image: Figure 1: Chest MRI 04/22/2021. Soft tissue tumor (32 × 23 mm) on the right chest wall, located deep to the superficial fascia, in relation to the serratus muscle and extending towards the intercostal musculature, superficial to the costal arches. Small subcutaneous tumors, the largest measuring 8 mm in diameter, on the thoracic and abdominal wall.
Credit: 2024 Arregui et al.
“This case report highlights the importance of full tumor genotyping to identify potentially actionable targets in rare tumors such as malignant glomus tumors.”
BUFFALO, NY- October 29, 2024 – A new case report was published in Oncotarget's Volume 15 on October 11, 2024, entitled “Complete response to encorafenib plus binimetinib in a BRAF V600E-mutant metastasic malignant glomus tumor.”
As highlighted in the abstract, glomus tumors (GT) are rare mesenchymal neoplasms originating in dermal arteriovenous structures involved in thermoregulation. While generally benign, some can exhibit malignant features, leading to aggressive behavior, metastasis, and limited response to standard chemotherapy. The identification of the BRAF V600E mutation in certain malignant GT cases offers a promising therapeutic target.
In their paper, researchers 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 from Gregorio Marañón University Hospital and Fundación Jiménez Díaz University Hospital in Madrid, Spain, document a remarkable clinical and metabolic response in a case of metastatic BRAF V600E-mutated glomangiosarcoma treated with the combination of encorafenib and binimetinib.
They report on a 45-year-old male patient with stage IV malignant GT carrying a BRAF V600E mutation, who was treated systemically with encorafenib and binimetinib. This approach led to a swift clinical and radiological improvement.
“To our knowledge, our patient represents the first reported case of a metastatic malignant GT successfully treated with BRAF and MEK inhibitors, achieving a long-lasting complete morpho-metabolic response.”
Continue reading: DOI: https://doi.org/10.18632/oncotarget.28654
Correspondence to: Carlos López-Jiménez - clopezjimenez@atbsarc.org
Video short: https://www.youtube.com/watch?v=xjbj3Iu16P4
Keywords: cancer, malignant glomus tumor, glomangiosarcoma, BRAF V600E, agnostic treatment, targeted therapy
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Journal
Oncotarget
Method of Research
Case study
Subject of Research
People
Article Title
Complete response to encorafenib plus binimetinib in a BRAF V600E-mutant metastasic malignant glomus tumor
Article Publication Date
11-Oct-2024
COI Statement
Authors have no conflicts of interest to declare.