Transient and Recurrent Pulmonary Infiltrations Associated with Familial Mediterranean Fever.

Chest symptoms and pleural effusion due to serositis in familial Mediterranean fever (FMF) are occasionally misdiagnosed as acute pneumonia. However, the actual pulmonary involvement of FMF is extremely rare. A 67-year-old man was referred to our hospital due to repeated and transient anterior chest pain. Chest images revealed a moderate amount of pericardial fluid, slight bilateral pleural effusion, and infiltrations in both lower lung lobes. Colchicine treatment without antibiotics rapidly improved these symptoms and findings. Pericarditis, pleurisy and the response to colchicine indicated FMF. FMF should be considered as a causative disease of pulmonary infiltrations, especially if it occurs repeatedly.

The Acute Onset of Autoimmune Hepatitis During Pregnancy in the Absence of Hypergammaglobulinemia and Autoantibodies.

The onset of autoimmune hepatitis (AIH) during pregnancy is rare and often poses a diagnostic challenge. A 29-year-old Japanese woman experienced epigastric pain and nausea during the third trimester of her third pregnancy. Three days after the symptom onset, an emergency Caesarean section was performed because of suspected acute fatty liver of pregnancy; however, the patient's liver dysfunction worsened afterward. Despite normal serum IgG concentration and absence of autoantibodies, biopsy-proven severe hepatitis with centrilobular zonal necrosis and good biochemical response to corticosteroids led to a diagnosis of AIH. Therefore, AIH should be included in the differential diagnosis of liver dysfunction during pregnancy.

[A case of pancreatic cancer with clusters of invasive micropapillary carcinoma markedly reduced by chemotherapy].

A man in his 60s was hospitalized with multiple cerebral infarctions and referred for Trousseau's syndrome. Computerized tomography confirmed a 60-mm mass in the pancreatic head and swollen lymph nodes around the abdominal aorta. Fine needle aspiration cytology of the pancreatic lesion and laparoscopic para-aortic lymph node biopsy revealed adenocarcinoma, including clusters of invasive micropapillary carcinoma (IMPC). Chemotherapy (gemcitabine and nab-paclitaxel) markedly decreased the primary and metastatic lesions, and no recurrence was clinically detected 24 months later. To the best of our knowledge, reports of pancreatic IMPCs are rare. Our case was the seventeenth case of pancreatic cancer with IMPC. In this case, chemotherapy was markedly effective.

Can colonic inflammatory polyp with numerous immunoglobulin G4-positive plasma cells represent a colonic manifestation of immunoglobulin G4-related disease? A case report.

We present an asymptomatic case of a 79-year-old Japanese man who had a 6 mm colonic inflammatory polyp with numerous immunoglobulin G4 (IgG4)-positive plasma cells. No symptoms or abnormal laboratory data, such as changes in serum IgG4 levels, were found at the time of diagnosis or during the 1 year of follow-up thereafter. Additionally, no diffuse/localized swelling or masses were found in organs, except for colonic polyps, by abdominal computed tomography 1 year prior to the polypectomy. Inflammatory myofibroblastic tumor was unlikely from the lack of spindle cell proliferation and ALK immunoreactivity. This is the first case of this colonic polyp in an asymptomatic person. This polyp could be probable for single organ manifestation of IgG4-related disease (IgG4-RD), according to the comprehensive diagnostic criteria for IgG4-RD published in 2012; however, colonic manifestation of IgG4-RD has not been clarified owing to its rarity, and colon-specific criteria for IgG4-RD have not been proposed. Thus, we could not definitively establish the colonic polyp as IgG4-RD. Therefore, careful clinicopathological evaluation is needed to reveal whether this colonic polyp represents a nonspecific inflammatory response or an early manifestation of IgG4-RD.

Russell body gastritis with Dutcher bodies evaluated using magnification endoscopy.

Russell body gastritis (RBG) is an unusual type of chronic gastritis characterized by marked infiltration of Mott cells, which are plasma cells filled with spherical eosinophilic bodies referred to as Russell bodies. It was initially thought that Helicobacter pylori (H. pylori) infection was a major cause of RBG and that the infiltrating Mott cells were polyphenotypic; however, a number of cases of RBG without H. pylori infection or with monoclonal Mott cells have been reported. Thus, diagnostic difficulty exists in distinguishing RBG with monoclonal Mott cells from malignant lymphoma. Here, we report an unusual case of an 86-year-old-Japanese man with H. pylori-positive RBG. During the examination of melena, endoscopic evaluation confirmed a 13-mm whitish, flat lesion in the gastric antrum. Magnification endoscopy with narrow-band imaging suggested that the lesion was most likely a poorly differentiated adenocarcinoma. Biopsy findings were consistent with chronic gastritis with many Mott cells with intranuclear inclusions referred to as Dutcher bodies. Endoscopic submucosal dissection confirmed the diagnosis of RBG with kappa-restricted monoclonal Mott cells. Malignant lymphoma was unlikely given the paucity of cytological atypia and Ki-67 immunoreactivity of monoclonal Mott cells. This is the first reported case of RBG with endoscopic diagnosis of malignant tumor and the presence of Dutcher bodies.

Hepatoid Adenocarcinoma of the Extrahepatic Bile Duct in a Patient with Polysplenia Syndrome.

Hepatoid adenocarcinoma (HAC) is a rare subtype of extrahepatic adenocarcinoma that is characterized by its morphological and functional similarities to hepatocellular carcinoma. We herein present a novel case of HAC arising from the extrahepatic bile duct in a 75-year-old Japanese woman with polysplenia syndrome. This is the second reported case of HAC arising from this site. The tumor induced jaundice and hemobilia. A total of four isolated intraductal polypoid masses of HAC were found. No recurrence was seen five months after surgery. Further reports of similar cases will be needed to clarify the clinical characteristics and the prognosis of this malignancy.

