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Hepatocellular adenoma in the paediatric population:Molecular classification and clinical associations

World Journal of Gastroenterology 2020年第19期26卷 2294-2304页

作者： Elan Hahn juan putra Division of Pathology Department of Paediatric Laboratory Medicinethe Hospital for Sick ChildrenToronto M5G 1X8ONCanada Department of Laboratory Medicine and Pathobiology University of TorontoToronto M5S 1A8ONCanada

Hepatocellular adenomas(HCAs)represent rare,benign liver tumours occurring predominantly in females taking oral *** children,HCAs comprise less than 5%of hepatic tumours and demonstrate association with various *** contemporary classification of HCAs,based on their distinctive genotypes and clinical phenotypes,includes hepatocyte nuclear factor 1 homeobox alpha-inactivated HCAs,beta-catenin-mutated HCAs,inflammatory HCAs,combined beta-catenin-mutated and inflammatory HCAs,sonic hedgehogactivated HCAs,and unclassified *** children,there is a lack of literature on the characteristics and distribution of HCA *** this review,we summarized different HCA subtypes and the clinicopathologic spectrum of HCAs in the paediatric population.

关键词： Paediatric Hepatocellular adenoma Malignant transformation Beta-catenin HNF1A Glycogen storage disorders

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Upper gastrointestinal tract involvement of pediatric inflammatory bowel disease: A pathological review

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World Journal of Gastroenterology 2019年第16期25卷 1928-1935页

作者： Dua Abuquteish juan putra Division of Pathology Department of Paediatric Laboratory Medicine Hospital for Sick Children

Upper gastrointestinal(UGI) tract involvement of inflammatory bowel disease(IBD) is commonly seen in pediatric patients. Upper endoscopy is included in the routine workup of children with suspected IBD to enhance the diagnosis and management of these patients. Currently, childhood IBD is classified into ulcerative colitis(UC), atypical UC, Crohn's disease(CD) and IBD *** confirmation of UGI tract involvement, in particular the presence of epithelioid(non-caseating) granulomas, is helpful in confirming the diagnosis of IBD and its classification. Herein, we reviewed selected IBD-associated UGI tract manifestations in children. Lymphocytic esophagitis, seen predominantly in CD,is histologically characterized by increased intraepithelial lymphocytes(> 20 in one high-power field) in a background of mucosal injury with absence of granulocytes. Focally enhanced gastritis is a form of gastric inflammation in pediatric IBD marked by a focal lymphohistiocytic pit inflammation with or without granulocytes and plasma cells in a relatively normal background gastric mucosa. Duodenal inflammation seen in children with IBD includes cryptitis,villous flattening, increased intraepithelial lymphocytes, and lamina propria eosinophilia. Finally, epithelioid granulomas not associated with ruptured gland/crypt are a diagnostic feature of CD. The clinicopathologic correlation and differential diagnosis of each microscopic finding are discussed. Clinicians and pathologists should be cognizant of the utility and limitations of these histologic features.

关键词： Pediatric Inflammatory bowel disease Lymphocytic esophagitis Focally enhanced gastritis Epithelioid granuloma Crohn’s disease Ulcerative colitis

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Primary malignant vascular tumors of the liver in children:Angiosarcoma and epithelioid hemangioendothelioma

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World Journal of Gastrointestinal Oncology 2021年第4期13卷 223-230页

作者： Sami Bannoura juan putra Department of Paediatric Laboratory Medicine Hospital for Sick Children555 University AveToronto M5G 1X8OntarioCanada

Primary malignant vascular neoplasms of the liver,angiosarcoma and epithelioid hemangioendothelioma,are extremely rare entities in the pediatric *** Society for the Study of Vascular Anomalies classification system is recommended for the pathologic diagnosis of hepatic vascular lesions in this age *** this article,we highlight the clinicopathologic characteristics of hepatic angiosarcoma and epithelioid hemangioendothelioma in the pediatric *** angiosarcoma in children shows a slight female predominance with an average age of 40 mo at *** distinct histologic features include whorls of atypical spindled cells and eosinophilic globules,in addition to the general findings of *** diagnosis of pediatric hepatic angiosarcoma is not always straightforward,and the diagnostic challenges are discussed in the *** epithelioid hemangioendothelioma also demonstrates a female predominance,but is more commonly identified in adolescents(median age at diagnosis:12 years).Histologically,the lesion is characterized by epithelioid cells and occasional intracytoplasmic lumina with a background of fibromyxoid *** WWTR1-CAMTA1 and YAP1-TFE3 fusions have been associated with epithelioid hemangioendothelioma,there are currently no known signature genetic alterations seen in pediatric hepatic *** in molecular pathology,particularly for pediatric hepatic angiosarcoma,is necessary for a better understanding of the disease biology,diagnosis,and development of targeted therapies.

关键词： Pediatric Hepatic angiosarcoma Epithelioid hemangioendothelioma Infantile hemangioma Liver tumor Molecular genetics

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X染色体倒位伴X连锁无汗性外胚叶发育不良一个家系的产前诊断

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中华医学杂志 2005年第26期85卷 1845-1848页

作者：石慧娟方群王连唐中山大学病理学教研室广州510080 中山大学附属第一医院妇产科胎儿医学中心

目的探讨对X连锁无汗性外胚叶发育不良(EDA)家系的EDA患胎进行产前诊断的可能性,为建立对该病的产前诊断及遗传咨询提供依据。方法应用染色体核型分析、系谱分析法检测了X连锁EDA1个家系的染色体畸变,结合胎儿脐血染色体核型分析、胎儿... 详细信息

目的探讨对X连锁无汗性外胚叶发育不良(EDA)家系的EDA患胎进行产前诊断的可能性,为建立对该病的产前诊断及遗传咨询提供依据。方法应用染色体核型分析、系谱分析法检测了X连锁EDA1个家系的染色体畸变,结合胎儿脐血染色体核型分析、胎儿尸检、胎儿下颌骨X线摄片,组织病理学分析及皮肤免疫组织化学细胞角蛋白(CK)、上皮膜抗原(EMA)检测对可疑EDA患胎行产前诊断及生后鉴定。结果EDA家系中先证者和携带者染色体核型分析显示,1条X染色体出现臂间倒位[inv(X)(p22q13)],胎儿脐血染色体核型与先证者相同。胎儿尸检及组织病理学分析显示表皮发育不良,汗腺、毛囊、皮脂腺缺如,符合无汗性外胚叶发育不良,面、胸、上腹、大腿部真皮组织CK呈阴性,EMA呈阴性,其余大体和镜下病理检查无特殊。结论该家系患者1条X染色体臂间倒位,[inv(X)(p22q13)]的断裂点位于致病基因区域内,可能是导致EDA发病的原因。

关键词： X连锁无汗性外胚叶发育不良产前诊断染色体倒位家系脐血染色体核型分析染色体臂间倒位免疫组织化学表皮发育不良染色体畸变系谱分析法细胞角蛋白组织病理学上皮膜抗原病理学分析 EDA 遗传咨询方法应用 X线摄片

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