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A novel sclerosing atypical lipomatous tumor/well-differentiated liposarcoma in a 7-year-old girl: report of a case with molecular confirmation

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机构: [1]Sichuan Univ, West China Hosp, Dept Pathol, Guoxuexiang 37, Chengdu 610041, Sichuan, Peoples R China [2]Kunming Univ Sci & Technol, Affiliated Hosp, Peoples Hosp Yunnan Prov 1, Dept Pathol, Kunming 650032, Yunnan, Peoples R China
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关键词: Atypical lipomatous tumor/well-differentiated liposarcoma Dedifferentiated liposarcoma Pediatric sarcoma 12q13-15 amplification Molecular analysis

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Atypical lipomatous tumor (ALT)/well-differentiated liposarcoma (WDL)/dedifferentiated liposarcoma (DDL) is a common type of liposarcoma in late adulthood. However, pediatric ALT/WDL/DDL is extremely rare, and only 3 cases have been described in children younger than 10 years of age. Notably, none of these cases harbored MDM2 gene amplification. Here, we reported a sclerosing ALT/WDL in a 7-year-old Chinese girl. Histologically, in most areas, the neoplastic cells were embedded within the collagenous background, and typical lipogenic areas were inconspicuous throughout the sclerotic areas. In addition, scattered small foci of atypical osseous/chondrous elements were identified. Notably, a small typical lipoma-like ALT/WDL area was detected in the periphery of the mass. Immunohistochemically, all the neoplastic components demonstrated positivity for MDM2, CDK4, and p16. Fluorescence in situ hybridization revealed MDM2 gene amplification in all the tumor components. To the best of our knowledge, this is the first example of MDM2-amplified ALT/WDL in this age group. (C) 2017 Elsevier Inc. All rights reserved.

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出版当年[2018]版:
大类 | 3 区 医学
小类 | 3 区 病理学
最新[2023]版:
大类 | 2 区 医学
小类 | 2 区 病理学
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出版当年[2017]版:
Q1 PATHOLOGY
最新[2023]版:
Q2 PATHOLOGY

影响因子: 最新[2023版] 最新五年平均 出版当年[2017版] 出版当年五年平均 出版前一年[2016版] 出版后一年[2018版]

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第一作者机构: [1]Sichuan Univ, West China Hosp, Dept Pathol, Guoxuexiang 37, Chengdu 610041, Sichuan, Peoples R China
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通讯机构: [1]Sichuan Univ, West China Hosp, Dept Pathol, Guoxuexiang 37, Chengdu 610041, Sichuan, Peoples R China [*1]Department of Pathology, West China Hospital, Sichuan University, Guoxuexiang 37, 610041, Chengdu, Sichuan, China
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