients, late complications occurred in 34 patients (13.0%) and recurrence of choledocholithiasis occurred in 29 patients (11.1%). The rate of late complications was higher in Group C (23.9%) than in Group B (7.8%) (P < 0.001). The rate of recurrent choledocholithiasis was higher in Group C (17.4%) than in Group B (7.8%) (P < 0.05). Univariate analysis indicated that pneumobilia after EST was associated with the recurrence of choledocholithiasis (P < 0.001). Acute cholecystitis occurred in eight (7.0%) of 115 patients with intact GB. A gallbladder carcinoma was found after EST. Late complications were not serious and endoscopically or surgically manageable. CONCLUSIONS: EST for choledocholithiasis is safe and effective. Cholecystectomy after EST is recommended in patients with calculous GB, but is not necessary in patients with acalculous GB. Pneumobilia was associated with the recurrence of choledocholithiasis.

[Case of pancreatic mucosa-associated lymphoid tissue (MALT) lymphoma].

A 77-year-old woman underwent abdominal ultrasonic diagnosis in a screening test for diabetes mellitus. A 65 x 45 mm tumor with low echo level was revealed and located from the uncinate process of the pancreas to the body. Contrast-enhanced computed tomography demonstrated the pancreas had a low density area in the arterial phase and a comparable area in the equilibrium phase, compared with the parenchyma of the normal pancreas. Gallium-scintigraphy showed strong accumulation, consistent with the tumor. Mucosa-associated lymphoid tissue (MALT) lymphoma was diagnosed by endosonography-guided fine-needle aspiration biopsy (EUS-FNAB). Complete remission was achieved after radiation therapy.

[Retroperitoneal fibrosis and Castleman disease in two patients with high IgG4 levels].

With the cumulative increase in the number of autoimmune pancreatitis cases, the disease is now widely accepted as a symptom of IgG4-related systemic disease. We recently experienced two cases of retroperitoneal fibrosis and Castleman disease presenting high IgG4 levels without evident pancreatic lesions. Both patients were successfully treated with steroid therapy. It is necessary to acknowledge that retroperitoneal fibrosis and Castleman disease, with or without pancreatic lesions, may have aspects of IgG4-related systemic disease and that the measurement of serum IgG4 and tissue immunostaining for IgG4 should be considered for diagnosing and treating the conditions.

[A case of colonic obstruction due to post-operative stenosis of the colon and multiple enteroliths].

An 81-year-old man who had under gone two abdominal surgeries and temporary colostomy 30 years previously was admitted due to lower abdominal pain and vomiting. An abdominal X-ray film and abdominal CT scan showed intestinal distension and multiple calcareous deposits in the colon. Gastrografin enema examination revealed smooth stenosis at the sigmoid colon and many additional defects. Endoscopy could not be performed due to the stenosis. He did not agree to surgery. Seven months later, he was admitted again, due to colonic obstruction. Surgery was performed which revealed colonic obstruction as the source of post-operative stenosis of the sigmoid colon and multiple enteroliths. The stones consisted of a core and a hull and contained ammonium magnesium phosphate.

[A case of Castlemans disease (plasma cell type) in which autoimmune pancreatitis developed 6 years later].

In 1998 a 74-year-old man, he had a medical checkup and mediastinal and hilar lymph node hyperplasia were discovered. Since the lymph nodes showed a tendency to increase in size, mediastinal lymph node biopsy was performed in the following year. Castlemans disease was diagnosed, and he was followed up. In 2005, autoimmune pancreatitis (AIP) developed. At this time, the mediastinal lymph node that had been biopsied was stained with anti-IgG4 antibody. Further examinations on pancreatic lesions associated with Castlemans disease and AIP are necessary in relation to IgG4-related systemic diseases.

[Superior mesenteric arterial thrombosis complicating ulcerative colitis: A case report].

36-year-old men with ulcerative colitis was attacked by the colic. The thrombus in superior mesenteric artery was revealed by computed tomography. Because the effect of the thrombolysis under intrarterial angiography was insufficient, thrombectomy was enforced under the laparotomy in the same day. The arterial thrombosis is extremely rare in the complication of ulcerative colitis. The activity of ulcerative colitis as one of factors of the appearance of thrombus was suggested.