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CANCER:

Synovial Sarcoma

2ms
Driver vs. passenger: Primary resistance to larotrectinib in synovial sarcoma harboring concurrent SS18 rearrangement and PDE3A-NTRK2 fusion. (PubMed, Tumori)
This case provides critical clinical evidence of the "driver versus passenger" phenomenon in precision oncology. It demonstrates that the epigenetic dominance of the SS18::SSX fusion can override the therapeutic relevance of a concurrent NTRK fusion. Our findings serve as a cautionary note: actionable targets detected via next-generation sequencing must be interpreted within the tumor's canonical biological context, as lineage-defining translocations may render secondary kinase alterations therapeutically inert.
Journal
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NTRK2 (Neurotrophic tyrosine kinase, receptor, type 2) • NTRK (Neurotrophic receptor tyrosine kinase) • PDE3A (Phosphodiesterase 3A) • SS18 (SS18 Subunit Of BAF Chromatin Remodeling Complex)
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NTRK fusion
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Vitrakvi (larotrectinib)
2ms
Primary renal synovial sarcoma: a clinicopathological study of 23 cases highlighting the diagnostic value of SS18-SSX, TLE1, and INI1 immunohistochemistry panel. (PubMed, Virchows Arch)
PRSS is a rare malignant tumor with a poor prognosis that presents significant diagnostic challenges. Our immunohistochemical panel (SS18-SSX, TLE1, INI1) enhances diagnostic accuracy and may assist in the triage of cases requiring molecular confirmation.
Clinical • Journal • IO biomarker
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BCL2 (B-cell CLL/lymphoma 2) • SS18 (SS18 Subunit Of BAF Chromatin Remodeling Complex)
2ms
BRD9 Degraders Unleash GBAF Chromatin Remodeling Activity in Synovial Sarcoma. (PubMed, Cancer Res)
Together, these findings show that BRD9 restrains GBAF activity, with BRD9 degradation increasing enzymatic remodeling and target gene expression by fusion oncoprotein-distributed GBAFs in SyS. This subtle epigenetic disturbance creates a low hurdle for SyS to surpass, limiting the therapeutic efficacy of BRD9 degraders.
Journal
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SMARCA4 (SWI/SNF related, matrix associated, actin dependent regulator of chromatin, subfamily A, member 4) • SS18 (SS18 Subunit Of BAF Chromatin Remodeling Complex)
2ms
Penis synovial sarcoma: a rare case report (PubMed, Medicina (B Aires))
The origin of these penile lesions is unknown and has been associated with predisposing factors such as previous exposure to radiotherapy and the presence of the SS18-SSX1/2/4 fusion gene. We present an unusual case of monophasic penile synovial sarcoma in an elder in the seventh decade of life with a metastatic lesion at the pulmonary level.
Journal
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SS18 (SS18 Subunit Of BAF Chromatin Remodeling Complex) • SSX1 (SSX Family Member 1)
2ms
Diagnostic challenges in synovial sarcoma: a case report. (PubMed, J Med Case Rep)
This case highlights the complexity of diagnosing SS, given its subtle clinical findings, slow growth, and atypical presentation. Multidisciplinary care is essential for optimizing diagnosis and treatment outcomes. Prompt recognition and comprehensive care can improve patients' prognosis.
Journal
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BCL2 (B-cell CLL/lymphoma 2) • VIM (Vimentin) • SS18 (SS18 Subunit Of BAF Chromatin Remodeling Complex)
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doxorubicin hydrochloride • ifosfamide • mesna
2ms
Enrollment change • Trial completion date
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zanzalintinib (XL092)
2ms
Diagnostic Dilemma and Therapeutic Challenges in EWSR1::PATZ1 Fusion Sarcomas: Insights From Two Patients. (PubMed, Int J Surg Pathol)
Here, we describe two patients with EWSR1::PATZ1 sarcoma, of which one patient was initially misdiagnosed as synovial sarcoma and RMS on two occasions. These patients underscore the diagnostic challenges and therapeutic uncertainties surrounding EWSR1::PATZ1 fusion sarcomas, emphasizing the need for further large collaborative studies to establish optimal prognostic implications and management strategies for this rare entity.
Journal
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EWSR1 (EWS RNA Binding Protein 1) • PATZ1 (POZ/BTB And AT Hook Containing Zinc Finger 1)
2ms
Trial completion • Trial completion date
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doxorubicin hydrochloride • pazopanib • cyclophosphamide • ifosfamide • daunorubicin
2ms
Case Report: Synovial sarcoma with diffuse myxoid stroma and complete absence of epithelial differentiation in the extremity. (PubMed, Front Oncol)
This case highlights that synovial sarcoma can exhibit a myxoid phenotype and may lack epithelial marker expression, both of which can complicate the diagnosis. It also underscores the importance of integrating molecular analyses, particularly fusion-oriented genomic testing, for accurate diagnosis in challenging soft-tissue tumors.
Journal
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SOX9 (SRY-Box Transcription Factor 9) • SS18 (SS18 Subunit Of BAF Chromatin Remodeling Complex) • SSX1 (SSX Family Member 1)
2ms
Assessment of SS18-SSX Fusion Specific Antibody in Carcinoma and Melanoma. (PubMed, Int J Surg Pathol)
Here, we demonstrate no cross reactivity in various carcinomas. These results further support that the SS18::SSX fusion specific antibody is highly specific for synovial sarcoma.
Journal
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SS18 (SS18 Subunit Of BAF Chromatin Remodeling Complex)
2ms
Case Report: A case of primary renal synovial sarcoma. (PubMed, Front Surg)
Postoperatively, the patient received chemotherapy with epirubicin combined with ifosfamide. Follow-up showed no significant evidence of metastasis or recurrence. This report aims to provide clinical reference regarding the imaging findings and diagnosis of this rare disease, primary renal synovial sarcoma.
Journal
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SS18 (SS18 Subunit Of BAF Chromatin Remodeling Complex)
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ifosfamide • epirubicin
2ms
NKG2D-based CAR-T cells for synovial sarcoma: A preclinical proof-of-concept study. (PubMed, Biomed Pharmacother)
These CAR-T cells showed effector responses and antitumor effects against synovial sarcoma cells in vitro, as demonstrated by the results of the intracellular cytokine production, cytokine secretion, CD107a degranulation assay, WST-8 assay, and real-time cell analysis, and in vivo in an NSG mouse xenograft model of synovial sarcoma. Although further mechanistic, translational, and safety validation studies are required, these findings provide a preliminary disease-specific preclinical proof of concept for HLA-independent NKG2D-based CAR-T cell therapy in synovial sarcoma, particularly in patients who are ineligible for or have not responded to TCR-T therapy.
Preclinical • Journal • IO biomarker
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LAMP1 (Lysosomal Associated Membrane Protein 1) • NKG2D (killer cell lectin like receptor K1)