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世界核心医学期刊文摘（儿科学分册） 2006年第4期2卷 41-41页

作者： Billups C.A. pappo a.s. s.L. spunt 刘凯 Department of Hematology/Oncology St. Jude Children's Research Hospital Memphis TN 38105- 2794 United States

Purpose: The aim of this study was to determine predictors of outcome in childhood truncal rhabdomyosarcoma. Methods: Retrospective chart review evaluating the impact of demographic features, disease characteristics, and the extent and timing of surgical intervention on outcome was performed. Results: Thirty-three patients with a median age of 8 yea.s.were identified. Most had advanced Intergroup Rhabdomyosarcoma.s.udy group III (n=13) or group IV (n=9) disease. Primary site included 20 (61% ) chest wall, 6 (21% ) paraspinal, 5 (15% ) periscapular, and 1 (3% ) abdominal wall. Most tumors were embryonal (n=21), larger than 5 cm (n=27), and locally invasive (n=13); 7 had positive nodes. Tumor size, nodal status, and gross total tumor resection (upfront or delayed) were significant predictors of event-free and overall survival. Tumors 5 cm or smaller were amenable to upfront surgical resection (P= .007). In patients with tumors larger than 5 cm, resection at any time was a.s.ciated with a 10- year overall survival 57% ± 13% compared with 8% ± 5% in those who had no surgery (P= .003). Tumor recurrence was local in 44% of cases, and survival after local recurrence was rare (1 of 8). Conclusion: Tumor size, nodal status, and gross total resection at any time are significant predictors of outcome in truncal rhabdomyosarcoma. Gross tumor excision should be the goal of surgical intervention in this disease.

关键词：横纹肌肉瘤预后预测吉德儿童研究总体生存率手术切除术原发病变研究协作组淋巴结转移体全

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Late onset fulminant Wilson's disease:A case report and review of the literature

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World Journal of Gastroenterology 2014年第46期20卷 17656-17660页

作者： Ella Weitzman Orit pappo Peretz Weiss Moshe Frydman Yael Haviv-Yadid Ziv Ben Ari Liver Disease Center Sheba Medical CenterRamat Gan 52621Israel Department of Pathology Sheba Medical CenterRamat Gan 52621Israel Institute of Genetics Sheba Medical CenterRamat Gan 52621Israel Intensive Care Unit Sheba Medical CenterRamat GanTel Aviv UniversityRamat Gan 52621Israel sackler school of Medicine Tel Aviv UniversityTel Aviv 69975Israel

Wilson’s disease(WD)is an autosomal recessive inherited disorder of hepatic copper *** can be present in different clinical conditions,with the most common ones being liver disease and neuropsychiatric *** cases present symptoms ata.s.of ***,few reports exist in the literature on patients in whom the disease presented beyond this *** this report,we present a case of late onset fulminant WD in a 58-year-old patient in whom the diagnosis was established clinically,by genetic analysis of the ATP7B gene disclosing rare mutations(G1099s and c.1707+3ins T)as well as by high hepatic copper *** a.s. reviewed the relevant *** diagnosis of WD with late onset presentation is easily *** diagnostic features and the geneticbackground in patients with late onset WD are not different from those in patients with early onset WD,except for the *** treatments for this disorder that can be fatal are available and will prevent or reverse many manifestations if the disease is discovered early.

关键词： Wilson's disease Late onset Fulminant ATP7B gene mutations Copper

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Cisplatin versus cisplatin plus doxorubicin for standard-risk hepatoblastoma.

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中国肿瘤临床 2010年第2期37卷 125-125页

作者： Alberto pappo Texas Chidren’s Cancer Center United States of America Oncology

This study shows that over 90% of children withnon-metastatic hepatoblastoma confined to 3 orfewer hepatic sectors can be cured with single-agentcisplatin chemotherapy.

关键词： Cisplatin versus cisplatin plus doxorubicin for standard-risk hepatoblastoma COG

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