Highest power magnification with narrow-band imaging is useful for improving diagnostic performance for endoscopic delineation of early gastric cancers.

BACKGROUND AND STUDY AIMS: Magnifying endoscopy with narrow-band imaging (ME-NBI) is more reliable than chromoendoscopy (CE) for delineating the horizontal extent of early gastric cancers prior to endoscopic submucosal dissection (ESD). However, the added benefits of ME-NBI over CE in terms of the difference in magnification level have yet to be elucidated. The aim of this study was to investigate the improvement in diagnostic accuracy for tumor delineation obtained with different magnification levels of ME-NBI following CE. PATIENTS AND METHODS: This was a retrospective study, performed at a single tertiary referral center. A series of 158 consecutive patients with 161 early gastric cancers resected en bloc using ESD was included in the study. The margins of each lesion were examined in their entirety using CE, followed by low power optical magnifying endoscopy with narrow-band imaging (LM-NBI), and finally the highest power optical magnifying endoscopy with narrow-band imaging (HM-NBI). The primary endpoint was the added benefit, as measured using the successful delineation rate, for the delineation of gastric cancer margins using CE + LM-NBI vs CE, and for CE + LM-NBI + HM-NBI vs CE + LM-NBI. RESULTS: The successful delineation rates (95 % CI) using CE, CE + LM-NBI and CE + LM-NBI + HM-NBI were 72.7 % (68.5-79.9 %), 88.9 % (84.2-93.8 %), and 98.1 % (95.8-100 %). The diagnostic accuracy improved significantly for CE + LM-NBI compared with CE (P < 0.001), and for HM-NBI compared with LM-NBI (P < 0.001). CONCLUSIONS: HM-NBI is useful for improving diagnostic performance for endoscopic delineation of early gastric cancers, following CE and LM-NBI.

Clinical and histological features of different types of Budd-Chiari syndrome: a comparison of 4 cases.

Budd-Chiari syndrome (BCS) is a rare condition characterized by hepatic venous outflow obstruction. In this report, we present 4 cases of BCS with complete and incomplete obstruction of the inferior vena cava (IVC) and hepatic vein (HV). Each case showed different and unique features of liver damage, which were attributed to the site and degree of obstruction. Interestingly, improved liver functions such as increased serum albumin levels, decreased hyaluronic acid levels and a normal indocyanine green clearance test were evident within 1 month of the balloon angioplasty. Pericellular fibrosis and hypervascular regenerative nodules were also reversible after obstruction removal. Therefore, it is very important to manage this rare disease before it progresses to liver cirrhosis.

Meckel's diverticulum preoperatively diagnosed by double-balloon endoscopy.

Symptomatic Meckel's diverticulum is an uncommon diagnosis in adults, and bleeding Meckel's diverticulum after childhood is even more infrequent. We present herein the case of a 22-year-old man with gastrointestinal hemorrhage secondary to Meckel's diverticulum containing ectopic gastric mucosa. As the source of bleeding could not be identified by upper and lower gastrointestinal endoscopy and visceral selective angiography, the new methods of capsule endoscopy and double-balloon endoscopy were used. Capsule endoscopy showed oozing hemorrhage in the ileum, and double-balloon endoscopy demonstrated a large diverticulum in the distal part of the ileum. Tc-99m pertechnetate Meckel's scan revealed an abnormal focus of uptake in the right lower abdomen. The diverticulum was resected laparoscopically. The postoperative course was uneventful, and the patient remains in complete remission as of this writing. Detecting Meckel's diverticulum endoscopically is difficult prior to surgery, but a combination of capsule endoscopy and double-balloon endoscopy facilitates examination of the entire small intestine, making precise diagnosis of Meckel's diverticulum possible.

Anaplastic carcinoma of the pancreas with rhabdoid features and hyaline globule-like structures.

A 59-year-old Japanese man presented with a giant submucosal tumor with ulceration during follow-up of duodenal ulcer. Pancreaticoduodenectomy was undertaken, and subsequent histological examination of the tumor disclosed anaplastic carcinoma of the pancreas head. The carcinoma components contained adenocarcinoma and squamous cell carcinoma. Additionally, undifferentiated spindle or pleomorphic cells were seen in continuity with the carcinoma component. Undifferentiated neoplastic cells with rhabdoid features and with hyaline globule-like structures positive for PAS stain with diastase pretreatment were also observed. Immunohistochemically, cytoplasmic inclusions corresponding to rhabdoid features showed aggregates of vimentin. Ultrastructurally, hyaline globule-like structures corresponded to lysosomes. Finally, we report here the first case of anaplastic carcinoma of the pancreas with hyaline globule-like structures.

Granular cell tumor arising metachronously in the bronchus and esophagus.

We present a case of granular cell tumor (GCT) occurring in the esophagus 7 years after operation for bronchial GCT. A 59-year-old Japanese man complained of epigastralgia, and endoscopic examination of the upper digestive tract disclosed a submucosal tumor in the lower esophagus. Histological examination of the endoscopic mucosal resection of the esophageal tumor showed a proliferation of neoplastic cells with an eosinophilic and granular cytoplasm. The cytoplasm of the neoplastic cells was histochemically positive for PAS stain and immunohistochemically positive for S-100. This tumor did not fulfill any of the diagnostic criteria for malignancy at either the macroscopic or microscopic level. I believe that this is the first case of GCT occurring metachronously in the respiratory and digestive tracts. Clinicians and pathologists should bear in mind that GCT may arise metachronously in the respiratory and digestive tracts.