Open Access

CASE REPORT

BRAF^V600E Metastatic Synovial Sarcoma Treated with BRAF & MEK Inhibitors Achieves Complete Response. A Case Report & Literature Review

Daniel Burg¹, Aryeh Babkoff¹, Omer Or², Noam Olshinka², Jonathan Abraham Demma³, Mohamad Adila^1,3, Marc Wygoda⁴, Philip Blumenfeld⁴, Judith Diment⁵, Masha Galiner⁶, Yusef Azraq⁶, Daniela Katz^1,7, Petachia Reissman⁸, Sadie Ostrowicki⁹, Gabriella Sebbag¹⁰, Narmine Elkhateeb^1,11, Anat Hershko Moshe^1,11, Dania Jaber^1,11, Adi Hollander^1,11, Limor Rubin^1,11, Aviad Zick^1,12,*

1 Faculty of Medicine, The Hebrew University of Jerusalem, Jerusalem, Israel
2 Orthopedic Department, Hadassah Medical Center and Faculty of Medicine, Hebrew University of Jerusalem, Jerusalem, Israel
3 Surgical Department, Hadassah Medical Center and Faculty of Medicine, Hebrew University of Jerusalem, Jerusalem, Israel
4 Radiotherapy Institute, Sharett Institute for Oncology, Hadassah Medical Center and Faculty of Medicine, Hebrew University of Jerusalem, Jerusalem, Israel
5 Pathology Department, Hadassah Medical Center and Faculty of Medicine, Hebrew University of Jerusalem, Jerusalem, Israel
6 Radiology Department, Hadassah Medical Center and Faculty of Medicine, Hebrew University of Jerusalem, Jerusalem, Israel
7 Helmsley Cancer Center, Shaare Zedek Medical Center, Jerusalem, Israel
8 Department of Surgery, The Hebrew University School of Medicine, Shaare Zedek Medical Center, Jerusalem, Israel
9 Department of Biology, University of Texas, Austin, TX, USA
10 Department of Molecular Biology & Biochemistry, Rutgers University, Piscataway, NJ, USA
11 Department of Medicine, Allergy and Clinical Immunology Unit, Hadassah Medical Center, Jerusalem, Israel
12 Oncology Department, Sharett Institute for Oncology, Hadassah Medical Center & Faculty of Medicine, Hebrew University of Jerusalem, Jerusalem, Israel

* Corresponding Author: Aviad Zick. Email:

(This article belongs to the Special Issue: Advances in Combined Therapy for Soft Tissue Sarcomas)

Oncology Research 2026, 34(4), 38 https://doi.org/10.32604/or.2026.070233

Received 11 July 2025; Accepted 16 October 2025; Issue published 23 March 2026

Abstract

Background: —Synovial sarcoma is a rare soft tissue sarcoma. Treatment of synovial sarcoma includes surgery, radiation, pazopanib, and chemotherapy. Targeted therapies, such as B-Raf proto-oncogene, serine/threonine kinase (BRAF) inhibitors, are emerging as a potential treatment option. We describe the sixth case of a BRAF^V600E synovial sarcoma, the first extra-thoracic case. This case is the first to show a complete pathological response to BRAF & mitogen-activated protein kinase kinase (MEK) inhibitors. Case description: —We treated a 22-year-old male with a left groin BRAF^V600E synovial sarcoma with doxorubicin, Ifosphamide & Sodium 2-Mercaptoethanesulfonate. When we identified BRAF^V600E in the tumor, the BRAF^V600E and MEK inhibitors (dabrafenib & trametinib) were initiated, followed by surgery, with a complete pathological response. Nine months after the surgery, a local recurrence prompted the resumption of dabrafenib & trametinib followed by radiotherapy, resulting in complete radiological response and the development of hemophagocytic lymphohistiocytosis treated with corticosteroids with resolution of symptoms. Conclusion: —Panel sequencing of synovial sarcoma can identify targetable mutations. Treatment of BRAF^V600E synovial sarcoma with dabrafenib & trametinib can lead to complete pathological response and prolonged radiological response, as well as the rare adverse event of hemophagocytic lymphohistiocytosis. Prospective clinical trials are needed to evaluate the efficacy and safety of BRAF^V600E & MEK inhibitors as a therapeutic approach in BRAF^V600E synovial sarcoma.

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Supplementary Material

Supplementary Material File

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