TY - EJOU AU - Burg, Daniel AU - Babkoff, Aryeh AU - Or, Omer AU - Olshinka, Noam AU - Demma, Jonathan Abraham AU - Adila, Mohamad AU - Wygoda, Marc AU - Blumenfeld, Philip AU - Diment, Judith AU - Galiner, Masha AU - Azraq, Yusef AU - Katz, Daniela AU - Reissman, Petachia AU - Ostrowicki, Sadie AU - Sebbag, Gabriella AU - Elkhateeb, Narmine AU - Moshe, Anat Hershko AU - Jaber, Dania AU - Hollander, Adi AU - Rubin, Limor AU - Zick, Aviad TI - BRAFV600E Metastatic Synovial Sarcoma Treated with BRAF \& MEK Inhibitors Achieves Complete Response. A Case Report \& Literature Review T2 - Oncology Research PY - 2026 VL - 34 IS - 4 SN - 1555-3906 AB - 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 BRAFV600E 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 BRAFV600E synovial sarcoma with doxorubicin, Ifosphamide & Sodium 2-Mercaptoethanesulfonate. When we identified BRAFV600E in the tumor, the BRAFV600E 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 BRAFV600E 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 BRAFV600E & MEK inhibitors as a therapeutic approach in BRAFV600E synovial sarcoma. KW - Synovial sarcoma; B-Raf proto-oncogene; serine/threonine kinaseV600E; serine/threonine kinase inhibitor; mitogen-activated protein kinase kinase inhibitor; case report DO - 10.32604/or.2026.070233