Department of Medical Oncology, Gregorio Marañón University Hospital, Madrid 28007, Spain.
Department of Medical Oncology, Fundación Jiménez Díaz University Hospital, Madrid 28040, Spain.
Oncotarget. 2024 Oct 11;15:717-724. doi: 10.18632/oncotarget.28654.
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.
血管球瘤（GT）是非常罕见的间叶性肿瘤，起源于血管球，这是一种位于真皮中的动静脉结构，参与体温调节。虽然大多数是良性的，但它们偶尔可能表现出恶性组织学特征，与侵袭性临床行为、转移扩散以及对常规化疗的不良反应相关。BRAF V600E 突变已在一部分恶性 GT 中被鉴定出来，这突显了一个有前途的治疗靶点。在这里，我们报告了一例转移性 BRAF V600E 突变性血管球肉瘤患者在接受 encorafenib 和 binimetinib 治疗后的显著临床和形态代谢反应。
