Rethinking fibrinogen storage disease of the liver: ground glass and globular inclusions do not represent a congenital metabolic disorder but acquired collective retention of proteins.

Three types of intracytoplasmic inclusions immunoreactive to fibrinogen are collectively diagnosed as hepatic fibrinogen storage disease. This study aimed to better characterize ground glass (type II) and globular (type III) fibrinogen inclusions by the pathological examination of 3 cases and a literature review. Three adults (age: 32-64 years; male/female = 2:1) were unexpectedly found to have fibrinogen-positive ground glass changes (type II inclusions) by liver needle biopsy, against a background of acute hepatitis E, resolving acute cholangitis, or severe lobular hepatitis of unknown etiology.

[A Case of Granulocyte-Colony Stimulating Factor Producing Breast Cancer That Caused Rapid Progression].

We report a case of granulocyte-colony stimulating factor(G-CSF)producing breast cancer in a 54-year-old woman. Eight months after surgery, multiple liver and pulmonary metastases appeared. They progressed rapidly in 2 weeks and she had a high fever of 38 degrees.

Hepatic angiomyolipomas may overexpress TFE3, but have no relevant genetic alterations.

The fusion or amplification of TFE3 has been identified as one of the molecular events underlying tumorigenesis in perivascular epithelioid cell tumors (PEComas). TFE3 rearrangements in PEComas are related to the morphological features of the epithelioid appearance and weaker expression of immunohistochemical muscular markers. This study aimed to clarify whether these genetic alterations are involved in hepatic angiomyolipomas (AMLs), which are a member of the PEComa tumor family.

Carcinosarcoma of the Extrahepatic Bile Duct Presenting with Stone-like Radiological Findings.

A 73-year-old woman was referred to our hospital due to epigastralgia and jaundice. The radiological findings showed a stone-like tumor in the extrahepatic bile duct. The patient was initially thought to have adenocarcinoma of the bile duct based on the findings of a pathological examination of the bile duct biopsy specimen and underwent pancreaticoduodenectomy; the final diagnosis of the lesion was so-called carcinosarcoma of the extrahepatic bile duct.

Gangliocytic paraganglioma: a multi-institutional retrospective study in Japan.

Background: Gangliocytic paraganglioma (GP) is an extremely rare benign tumor that commonly arises from the second part of the duodenum. Since GP exhibit neither prominent mitotic activity nor Ki-67 immunoreactivity, this tumor is often misdiagnosed as neuroendocrine tumor (NET) G1 (carcinoid tumor). However, patients with GP may have a better prognosis than patients with NET G1.

Regression of follicular lymphoma of the duodenum following eradication of H. pylori infection.

A 64-year-old woman was referred for an examination of the upper gastrointestinal (GI) tract. Endoscopy showed an elevated lesion in the duodenum with central depression and multiple white granules. Biopsy specimens revealed lymphoid follicles composed predominantly of centrocytes with scattered centroblasts.

Extra-gastrointestinal stromal tumor of the pelvic cavity: case report.

Extra-gastrointestinal stromal tumors (E-GISTs) not associated with the alimentary tract in the pelvic cavity are extremely rare. We treated a 49-year-old Japanese man with such an E-GIST in the pelvic cavity who underwent an intrapelvic tumorectomy with a total prostatectomy and partial rectum resection. Gross examination of the specimen revealed an 8.

[Effectiveness of systemic chemotherapy of GEM+CBDCA+5-FU/LV and hepatic arterial infusion of CDDP in a case of advanced, combined hepatocellular-cholangiocarcinoma with multiple lung metastases].

This patient is a male in his 30's. He was diagnosed as hepatitis B virus-related huge primary liver cancer, 10cm in diameter, located in segment 4, accompanied with left portal thrombus and multiple lung metastases. Ten months after repeating systemic chemotherapy using gemcitabine (GEM)+carboplatin (CBDCA)+5-FU/leucovorin (LV) and hepatic arterial infusion chemotherapy with cisplatin (CDDP) 4 times, extended left lobectomy with caudate lobe could be successfully performed because of marked reducion of the huge tumor.

