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Background: Clonal evolution of malignancy is a complex process related to intratumoral heterogeneity, as recent studies have also demonstrated in rhabdomyosarcoma. The purpose of this study is to present a distinct clonal feature of a case with anaplastic embryonal type rhabdomyosarcoma (ERMS) using molecular analysis. Methods: A five‐year‐old girl developed a metastatic pelvic tumor. We cultured neoplastic cells isolated from the biopsy sample. Next, to characterize the current case, we analyzed the biopsy sample, autopsy sample, and established cell line using combined modalities, including histopathological, cytogenetic, and molecular assay. We also undertook the backtrack mutation‐specific polymerase chain reaction to reveal clonal composition. Results: The histology of the biopsy sample was consistent with ERMS with focal anaplasia. We established a permanently growing cell line, ICH‐ERMS‐1, from the biopsy sample. On molecular analysis, the biopsied tissue revealed a missense mutation at codon 245 of TP53. In contrast, the autopsy tumor tissue and the cell line established from the biopsied tissue showed a missense mutation at codon 248. A backtrack study using mutation‐specific polymerase chain reaction detected a TP53 codon 248 mutation in the original biopsy sample. All the specimens examined had a missense mutation at PTPN11 codon 69. Conclusions: This study highlights intratumoral heterogeneity and distinct clonal change related to the functional context in our anaplastic ERMS case, supporting the concept of intratumoral heterogeneity and clonal evolution. It requires further case collection to reveal whether p14ARF–p53–MDM2 tumor suppressor pathway alteration, considered a late event in ERMS tumorigenesis, is responsible for anaplasia in ERMS. [ABSTRACT FROM AUTHOR]

Background and Objectives. Recently, a 21G Menghini-type needle for EUS-guided fine-needle aspiration biopsy (EUS-FNAB) has been developed. The stylet of the EUS Sonopsy CY™ remains inside the needle during aspiration. Therefore, it is expected to obtain higher-quality histological core specimens without crushing the material or blood contamination. The aim of this study is to evaluate the feasibility and diagnostic accuracy of EUS-FNAB of solid pancreatic masses with this new biopsy needle. Methods. A total of 30 patients with solid pancreatic masses who underwent EUS-FNAB with two different types of needles, EUS Sonopsy™ and ProCore™, were included in a prospective, randomized, controlled, crossover study. All the pancreatic masses were punctured with the two needles and were randomized regarding the order of the needle to be used. The primary outcome was to compare the diagnostic accuracy and the rates of tissue acquisition of the two needles. Results. The tissue acquisition rate was not significantly different between the EUS Sonopsy CY™ needle and the ProCore™ needle (78.6% vs. 82.1%, P=1.00). The histological diagnostic accuracy was also similar between the two needles (73% vs. 80%, P=.63). There was also no difference regarding the accuracy of cytology alone and the combination of both histological and cytological assessments between the EUS Sonopsy CY™ needle and the ProCore™ needle (90% vs. 87%, P=1.00 and 90% vs. 90%, P=1.00, respectively). Conclusions. EUS Sonopsy CY™ is a reliable device for EUS-FNAB of solid pancreatic masses. [ABSTRACT FROM AUTHOR]

Objectives. Recently, opportunities to encounter superficial nonampullary duodenal epithelial tumor (SNADET) have increased. EMR and ESD are performed to treat SNADET. However, the rate of perforation is higher than that of other gastrointestinal lesions, regardless of which method is used. Underwater EMR (UW-EMR) is immersion treatment of SNADET, which has low risk of perforation and can remove lesions safely and completely. In the present study, we retrospectively investigated patients in whom UW-EMR was performed to evaluate the feasibility and safety of UW-EMR for the treatment of SNADET. Methods. The primary endpoint was to evaluate the feasibility of UW-EMR for the treatment of SNADET, and secondary objective was to determine the operation’s safety. Results. There were 14 participants, with a total of 16 lesions, who underwent UW-EMR between August 2015 and December 2017. Histological heteromorphism revealed that seven patients had low-grade adenoma, seven had high-grade adenoma, and two had adenocarcinoma. En bloc resection was performed in 14 lesions. In two patients, nodular lesions were observed in the scar and biopsy confirmed recurrences. There were no serious adverse events including bleeding or perforation. Conclusions. UW-EMR may be a safe and effective treatment method for SNADET, if its therapeutic indication is adequately considered. [ABSTRACT FROM AUTHOR]

