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Background & aimsHepatoblastoma (HB) and hepatocellular carcinoma (HCC) are the predominant liver cancers in children, though their respective treatment options and associated outcomes differ dramatically. Risk stratification using a combination of clinical, histological, and molecular parameters can improve treatment selection, but it is particularly challenging for tumors with mixed histological features, including those in the recently created hepatocellular neoplasm not otherwise specified (HCN NOS) provisional category. We aimed to perform the first molecular characterization of clinically annotated cases of HCN NOS.MethodsWe tested whether these histological features are associated with genetic alterations, cancer gene dysregulation, and outcomes. Namely, we compared the molecular features of HCN NOS, including copy number alterations, mutations, and gene expression profiles, with those in other pediatric hepatocellular neoplasms, including HBs and HCCs, as well as HBs demonstrating focal atypia or pleomorphism (HB FPAs), and HBs diagnosed in older children (>8).ResultsMolecular profiles of HCN NOS and HB FPAs revealed common underlying biological features that were previously observed in HCCs. Consequently, we designated these tumor types collectively as HBs with HCC features (HBCs). These tumors were associated with high mutation rates (∼3 somatic mutations/Mb) and were enriched with mutations and alterations in key cancer genes and pathways. In addition, recurrent large-scale chromosomal gains, including gains of chromosomal arms 2q (80%), 6p (70%), and 20p (70%), were observed. Overall, HBCs were associated with poor clinical outcomes.ConclusionsOur study indicates that histological features seen in HBCs are associated with combined molecular features of HB and HCC, that HBCs are associated with poor outcomes irrespective of patient age, and that transplanted patients are more likely to have good outcomes than those treated with chemotherapy and surgery alone. These findings highlight the importance of molecular testing and early therapeutic intervention for aggressive childhood hepatocellular neoplasms.Lay summaryWe molecularly characterized a class of histologically aggressive childhood liver cancers and showed that these tumors are clinically aggressive and that their observed histological features are associated with underlying recurrent molecular features. We proposed a diagnostic algorithm to identify these cancers using a combination of histological and molecular features, and our analysis suggested that these cancers may benefit from specialized treatment strategies that may differ from treatment guidelines for other childhood liver cancers.

The oil and gas industry remains critical to the global economy, as it contributes to the provision of energy and raw materials. Nonetheless, this sector continued to face clear challenges in operational effectiveness, risk and security. Regular tracking methods are limited to latency issues; they are not secure, and data may face integrity issues. To this end, this paper lays out an efficient fog-enabled private blockchain-based identity authentication approach for oil and gas field monitoring. By integrating IoT devices to blockchain,, decentralized control systems are created that enhance security, transparency, and efficient execution of transactions. In this scheme, by making full use of the decentralized structure of blockchain technology and applying the computational power of fog nodes, a secure and efficient identity authentication framework is designed. Fog nodes are an intermediary between IoT devices and blockchain technology, providing lower latency in communication, and therefore more efficient. The main contributions of this paper include: developing a decentralized authentication system based on private blockchains and fog nodes to overcome the drawbacks of centralized models. Create a network model using a private blockchain that dramatically improves feasibility by incorporating strict admission and authorization procedures. Hence, this leads to simultaneous registrations with minimal network time consensus Authentication that incorporating fuzzy extractor to connect the privacy-centric approach and to improve the security analysis and performance evaluation proving that the proposed solution provides better. According to the previous security analysis, it is clear that the scheme conflicts with different types of threats including DoS, MITM attacks, replay, Sybil, and message substitution attacks. The performance evaluation also shows low computational and communication costs, high compatibility, and real-time operation, which indicates that the proposed scheme is effective and can be implemented as a real-time oil and gas field monitoring system. [ABSTRACT FROM AUTHOR]