A case of mixed ductal-endocrine carcinoma of the pancreas.

A 66-year-old male patient underwent a stomach-preserving pancreatoduodenectomy procedure because of a tumor located around the lower bile duct under the diagnosis of carcinoma of the lower bile duct. The tumor (3.5 × 2.

Adenocarcinomas associated with perianal fistulae in Crohn's disease have a rectal, not an anal, immunophenotype.

Aims: Perianal fistulae are often observed in patients with Crohn's disease (CD), although the development of associated adenocarcinomas is very rare. The origin of adenocarcinomas in perianal fistulae associated with CD remains controversial and includes adjacent anal glands or rectal mucosa. Here, we attempted to determine the origin.

CD44 expression is related to poor prognosis of hypopharyngeal squamous cell carcinoma.

Conclusions: CD44 expression in hypopharyngeal squamous cell carcinomas (SCCs) is closely associated with poor prognosis for patients. CD44 may serve as a prognostic marker for hypopharyngeal SCCs.

Objectives: CD44, an adhesion molecule binding to extracellular matrix, is believed to participate in the progression of malignancies.

Concomitant adenocarcinoma and colonic non-Hodgkin's lymphoma in a patient with ulcerative colitis: a case report and molecular analysis.

Ulcerative colitis (UC) complicated by colonic lymphoma is rare, although UC is often accompanied by adenocarcinoma of the colon. A concurrent existence of adenocarcinoma and lymphoma in a patient with UC is extremely rare, and has not yet been analyzed at the molecular level. We report a 64-year-old female patient with concomitant adenocarcinoma and diffuse large B-cell lymphoma (DLBCL) in the colon of UC.

Detection of desmoplastic reaction in biopsy specimens is useful for predicting the depth of invasion of early colorectal cancer: a Japanese collaborative study.

Background: We have previously demonstrated a relationship between the depth of submucosal invasion (SM depth) and the frequency of lymph node metastasis in resected submucosal invasive colorectal cancers (SICRCs). Here, we assessed the desmoplastic reaction (DR) in pretreatment biopsy specimens of SICRC to predict the SM depth.

Methods: A total of 359 patients with SICRCs, who had undergone surgical or endoscopic mucosal resection, were enrolled.

Immunocytochemistry of CD146 is useful to discriminate between malignant pleural mesothelioma and reactive mesothelium.

Malignant pleural mesothelioma is a refractory tumor with poor prognosis associated with asbestos exposure. Pleural effusion is frequently observed in patients with malignant pleural mesothelioma, and cytological analysis is effective to detect malignant pleural mesothelioma. However, cytological discrimination between malignant pleural mesothelioma and reactive mesothelium is often difficult.

Crucial role of impaired Kupffer cell phagocytosis on the decreased Sonazoid-enhanced echogenicity in a liver of a nonalchoholic steatohepatitis rat model.

Aims: To evaluate the dynamics of Kupffer cell (KC) phagocytosis by performing both in vivo and in vitro studies using Sonazoid (GE Healthcare, Oslo) in a rat nonalcoholic steatohepatitis (NASH) model.

Methods: Contrast enhanced ultrasonography (CEUS) was performed on a rat NASH model induced by a methionine choline deficient diet (MCDD) and control rats, and Sonazoid was used to measure the signal intensity in the liver parenchyma. The uptake of Sonazoid by the KCs was observed by intravital microscopy.

Malignant mesothelioma of the peritoneum: case reports and immunohistochemical findings including Ki-67 expression.

A malignant mesothelioma (MM) is an aggressive neoplasm, although some patients have shown long-term survival, and factors related to survival remain uncertain. We present three cases of MM of the peritoneum including autopsy results, in which factors related to long-term survival were investigated. Case 1 was a 69-year-old man who died 6 years after the initial diagnosis.

Immunohistochemical analyses of E-cadherin, beta-catenin, CD44s, and CD44v6 expressions, and Ki-67 labeling index in intraductal papillary mucinous neoplasms of the pancreas and associated invasive carcinomas.

