Complete response to encorafenib plus binimetinib in a BRAF V600E-mutant metastasic malignant glomus tumor.

Q2 Medicine Oncotarget Pub Date : 2024-10-11 DOI:10.18632/oncotarget.28654
Marta Arregui, Antonio Calles, María Del Mar Galera, Ana Gutiérrez, Carlos López-Jiménez, Carolina Agra, Adriana Fernández, Natalia Gutiérrez, María de Toro, Rosa Álvarez
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Abstract

Glomus tumors (GT) are very rare mesenchymal neoplasms arising from glomus bodies, arteriovenous structures located in the dermis and involved in thermoregulation. Although most are benign, they may occasionally present malignant histological features associated with aggressive clinical behavior, metastatic spread, and poor response to conventional chemotherapy. The BRAF V600E mutation has been identified in a subset of malignant GT, highlighting a promising therapeutic target. Here, we report the impressive clinical and morpho-metabolic response of a metastatic BRAF V600E-mutated glomangiosarcoma after treatment with encorafenib and binimetinib.

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BRAF V600E突变转移性恶性胶质瘤患者对安戈非尼加比尼替尼治疗的完全应答。
胶状体瘤(GT)是一种非常罕见的间叶肿瘤,产生于位于真皮层并参与体温调节的动静脉结构--胶状体。虽然大多数是良性的,但偶尔也会出现恶性组织学特征,并伴有侵袭性临床表现、转移扩散和对常规化疗反应差等症状。在一部分恶性 GT 中发现了 BRAF V600E 基因突变,这突显了一个很有前景的治疗靶点。在此,我们报告了一例转移性 BRAF V600E 突变的糯米肉瘤在接受安戈非尼和乐替米尼治疗后令人印象深刻的临床和形态代谢反应。
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来源期刊
Oncotarget
Oncotarget Oncogenes-CELL BIOLOGY
CiteScore
6.60
自引率
0.00%
发文量
129
审稿时长
1.5 months
期刊介绍: Information not localized
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