Context.--Biliary atresia (BA) patients can have portal vein (PV) abnormalities. Objective.--To investigate the explant pathology of BA patients transplanted in adulthood with a focus on portal venous abnormalities. Design.--Adult BA liver explants were reviewed, along with prior biopsies, Kasai portoenterostomy (KP), and relevant medical records. Results.--Three explants were identified; all patients were female, with age at diagnosis, KP, and liver transplantation (LT) as follows: (1) less than 1 week, 8 days, and 25 years; (2) 15 weeks, 16 weeks, and 32 years; and (3) 7 weeks, 8 weeks, and 33 years, respectively, with normalization of conjugated bilirubin within 6 months of KP and development of portal hypertension (PHTN) within 3 years of KP for all 3. The first 2 had recurrent cholangitis. Duration of pre-LT PHTN was 22, 29, and 30 years, and that of pre-LT cholangitis was 9, 3, and 0 years, respectively. All 3 explants showed hilar and extrahepatic fibromyxoid intimal hyperplasia of the PV with parenchymal hepatoportal sclerosis. Cholestasis was limited to those with a history of cholangitis. Patient 3, without cholangitis, showed delicate septal fibrosis with peripheral accentuation without biliary cirrhosis. Conclusions.--In the context of a functioning KP, cholestasis and biliary cirrhosis are likely related to recurrent cholangitis, which may or may not occur after KP. In the absence of biliary cirrhosis, PHTN may be secondary to obliterative venopathy. Adult BA explants should be sampled thoroughly, with a focus on hilar/ perihilar connective tissue to include PV branches. Explants may not show biliary cirrhosis and should be reported with appropriate clinicopathologic correlation. [ABSTRACT FROM AUTHOR]

Electronic Healthcare Record (EHR) has been actively promoted recently. Healthcare records and paperbased medical prescriptions are scattered and unorganized in medical institutions and clinics, thereby prompting many efforts to advance the EHR from traditional clinical settings to an efficient electronic medical model to regulate health records appropriately and accurately. With the development of technology and data exchange over the Internet or local networks, protecting transmitted data in healthcare records is necessary. This paper presents a secure EHR system utilizing robust authentication and RSA digital signature. We performed formal and informal security analyses utilizing the Scyther tool and the Canetti-Krawczyk (CK) model. Therefore, the proposed scheme has a higher level of security than the previous relevant work by resisting the dangers of well-known cyberattacks, such as impersonation, DoS, replay, man-in-themiddle (MITM), and insider attacks. Our work balances the security complexity and the communication cost, and the comparison tables show that the proposed scheme has the lowest communication cost. Moreover, we presented a secure prescription through a QR code generated using the prescription and the physician's RSA digital signature. [ABSTRACT FROM AUTHOR]

Context.--Image-guided, fine-needle aspiration-assisted core needle biopsy with an on-site evaluation by a pathologist (FNACBP) of osseous lesions is not a common practice in pediatric institutions. Objectives.--To evaluate the diagnostic adequacy and accuracy of FNACBP for pediatric osseous lesions and to compare the adequacy with procedures that do not use fine-needle aspiration. Design.--Six-year, retrospective review of 144 consecutive children biopsied for osseous lesions with and without fine-needle aspiration assistance. Results.--Pathologic diagnosis was achieved in 79% (57 of 72) of the core biopsies without an on-site evaluation, 78% (32 of 41) of the open biopsies (9 with intra-operative consultation), and 97% (30 of 31) of the FNACBPs as the initial diagnostic procedure. Three FNACBP cases were preceded by nondiagnostic open biopsies. Among 34 lesions sampled by FNACBP, 33 (97%) succeeded with diagnostic tissue, with most (30 of 33; 91%) being neoplasms, including 16 malignant (48%), 13 benign (39%), and 1 indeterminate (3%) lesions. The most-common diagnoses were osteosarcoma (9 of 33; 27%) and Langerhans cell histiocytosis (7 of 33; 21%). In cases with follow-up information available, 93% (28 of 30) of the FNACBP-rendered diagnoses were clinically useful, allowing initiation of appropriate therapy. The FNACBP procedure had 100% specificity, sensitivity, and positive predictive value for all 14 malignant lesions, with the sensitivity being 88% in benign lesions. Most FNACBP procedures (32 of 34; 94%) yielded adequate material for ancillary testing. A gradual upward trend was observed for the choice of FNACBP as an initial diagnostic procedure for osseous lesions. Conclusions.--The FNACBP procedure yields sufficient material for diagnosis and ancillary studies in pediatric, osseous lesions and may be considered an initial-diagnostic procedure of choice. (Arch Pathol Lab Med. 2017;141:678-683; doi: 10.5858/arpa.2016-0269-OA), No unified, optimal biopsy technique for the diagnosis of osseous lesions has been established, although incisional biopsy is considered the gold standard. Imaging-guided core needle biopsy has become a routine [...]