We examined the expressions of adhesion molecules (E-cadherin, beta-catenin, CD44s, and CD44v6) and Ki-67 labeling index (Ki-67 LI) in low- and moderate-grade dysplasia and invasive carcinoma components in ten noninvasive intraductal papillary mucinous neoplasms (IPMNs) of the pancreas and eight invasive carcinomas associated with IPMNs of the pancreas using immunohistochemical methods. There was no significant difference in regard to the proportion of components expressing either E-cadherin or beta-catenin in more than 70% of the tumor cells between the low- and moderate-grade dysplasia components. In contrast, the proportion of those in invasive carcinoma components was significantly lower than in low- or moderate-grade dysplasia components.

Well-differentiated papillary mesothelioma with invasion to the chest wall.

Well-differentiated papillary mesothelioma (WDPM) is an uncommon tumor with a papillary architecture, bland cytologic features, a tendency toward superficial spread without invasion, and good prognosis with prolonged survival. WDPM occurs primarily in the peritoneum of women, but also rarely in the pleura. We here report a case of 48-year-old woman who developed WDPM in the pleura with no history of asbestos exposure.

Comparison between colorectal low- and high-grade mucinous adenocarcinoma with MUC1 and MUC5AC.

Aim: To explore useful prognostic factors for mucinous adenocarcinoma (MAC) in the colon and rectum.

Methods: MAC was divided into low- and high-grade types based on the degree of structural differentiation; low-grade MAC arisen from well to moderately differentiated adenocarcinoma and papillary carcinoma, and high-grade MAC from poorly differentiated adenocarcinoma and signet ring cell carcinoma. Immunohistochemically, the expression of 2 types of MUC1 (MUC1/DF and MUC1/CORE), MUC2, 2 types of MUC5AC (MUC5AC/CHL2 and HGM), MUC6, CDX2, and CD10 was examined in 16 cases of MAC consisting of 6 low- and 10 high-grade types.

Comparison between mucinous cystic neoplasm and intraductal papillary mucinous neoplasm of the branch duct type of the pancreas with respect to expression of CD10 and cytokeratin 20.

Objective: Mucinous cystic neoplasm (MCN) and intraductal papillary mucinous neoplasm of the branch duct type (IPMN-BD) differ in biological and clinical behaviors, but MCN is often misdiagnosed as IPMN-BD. The purpose of this study was to find useful markers for the differential diagnosis of MCN and IPMN-BD.

Methods: Immunohistochemically, the expression of the 2 types of mucin (MUC) 1 (MUC1/DF3 and MUC1/CORE), MUC2, MUC5AC, MUC6, human gastric mucin (HGM), caudal-related homeobox transcription factor 2 (CDX2), CD10, cytokeratin (CK) 7, and CK20 was examined in 7 cases of MCN and 16 cases of IPMN-BD.

Undifferentiated carcinoma with osteoclast-like giant cells arising in a mucinous cystic neoplasm of the pancreas.

A 26-year-old woman presented with pain in the left hypochondrium, for which pancreatectomy and splenectomy was performed, with total gross excision of a mass. A tumor measuring 11 x 9 cm was found in the pancreas. On cut surface there were two cysts filled with a necrotic substance and hemorrhagic content.

Perivascular epithelioid cell tumor of the rib.

We present a rare case of perivascular epithelioid cell tumor (PEComa) in the right 6th rib of a 28-year-old man. A plain computed tomography scan showed a round osteolytic lesion in the right 6th rib. The resected tissue contained a globular-shaped, soft tumor.

Poorly differentiated adenocarcinoma with signet-ring cell carcinoma in a hyperplastic polyp of the stomach: report of a case.

Hyperplastic polyps (HPs) of the stomach have been reported to be mostly benign. However, in rare cases, carcinomas have been found in HPs. We treated a 59-year-old Japanese male who underwent a total gastrectomy, and a gross examination of the resected stomach revealed a 4.