Oncotarget

Case Reports:

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

Marta Arregui, Antonio Calles, María del Mar Galera, Ana Gutiérrez, Carlos López-Jiménez _, Carolina Agra, Adriana Fernández, Natalia Gutiérrez, María de Toro and Rosa Álvarez

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Oncotarget. 2024; 15:717-724. https://doi.org/10.18632/oncotarget.28654

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Abstract

Marta Arregui1, Antonio Calles1, María del Mar Galera1, Ana Gutiérrez1, Carlos López-Jiménez2, Carolina Agra3, Adriana Fernández4, Natalia Gutiérrez1, María de Toro1 and Rosa Álvarez1

1 Department of Medical Oncology, Gregorio Marañón University Hospital, Madrid 28007, Spain

2 Department of Medical Oncology, Fundación Jiménez Díaz University Hospital, Madrid 28040, Spain

3 Department of Pathology, Gregorio Marañón University Hospital, Madrid 28007, Spain

4 Department of Radiology, Gregorio Marañón University Hospital, Madrid 28007, Spain

Correspondence to:

Carlos López-Jiménez, email: [email protected]

Keywords: malignant glomus tumor; glomangiosarcoma; BRAF V600E; agnostic treatment; targeted therapy

Received: May 06, 2024     Accepted: September 17, 2024     Published: October 11, 2024

Copyright: © 2024 Arregui et al. This is an open access article distributed under the terms of the Creative Commons Attribution License (CC BY 4.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.

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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