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Ref Type Journal Article
PMID (39392364)
Authors Arregui M, Calles A, Galera MDM, Gutiérrez A, López-Jiménez C, Agra C, Fernández A, Gutiérrez N, Toro M, Álvarez R
Title Complete response to encorafenib plus binimetinib in a BRAF V600E-mutant metastasic malignant glomus tumor.
Journal Vol Issue Date Pages Journal Short Title
Oncotarget 15 2024 Oct 11 717-724 Oncotarget
URL
Abstract Text 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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Molecular Profile Treatment Approach
Gene Name Source Synonyms Protein Domains Gene Description Gene Role
Therapy Name Drugs Efficacy Evidence Clinical Trials
Drug Name Trade Name Synonyms Drug Classes Drug Description
Gene Variant Impact Protein Effect Variant Description Associated with drug Resistance
Molecular Profile Indication/Tumor Type Response Type Therapy Name Approval Status Evidence Type Efficacy Evidence References
BRAF V600E glomus tumor predicted - sensitive Binimetinib + Encorafenib Case Reports/Case Series Actionable In a clinical case study, treatment with the combination of Mektovi (binimetinib) and Braftovi (encorafenib) resulted in a complete response lasting more than 2 years in a patient with metastatic malignant glomus tumor harboring BRAF V600E (PMID: 39392364). 39392364