A 76‐year‐old man presented with shortness of breath. Computed tomography revealed ground‐glass opacity and interlobular thickening in the right lower lobe. Blood examination showed elevated levels of white blood cell count and lymphocytes. Bone marrow aspiration revealed low‐grade follicular lymphoma. Histopathological examination of the surgical lung biopsy from the right lower lobe demonstrated usual interstitial pneumonia and scattered aggregation of lymphocytes with poorly formed non‐necrotizing granuloma. An 18F‐fluorodeoxyglucose positron emission tomography‐computed tomography (18F‐FDG PET‐CT) did not show intense uptake in areas other than the right lower lobe. We concluded that the granuloma in the lung was presumed to be a sarcoid reaction associated with bone marrow follicular lymphoma, and the intense 18F‐FDG uptake in the right lower lobe might have been due to a sarcoid reaction. Immunohistochemistry or other genetic examinations are important even if 18F‐FDG uptake on PET‐CT seems to be a false‐positive because of the possibility of a sarcoid reaction. [ABSTRACT FROM AUTHOR]

Here we report a case of aggressive neuroendocrine tumor (NET), which is an extremely rare secondary solid tumor that occurs after allogeneic hematopoietic cell transplantation (allo-HSCT). A patient with chronic active Epstein- Barr virus infection received allo-HSCT from an HLA-DR two allele-mismatched unrelated donor. Four years later, he developed NET with multiple metastases. He received thoraco-abdominal irradiation as a conditioning regimen, and developed repeated episodes of intestinal graft-versus-host disease, for which he received long-term immunosuppressive therapy. Although these factors may be potential contributing factors to the development of secondary NET, the exact pathogenesis remains unclear. [ABSTRACT FROM AUTHOR]

Renal chromophobe cell carcinomas (ChCC) and oncocytomas express KIT. This character seems to reflect their common histogenesis from distal nephrons. In the normal kidney, however, the expression and localization of KIT are unclear. KIT expression in angiomyolipoma and congenital mesoblastic nephroma (CMN), is still controversial. c-kit mutations are reportedly rare in ChCC, but there is little information in other renal neoplasms, and no reported data on mutations of platelet-derived growth factor receptor (PDGFR). In order to address these issues the authors examined five ChCC, five oncocytomas, seven papillary cell carcinomas, two collecting duct carcinomas, 12 angiomyolipomas, and three CMN, as well as 10 normal renal tissues. In the normal kidney KIT was specifically expressed in the distal nephrons. Nine of 12 (75%) angiomyolipomas contained scattered KIT-positive cells, whereas all three CMN were completely negative for KIT. The presence of KIT-positive cells in angiomyolipomas was likely to correspond to that of melanocytic marker-positive cells, which mainly showed epithelioid morphology. Polymerase chain reaction-single-strand conformation polymorphism showed no evidence of mutations of c-kit or PDGFR in any of the tumors examined. [ABSTRACT FROM AUTHOR]

Protein kinase C (PKC) enzymes play a major role in signal transduction and contribute to the regulation of cellular differentiation and proliferation. However, little is known about subtype-specific intracellular expression of PKC in human malignant lymphoma. To characterize the relationship between expression of PKC and B-cell lymphomas based on the different subspecies, we investigated the expression of four subspecies (α, βII, γ and δ) in five cases of reactive lymphoid tissues, 77 cases of human B-cell lymphoma and 17 human lymphoma cell lines. In the reactive lymphoid tissues, PKC βII-positive cells were found in the mantle zones and marginal zones, and centroblasts and centrocytes in the germinal centers showed cytoplasmic staining with strong intensity against PKC δ. The present study is the first report to examine the expression of PKC δ in reactive lymphoid tissues. In interfollicular areas, a small number of T-cells were positive for PKC α. Protein kinase C γ-positive cells were not found in these lymphoid tissues. Eight cases of Burkitt lymphoma (BL) (8/10; 80%) showed the overexpression of PKC α ( P < 0.01), but other B-cell lymphoma cases except three cases of diffuse large B-cell lymphoma did not express PKC α. In addition, six and eight out of nine BL cell lines expressed the protein and mRNA of PKC α, respectively. These results indicate that PKC α was predominantly expressed on BL in comparison with other types of lymphoma. The expression of PKC γ was observed in only five cases of BL. The overall survival of PKC γ-positive BL was significantly better than that of PKC γ-negative BL ( P < 0.05). The expression of PKC γ seems to be associated with a better prognosis in the limited number of BL cases in the present study. [ABSTRACT FROM AUTHOR]

Abstract: At least two types of models, the vector model and the unfolding model can be used for the analysis of dichotomous choice data taken from, for example, the pick any/n method. The previous vector threshold models have a difficulty with estimation of the nuisance parameters such as the individual vectors and thresholds. This paper proposes a new probabilistic vector threshold model, where, unlike the former vector models, the angle that defines an individual vector is a random variable, and where the marginal maximum likelihood estimation method using the expectation-maximization algorithm is adopted to avoid incidental parameters. The paper also attempts to discuss which of the two models is more appropriate to account for dichotomous choice data. Two sets of dichotomous choice data are analyzed by the model. [ABSTRACT FROM AUTHOR]

