[An autopsy case of nasal-type primary gastric extranodal NK/T-cell lymphoma with early liver failure].

An 82-year-old woman was admitted to the hospital because of tiredness and fever. She was diagnosed with acute hepatitis. Although the cause of acute hepatitis was undetermined, her health condition and liver function improved, and she was discharged. Four weeks later, she was hospitalized again because of anorexia. Laboratory data revealed worsened anemia. Endoscopy results revealed a huge ulcerative lesion in the lesser curvature of the stomach. After 4 days, she vomited blood and died of hemorrhagic shock. The autopsy revealed a nasal-type primary gastric extranodal NK/T-cell lymphoma (ENKTL). Although no lymphoma cells were found in the liver biopsy collected during the first hospitalization, lymphoma cells and lymphocytes in the liver tissue were identified during autopsy because the lymphoma had infiltrated the liver. Primary gastric ENKTL is extremely rare and poorly understood. However, the general prognosis of progressive ENKTL is poor. Early diagnosis of liver metastasis of lymphoma cells is difficult;thus, in some cases, lymphoma metastases to the liver are diagnosed during autopsy. Although further experiments are required, we report a rare case of primary gastric ENKTL.

Efficacy of polyglycolic acid sheeting with fibrin glue for perforations related to gastrointestinal endoscopic procedures: a multicenter retrospective cohort study.

OBJECTIVES: Gastrointestinal (GI) perforations are one of the major adverse events of endoscopic procedures. Polyglycolic acid (PGA) sheets with fibrin glue have been reported to close GI perforations. However, its clinical outcome has not yet been fully investigated; thus, we conducted a multicenter retrospective observational study to assess the efficacy of PGA sheeting for GI perforation. METHODS: The medical records of patients who underwent PGA sheeting for endoscopic GI perforations between April 2013 and March 2018 in 18 Japanese institutions were retrospectively analyzed. PGA sheeting was applied when the clip closure was challenging or failed to use. Perforations were filled with one or several pieces of PGA sheets followed by fibrin glue application through an endoscopic catheter. Nasal or percutaneous drainage and endoscopic clipping were applied as appropriate. Clinical outcomes after PGA sheeting for intraoperative or delayed perforations were separately evaluated. RESULTS: There were 66 intraoperative and 24 delayed perforation cases. In intraoperative cases, successful closure was attained in 60 cases (91%). The median period from the first sheeting to diet resumption was 6 days (interquartile range [IQR], 4-8.8 days). Large perforation size (≥ 10 mm) and duodenal location showed marginal significant relationship to higher closure failure of intraoperative perforations. In delayed perforation cases, all cases had successful closure. The median period from the first sheeting to diet resumption was 10 days (IQR, 6-37.8 days). No adverse events related to PGA sheeting occurred. CONCLUSION: Endoscopic PGA sheeting could be a therapeutic option for GI perforations related to GI endoscopic procedures.

A case of recurrence of a solid pseudopapillary neoplasm of the pancreas effectively treated with proton beam radiotherapy.

We report a case of a male in his 50 s who underwent pancreaticoduodenectomy for solid pseudopapillary neoplasm (SPN) of the pancreas at 30 years. He developed a liver abscess 15 years after the surgery, and CT scan revealed a swollen retroperitoneum lymph node and a tumor in the liver. Symptoms, including abdominal distension, appetite loss, and epigastric pain, appeared due to lymph node metastasis. Endoscopic ultrasonography-guided fine-needle aspiration against the lymph node revealed SPN recurrence. The tumor had invaded the common hepatic artery, and surgery was not indicated. Chemotherapy of Gemcitabine/nab-Paclitaxel biweekly was performed 8 times; however, no reduction in tumor size was observed, and the patient's symptoms worsened. Proton beam therapy (67.5 GyE in 25 fractions) was subsequently performed for lymph node metastasis, and led to a gradual reduction in lymph node metastasis, and an improvement in symptoms. No re-expansion of lymph node metastasis has been observed 3 years after proton beam therapy. Since SPN is low malignancy and most cases can be expected to be cured by surgery, there is currently no standard treatment of unresectable SPN. This case is the first report of proton beam therapy for SPN, and was considered to be effective.

[Mesalazine-induced myocarditis].

A 30-year-old man presented with constipation and abdominal pain. He was suspected of having ulcerative colitis, and administration of 2400mg/day of oral mesalazine was initiated. After 10 days of treatment, he experienced fever and chest pain. Blood examination, electrocardiography, and cardiac ultrasonography revealed elevated cardiac enzymes, ST-segment elevation, and diffuse hypokinesis, respectively. Mesalazine-induced acute myocarditis was diagnosed based on a positive drug-induced lymphocyte stimulation test and the absence of other myocarditis-causing conditions. Prompt cessation of mesalazine quickly improved his heart function and test results. Although rare, clinicians should consider the possibility of cardiac adverse events caused by mesalazine.

Endoscopic ultrasonography-guided drainage of infected intracystic papillary adenocarcinoma of the liver.

