Multicystic biliary hamartoma mimicking intrahepatic cholangiocarcinoma: report of a case

Multicystic biliary hamartoma (MCBH) is a rare cystic disease of the liver. A 69-year-old man was referred to our hospital with radiographic abnormality. Physical examination of the patient was unremarkable, and he denied any previous medical, travel, or surgical history. An abdominal computed tomography (CT) scan demonstrated a 3-cm low-density lesion in segment 3 of the liver, with dilation of the intrahepatic bile duct. The peripheral site of this lesion was slightly enhanced in the arterial phase. In the portal phase, the peripheral site was enhanced more clearly and showed a honeycomb-like dilated bile duct. Ultrasonography also revealed that the lesion was an irregularly shaped mass. On magnetic resonance imaging (MRI), T1-weighted images revealed a low-density mass and T2-weighted images revealed a dappled-density mass with honeycomb-like dilated bile duct and dilation of major intrahepatic bile duct. The patient was diagnosed with intrahepatic cholangiocarcinoma (ICC) and underwent left hepatectomy. However, pathological findings revealed that the lesion was MCBH. Our case highlights the potential difficulties in differentiating between MCBH and ICC under such circumstances.     

Pulmonary involvement: a rare extraintestinal manifestation of ulcerative colitis

Ulcerative colitis (UC) is associated with a number of extraintestinal manifestations (EIMs) that may affect most organ systems. Among the EIMs, those involving the lung are rare. We report a case of pulmonary involvement and pyoderma gangrenosum in a patient with refractory UC. A chest computed tomography showed multiple nodular infiltrates in bilateral lungs. The patient had no respiratory symptoms. No infectious agents were detected. A transbronchial biopsy specimen showed nonspecific features. Prednisolone was initiated with significant improvement in the patient’s abdominal symptoms and pyoderma gangrenosum. Subsequent imaging after steroid therapy showed improvement of the pulmonary infiltrates. The patient’s abdominal symptoms relapsed when prednisolone was tapered. The patient subsequently received a proctocolectomy. Chest radiographs have shown resolution of pulmonary infiltrates. Because pulmonary involvement follows an independent course and a proctocolectomy may not be protective against a recurrence of pulmonary involvement, a careful follow-up should be continued.     

The efficacy and safety of piperacillin-tazobactam for febrile neutropenic patients in Japan

The IDSA guideline for management of febrile neutropenic patients updated in 2010 recommends monotherapy with anti-pseudomonal-lactam agents, including piperacillin-tazobactam (PIPC/TAZ) for high-risk patients. However, clinical studies of PIPC/TAZ are limited in Japanese patients. In this study, we conducted an open-labeled non-randomized prospective trial to examine the efficacy and safety of PIPC/TAZ as an empirical treatment for Japanese patients with febrile neutropenia. Forty-nine febrile episodes in neutropenic patients excluding those undergoing allogeneic stem cell transplantation (high risk 36, low risk 13) were analyzed. The overall response rate was 71%, and no significant differences between the high-risk and the low-risk group were observed (high risk 72%, low risk 69%). Neither PS nor usage of G-CSF affected the response rate. No major side effects were observed in the study. The efficacy and the safety profile of PIPC/TAZ treatment were comparable to those in other previous Western studies. In conclusion, this study suggests PIPC/TAZ is effective and well tolerated as an initial empirical treatment for febrile neutropenic Japanese patients.     

Serum immunoglobulin G4 associated with number and distribution of extrapancreatic lesions in type 1 autoimmune pancreatitis patients

Background and Aim: Type 1 autoimmune pancreatitis (AIP) is characterized by the increase of serum immunoglobulin (Ig)G4 and abundant IgG4 plasma cell infiltration in the pancreas and various extrapancreatic lesions (EPL), which are proposed as IgG4‐related disease. We assessed the correlation between serum IgG4 and the number of EPL, and the association between serum IgG4 and the distribution of EPL in type 1 AIP patients. Methods: Serum IgG4 was measured in 35 type 1 AIP patients and 71 non‐AIP patients. The clinical characteristics and distribution of eight EPL were determined in 35 type 1 AIP patients. Results: Serum IgG4 in type 1 AIP was significantly higher than in non‐AIP (P < 0.001). A total of 33 patients had EPL among 35 patients with type 1 AIP (94.3%). There was a significant correlation between serum IgG4 and the number of EPL (ρ = 0.75, P < 0.001). Further, to assess the association between serum IgG4 and the distribution of EPL, type 1 AIP patients were divided into two groups: as abdominal localized EPL and systemic EPL. Both serum IgG4 and total numbers of EPL in systemic EPL were remarkably higher than those in abdominal localized EPL. Serum IgG4 cut‐off value was 346 mg/dL to distinguish between abdominal localized EPL and systemic EPL according to the receiver–operator characteristic curve data. Conclusions: Our findings indicated that serum IgG4 was useful in both the diagnosis of type 1 AIP and the detection of systemic EPL. Our finding may help the concept and diagnostic criteria of IgG4‐related disease with type 1 AIP.