PDF(Pubmed)
Abstract：
UNASSIGNED: Solitary fibrous tumor (SFT) represents a fibroblastic neoplasm exhibiting NAB2::STAT6 gene rearrangement, displaying diverse clinical manifestations, spanning from benign to malignant. To predict prognosis, the modified (four-variable) Demicco (mDemicco) model was introduced. This investigation aims to authenticate the mDemicco risk model\'s precision in Asian patients while investigating the clinicopathological and molecular factors linked to the prognosis of extrameningeal SFTs.
UNASSIGNED: Clinicopathological data from 111 extrameningeal SFT cases in East China, covering the period from 2010 to 2020, were thoroughly analyzed. The tumors were classified using the mDemicco model. Immunohistochemical evaluation of P16 and P53, molecular detection of TP53 and TERT promoter mutation, and fluorescence in situ hybridization for CDKN2A gene alterations were performed. Statistical methods were utilized to assess the associations between clinicopathological or molecular factors and prognosis.
UNASSIGNED: Histologically, only one parameter, the mitotic count, exhibited a statistical correlation with progression-free survival (PFS) and overall survival (OS). During the Kaplan-Meier analysis, the variation in PFS among the different risk groups exhibited a notable trend towards statistical significance. Nevertheless, 3 out of 74 patients classified as low-risk SFTs and 7 out of 21 patients classified as intermediate-risk exhibited disease progression. Among the 5 patients with TP53 mutations and/or mutant-type P53 immunophenotype, 3 experienced disease progression, including 2 intermediate-risk patients. Additionally, among the 4 patients with TERT promoter mutations who were followed up, 3 showed progression, including 2 intermediate-risk patients. Moreover, it was observed that hemizygous loss of CDKN2A was detected in more than 30% of one case, yet the patient exhibited a favorable survival outcome.
UNASSIGNED: The mDemicco risk model exhibits certain limitations when dealing with smaller tumor sizes, younger age groups, and occurrences of malignant and dedifferentiated SFTs. Furthermore, molecular factors, such as TP53 or TERT promoter mutations, may identify intermediate-risk SFTs with poorer prognoses.
UNASSIGNED: Clinicopathological data from 111 extrameningeal SFT cases in East China, covering the period from 2010 to 2020, were thoroughly analyzed. The tumors were classified using the mDemicco model. Immunohistochemical evaluation of P16 and P53, molecular detection of TP53 and TERT promoter mutation, and fluorescence in situ hybridization for CDKN2A gene alterations were performed. Statistical methods were utilized to assess the associations between clinicopathological or molecular factors and prognosis.
UNASSIGNED: Histologically, only one parameter, the mitotic count, exhibited a statistical correlation with progression-free survival (PFS) and overall survival (OS). During the Kaplan-Meier analysis, the variation in PFS among the different risk groups exhibited a notable trend towards statistical significance. Nevertheless, 3 out of 74 patients classified as low-risk SFTs and 7 out of 21 patients classified as intermediate-risk exhibited disease progression. Among the 5 patients with TP53 mutations and/or mutant-type P53 immunophenotype, 3 experienced disease progression, including 2 intermediate-risk patients. Additionally, among the 4 patients with TERT promoter mutations who were followed up, 3 showed progression, including 2 intermediate-risk patients. Moreover, it was observed that hemizygous loss of CDKN2A was detected in more than 30% of one case, yet the patient exhibited a favorable survival outcome.
UNASSIGNED: The mDemicco risk model exhibits certain limitations when dealing with smaller tumor sizes, younger age groups, and occurrences of malignant and dedifferentiated SFTs. Furthermore, molecular factors, such as TP53 or TERT promoter mutations, may identify intermediate-risk SFTs with poorer prognoses.
摘要：
■孤立性纤维瘤（SFT）代表表现出NAB2::STAT6基因重排的成纤维细胞肿瘤，表现出多样化的临床表现，从良性到恶性。为了预测预后，引入了改进的(四变量)Demicco(mDemicco)模型。这项调查旨在验证mDemicco风险模型在亚洲患者中的准确性，同时调查与预后外SFT相关的临床病理和分子因素。
■华东地区111例脑外SFT病例的临床病理资料，涵盖2010年至2020年期间，进行了全面分析。使用mDemicco模型对肿瘤进行分类。P16和P53的免疫组织化学评估，TP53和TERT启动子突变的分子检测，并对CDKN2A基因改变进行荧光原位杂交。统计方法用于评估临床病理或分子因素与预后之间的关联。
■组织学，只有一个参数，有丝分裂计数，与无进展生存期（PFS）和总生存期（OS）具有统计学相关性。在Kaplan-Meier分析期间，不同风险组之间的PFS差异具有显著的统计学意义.然而，被分类为低风险SFT的74例患者中有3例，被分类为中等风险的21例患者中有7例表现出疾病进展。在5例TP53突变和/或突变型P53免疫表型患者中，3经历了疾病进展，包括2名中危患者。此外,在随访的4例TERT启动子突变患者中，3显示进展，包括2名中危患者。此外，据观察，CDKN2A的半合子丢失在一例中检测到超过30%，然而，患者表现出良好的生存结果。
■mDemicco风险模型在处理较小的肿瘤时表现出一定的局限性，年轻的年龄组，以及恶性和去分化SFT的发生。此外，分子因素，如TP53或TERT启动子突变，可以识别预后较差的中等风险SFT。
■华东地区111例脑外SFT病例的临床病理资料，涵盖2010年至2020年期间，进行了全面分析。使用mDemicco模型对肿瘤进行分类。P16和P53的免疫组织化学评估，TP53和TERT启动子突变的分子检测，并对CDKN2A基因改变进行荧光原位杂交。统计方法用于评估临床病理或分子因素与预后之间的关联。
■组织学，只有一个参数，有丝分裂计数，与无进展生存期（PFS）和总生存期（OS）具有统计学相关性。在Kaplan-Meier分析期间，不同风险组之间的PFS差异具有显著的统计学意义.然而，被分类为低风险SFT的74例患者中有3例，被分类为中等风险的21例患者中有7例表现出疾病进展。在5例TP53突变和/或突变型P53免疫表型患者中，3经历了疾病进展，包括2名中危患者。此外,在随访的4例TERT启动子突变患者中，3显示进展，包括2名中危患者。此外，据观察，CDKN2A的半合子丢失在一例中检测到超过30%，然而，患者表现出良好的生存结果。
■mDemicco风险模型在处理较小的肿瘤时表现出一定的局限性，年轻的年龄组，以及恶性和去分化SFT的发生。此外，分子因素，如TP53或TERT启动子突变，可以识别预后较差的中等风险SFT。