CD10, a proteolytic enzyme seen in germinal center cells and in the majority of follicular lymphomas, is occasionally expressed in diffuse large B-cell lymphomas (DLBCL). To clarify the origin and cellular characteristics of CD10-positive DLBCL, we analyzed 36 de novo cases of DLBCL for somatic mutations of the immunoglobulin heavy chain variable region (VH) genes and for their immunophenotypes. Expression greater than that of grade 2 Bcl-6 was observed in 11 of the 30 CD10-negative cases (37%) and in all six CD10-positive cases (100%; P < 0.05) without expression of CD5, CD23, cyclin D1, CD30 or CD138. The average mutation frequencies of the six CD10-positive and 30 CD10-negative DLBCL were 12.9 and 9.8%, respectively. The range of SM frequencies in CD10-positive DLBCL (9.52–18.06) was distinctly narrower than that observed for CD10-negative DLBCL (0.69–26.89). These findings seem to indicate that CD10-positive DLBCL, originating from germinal center B cells, is a genetically and immunophenotypically more homogeneous group than CD10-negative DLBCL. Furthermore, three extranodal lymphomas, in five of the six CD10-positive DLBCL, showed ongoing mutation, indicating that antigen-driven, high-affinity somatic mutation may play an important role in clonal expansion in CD10-positive DLBCL. All four extranodal cases of the six CD10-positive DLBCL showed ongoing mutation and/or bcl-2/JH rearrangement. This result suggests that the cell origin of extranodal CD10-positive DLBCL may be the same as that of follicular lymphomas. [ABSTRACT FROM AUTHOR]

We have analyzed the immunoglobulin heavy chain (VH) gene variable regions (CDR2 and FW3) of 101 Japanese cases with peripheral B cell neoplasms. When all except one case with a deletion were graphed by frequency of replacement mutation, the 100 cases could be separated into two groups: 24 cases with zero, one and two mutations (germline or low frequency of somatic mutation); and 76 cases with three or more mutations (medium to high frequency of somatic mutation). While most mantle cell lymphoma cases (11/13) showed germline or low frequency of somatic mutation, all cases of mucosa-associated lymphoid tissue (MALT) lymphoma (11/11), follicular lymphoma (three of three cases), plasma cell myeloma (seven of seven cases) and most cases of diffuse large B cell lymphoma (DLBCL; 42/47) belonged to the latter group. These 76 cases, therefore, may be considered to show somatic hypermutation. More than half of chronic lymphocytic leukemia/small lymphocytic lymphoma cases (CLL/SLL; eight of 13) showed a hypermutated VH gene and the ratio of replacement mutation: silent mutation in CDR2 of CLL/SLL was considerably higher compared with DLBCL and MALT lymphoma, showing somatic hypermutation. When comparing VH gene type of B cell-CLL (B-CLL) among our series and those in the literature, more cases of CD5+ B-CLL in the Western literature have the VH5 and VH6 family types, while more cases in Japan are reported to have VH4 family. The occurrence of VH families in B-CLL between Japanese and Western people seems to be comparable. [ABSTRACT FROM AUTHOR]

One probabilistic version of Coombs' unfolding model called the MMUR (Marginalization model for the Multidimensional Unfolding analysis of Ranking data) is extended to treat ranking data for groups. One favorable feature of the model is that it can both take into consideration individual differences without estimating the subject parameters and capture the differences between the groups in a systematic manner. Another advantage lies in the fact that one can see the group differences in the geometrical point configuration, since the model shows how the ideal points of the groups differ from each other in space. Four applications are provided which demonstrate that the model is useful for clarifying systematic differences in this type of data. [ABSTRACT FROM AUTHOR]

The terrestrial communication network is making progress from the analog public network based mostly on telephone traffic to the N-ISDN. The development to B-ISDN also is considered, aiming at an integrated network which can provide large-scale file transfer, high-speed data communication such as the communication between high-speed LAN, and dynamic image communication such as TV and HDTV. This paper proposes the application of the multibeam satellite communication to such B-ISDN, and presents such technical subjects as the satellite communication. First, the purpose of application of the satellite communication to B-ISDN is described. The requirement in the application to B-ISDN is presented. Then, aiming at the fulfillment of the requirement, the technical subjects and evolution are shown for the configuration of the communication system, the channel control system, the synchronization control system, OAM system, and the interface to the terrestrial network. [ABSTRACT FROM AUTHOR]

The article presents a case study of a 19-year-old woman found a painless soft-tissue mass in her right knee after climbing. Magnetic resonance imaging showed a soft-tissue tumor which measures 84 x 56 x 60 millimeter (mm) and showing low intensity on T1-weighted imaging. Whole-body thallium scintigraphy and radiological analyses with chest-computed tomography showed no findings of other tumorous regions across the whole body.

We report a case of multiple unruptured sinus of Valsalva aneurysms in an adult patient over nine years. doi: 10.1111/jocs.12174 (J Card Surg 2013;28:677-679) [ABSTRACT FROM AUTHOR]