Marked Response to Larotrectinib in Pelvic Spindle Cell Sarcoma With Concurrent TPM3-NTRK1 Fusion and CDK4/MDM2 Amplification: A Case Report

Case report: Adult NTRK-rearranged spindle cell neoplasms with TPM3-NTRK1 fusion in the pelvic

“…In conclusion, we report a novelty case of high-grade NTRK-RSCNs with TPM3-NTRK1 fusion occurring in the pelvis. Firstly, Although NTRK-rearranged spindle cell neoplasms have been reports before (21,22), this case with different clinical and molecular characteristics. This case represents the first reported instance of imaging, pathology, molecular characteristics, and clinical features integration.…”

“…Huang W K et al. newly reported a patient with locally advanced and TPM3- NTRK1 fusion pelvic spindle cell tumor, who responded well to neoadjuvant Larotrectinib therapy, after 4 months of adjuvant Larotrectinib therapy, the patient remained disease-free for 8 months and underwent successful surgical resection ( 21 ).…”

“…Identification of NTRK fusion tumors has been proven to be meaningful because chemotherapeutic NTRK inhibitors, including Entrectinib and Larotrectinib, showed the efficacy and safety in treating advanced TRK fusion sarcomas (20). Huang W K et al newly reported a patient with locally advanced and TPM3-NTRK1 fusion pelvic spindle cell tumor, who responded well to neoadjuvant Larotrectinib therapy, after 4 months of adjuvant Larotrectinib therapy, the patient remained disease-free for 8 months and underwent successful surgical resection (21).…”

Identification and validation of protein biomarkers for predicting gastrointestinal stromal tumor recurrence

“…Another study showed suppressing TPM3 expression with TPM3-siRNA reduced cellular invasion and migration and decreased MMP-9 and SNAI1 levels in glioma cells [42] . However, research on TPM3's role in GIST is limited, with some exceptions like reported TPM3-NTRK1 fusion cases in mesenchymal spindle-cell neoplasms, distinct from GIST [43] , [44] .…”