image: Figure 2: Morphological and genetic findings.
(A, B) Representative histological images, H&E, ×50 (A) and ×200 (B), showing short fascicles of spindle-shaped tumor cells with large ovoid nuclei, fibrous stroma with diffuse focal myxomatosis, small adipose clusters and signs of perivascular lymphoid infiltration. (C, D) Two closely spaced missense mutations identified in BRAF exon 15: (C) Schematic representation of B-Raf protein structure (SwissModel) with amino acid substitutions at positions 600 and 607 of the polypeptide shown in red and orange, respectively; (D) an IGV screenshot demonstrating BRAF mutations c.1799T>A p.V600E and c.1819T>A; p.S607T in cis-position.
Credit: 2024 Sinichenkova et al.
“This is the first report of spindle cell sarcoma BRAF V600E/S607T double-mutated, responding to a combination of B-Raf and MEK inhibitors.”
BUFFALO, NY- August 14, 2024 – A new case report was published in Oncotarget's Volume 15 on July 17, 2024, entitled, “Rapid but nondurable response of a BRAF exon 15 double-mutated spindle cell sarcoma to a combination of BRAF and MEK inhibitors.”
As noted in the introduction of the Abstract, the BRAF V600E substitution predicts a cancer's sensitivity to BRAF inhibitor therapy, though the mutation is rarely found in soft-tissue sarcomas.
Researchers Kseniya Sinichenkova, Iliya Sidorov, Nataliya Kriventsova, Dmitriy Konovalov, Ruslan Abasov, Nataliya Usman, Alexander Karachunskiy, Galina Novichkova, Dmitriy Litvinov, and Alexander Druy from the Dmitriy Rogachev National Medical Research Center of Pediatric Hematology, Oncology, Immunology (Ministry of Healthcare of Russian Federation) and the Research Institute of Medical Cell Technologies in Yekaterinburg, Russia, describe a case of undifferentiated spindle cell sarcoma that exhibited primary insensitivity to standard chemotherapy and a pronounced but non-sustained response to BRAF/MEK inhibitors at recurrence.
The case presentation involved a 13-year-old girl that was diagnosed with low-grade spindle cell sarcoma of pelvic localization, BRAF exon 15 double-mutated: c.1799T>A p.V600E and c.1819T>A p.S607T in cis-position.
“This is the first report of spindle cell sarcoma BRAF V600E/S607T double-mutated, responding to a combination of B-Raf and MEK inhibitors.”
Continue reading: DOI: https://doi.org/10.18632/oncotarget.28606
Correspondence to: Kseniya Sinichenkova - ksinichenkova@gmail.com
Keywords: undifferentiated sarcoma, BRAF V600E mutation, low grade spindle cell sarcoma, abdominal cocoon
Click here to sign up for free Altmetric alerts about this article.
About Oncotarget:
Oncotarget (a primarily oncology-focused, peer-reviewed, open access journal) aims to maximize research impact through insightful peer-review; eliminate borders between specialties by linking different fields of oncology, cancer research and biomedical sciences; and foster application of basic and clinical science.
Oncotarget is indexed and archived by PubMed/Medline, PubMed Central, Scopus, EMBASE, META (Chan Zuckerberg Initiative) (2018-2022), and Dimensions (Digital Science).
To learn more about Oncotarget, visit Oncotarget.com and connect with us on social media:
X
Facebook
YouTube
Instagram
LinkedIn
Pinterest
Spotify, and available wherever you listen to podcasts
Click here to subscribe to Oncotarget publication updates.
For media inquiries, please contact media@impactjournals.com.
Oncotarget Journal Office
6666 East Quaker St., Suite 1
Orchard Park, NY 14127
Phone: 1-800-922-0957 (option 2)
Journal
Oncotarget
Method of Research
Case study
Subject of Research
People
Article Title
Rapid but nondurable response of a BRAF exon 15 double-mutated spindle cell sarcoma to a combination of BRAF and MEK inhibitors
Article Publication Date
17-Jul-2024
COI Statement
The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.