Context.--The World Health Organization has recently recognized lymphoepithelioma-like carcinoma, or inflammatory hepatocellular carcinoma, as a variant of hepatocellular carcinoma. Objective.--To identify and characterize the inflammatory hepatocellular carcinomas in our institution from 1988 to the present. Design.--All cases of hepatocellular carcinoma in our institution from 1988 to the present were reviewed and reclassified as lymphoepithelioma-like carcinoma and were studied in comparison to appropriately matched controls. Results.--Among the 8 cases of lymphoepithelioma-like carcinoma identified, the male to female ratio was 1:3, the mean age was 68.5 years (range, 57-78 years), and all of the cases were seen in noncirrhotic livers. The average numbers of lymphocytes were significantly higher in the cases than in the controls. T cells were predominant, with a uniform distribution of CD4 and CD8 positive cells. Cholangiolar differentiation was seen by K19 positivity as focal in 1 case and diffuse in 2 cases. In situ hybridization for Epstein-Barr virus was negative in all of the cases. Diffuse overexpression of p16 (>75% of cells) was seen in 2 cases, both of which were negative for the presence of transcriptionally active human papilloma virus by in situ hybridization. In our series, 3 of 8 cases (37.5%) showed local recurrence, which was similar to the controls (6 of 18; 33%), P > .99. Although the rate of distant metastases was lower among the cases (12.5%) than the controls (22.2%), the difference was not statistically significant (P > .99). Conclusion.--We present the first series of 8 cases of lymphoepithelioma-like carcinoma of the liver occurring in patients without cirrhosis and with a female preponderance and the absence of Epstein-Barr virus. Although clinical outcomes were similar to those of controls in our small series, additional data may be required for confirmation. (Arch Pathol Lab Med. 2014;138:1193-1202; doi: 10.5858/arpa.2013-0371-OA), Hepatocellular carcinoma (HCC) is the fifth most common cancer worldwide. (1) The incidence is increasing because of the increasing prevalence of chronic liver disease, both viral and metabolic. (2) Uncommonly, [...]

Mucosal prolapse syndrome most commonly involves the rectum and presents as solitary rectal ulcer syndrome and proctitis cystica profunda. Symptoms and endoscopic appearances are nonspecific. Histologically, mucosal prolapse is characterized by fibromuscular obliteration of the lamina propria, and displacement of crypts into submucosa and muscularis mucosae. Mucosal prolapse presenting as polyposis is rare and has only been reported involving the rectosigmoid colon. In this report, we describe a case of mucosal prolapse syndrome presenting as diffuse polyposis and colitis cystica profunda involving the hepatic, splenic flexures and descending colon in a teenage boy suffering from refractory fibrostenosing Crohn's disease. This patient was found to have possibly deleterious homozygous single nucleotide polymorphisms in both SULT1A1 and SULT1A2 genes within a unique polygenic variation of altered cell adhesion., (© 2021 by the Association of Clinical Scientists, Inc.)

► Lynch syndrome involves many rare cancers, such as urothelial carcinoma. ► The case describes incidental urothelial carcinoma associated with Lynch syndrome. ► Clinical suspicion of rare cancers must be maintained in certain patients.