We describe a case of effective use of endoscopic ultrasonography (EUS)-guided drainage of an infected intracystic papillary adenocarcinoma (ICPA) of the liver. The patient was an 84-year-old woman who was admitted with complaints of continuous epigastric pain and a slight fever. Laboratory data revealed severe inflammation. Computed tomography scanning showed a 110-mm cystic lesion with enhanced papillary tumors in the medial segment of the liver associated with a cyst in the right lobe and subcapsular cyst of the liver. Streptococcus species were detected in the culture of cystic fluid, and a diagnosis of infected ICPA was suspected. Although the patient was medicated by antibiotics, the fever did not resolve. EUS-guided transgastric drainage was performed for the abscess of the medial segment of the liver. Fourteen days after the endoscopic procedure, the plastic drainage tube was replaced with a metal stent. Inserting an endoscope into the liver cyst through the metal stent permitted observation and biopsy of an intracystic tumor, and the diagnosis of ICPA was confirmed. The patient was discharged with the internal metal stent still in place.

Recurrent attacks of autoimmune pancreatitis result in pancreatic stone formation.

OBJECTIVES: Autoimmune pancreatitis has been characterized by irregular narrowing of the main pancreatic duct and sonolucent swelling of the parenchyma, both of which are due to lymphoplasmacytic inflammation at the active stage of the disease, and by the absence of pancreatic stone formation. The aim of the present study was to confirm or deny whether or not this disease is progressive with recurrent attacks, resulting in pancreatic stone formation like ordinary chronic pancreatitis. METHODS: Forty-two patients, 36 of whom were treated with prednisolone, were followed up for periods longer than 12 months (median follow-up period: 54.5 months, range: 13-111 months) by regular interview and examination of their medical records for laboratory tests and image tests. RESULTS: Eleven patients (26.2%) who were treated with prednisolone showed recurrent attacks during median follow-up periods of 22 months. Eight patients (19%) showed the formation of pancreatic stones during the follow-up periods. Because 6 of 11 patients (54.5%) who suffered relapse showed pancreatic stone formation, it is significantly associated with relapse in comparison with nonrelapse (p= 0.0019). CONCLUSIONS: Contrary to previous reports, we observed both relapse and pancreatic stone formation in some patients with autoimmune pancreatitis, which suggests that autoimmune pancreatitis has the potential to be a progressive disease with pancreatic stones.

Hilar and pancreatic gallium-67 accumulation is characteristic feature of autoimmune pancreatitis.

INTRODUCTION AND AIMS: Autoimmune pancreatitis is characterized by severe lymphocytic inflammation, suggesting that gallium-67 scintigraphy provides a useful tool for detecting characteristic lesions of this disease, because gallium-67 concentrates in lymphoid cells. We tried to determine whether gallium-67 accumulates in the characteristic lesions. METHODOLOGY: We performed gallium-67 scintigraphy in 24 patients with autoimmune pancreatitis before and after 4 weeks of corticosteroid therapy and determined the factors associated with positive images. RESULTS: Sixteen patients (67%) had marked gallium-67 accumulation in the pancreas before corticosteroid therapy and negative images after 4 weeks of therapy, and they had significantly higher serum IgG4 values than did those without gallium-67 accumulation (median, 758 mg/dL versus 329 mg/dL; p = 0.011). Marked hilar gallium-67 accumulation was found in 16 patients (67%) and was also associated with significantly higher serum IgG4 values than did those without it (median, 758 versus 239 mg/dL; p = 0.0044). Among 16 patients with positive hilar images, 12 had positive pancreatic uptake and 5 had both pancreatic and salivary gland uptakes. CONCLUSIONS: Hilar and pancreatic accumulation of gallium-67 is a characteristic feature of autoimmune pancreatitis during the active stage of the disease, when IgG4 serum levels are high.

Usefulness of endoscopic observation of the main duodenal papilla in the diagnosis of sclerosing pancreatitis.

BACKGROUND: There are no descriptions of the appearance of the main duodenal papilla in sclerosing pancreatitis. The intent of the present study was to clarify the characteristics of the main duodenal papilla in patients with sclerosing pancreatitis. METHODS: Macroscopic findings at ERCP with reference to the main duodenal papilla of 17 patients with sclerosing pancreatitis were compared with those of 24 patients with normal ERCP findings, 11 with chronic pancreatitis, 13 with primary sclerosing cholangitis, 21 with pancreatic cancer, and 18 with bile duct cancer. Endoscopic photographs of the papilla were reviewed retrospectively by 3 observers blinded to the underlying pancreaticobiliary pathology. Degree of swelling was scored in all patients. Biopsy specimens from swollen papillae were assessed histopathologically in 3 patients with sclerosing pancreatitis. RESULTS: Severe swelling of the main duodenal papilla was observed in 7 (41%) of 17 patients with sclerosing pancreatitis. The total score for the degree of swelling in patients with sclerosing pancreatitis was significantly higher than that for patients with a normal ERCP, chronic pancreatitis, primary sclerosing cholangitis, pancreatic cancer, and bile duct cancer (p < 0.01). T-lymphocyte infiltration of the papilla was evident in the biopsies from 3 patients with sclerosing pancreatitis. CONCLUSIONS: A swollen main duodenal papilla was a characteristic finding in patients with sclerosing pancreatitis. T-lymphocyte infiltration is present in the swollen main duodenal papilla. These features may be useful in the diagnosis of sclerosing pancreatitis.