CASE REPORT article
Front. Oncol.
Sec. Pediatric Oncology
Volume 15 - 2025 | doi: 10.3389/fonc.2025.1561051
Novel findings of Larotrectinib in children with NTRK-rearranged spindle cell tumor
Provisionally accepted
- 1Sun Yat-sen University Cancer Center; State Key Laboratory of Oncology in South China; Collaborative Innovation Center for Cancer Medicine, Guangzhou, China
- 2Department of pediatric oncology, Sun Yat-sen University Cancer Center, Guangzhou, China
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Neurotrophic tropomyosin receptor kinase (NTRK)-rearranged spindle cell tumors are often resistant to chemotherapy and radiotherapy. Fortunately, they are sensitive to targeted therapy of tropomyosin receptor kinase (TRK) inhibitors. However, the data on larotrectinib in Chinese children with NTRK-rearranged spindle cell tumor are still scarce. We reported 4 children with TRK fusion-positive solid tumors received larotrectinib in different clinical scenarios, including second-line treatment after progressive disease (patient #1), relapse after resection (patients #2 and #3), and metastatic disease (patient #4) and all of them benefited from the treatment. The patients harbored different TRK fusion genes (patient #1: TP53-NTRK1; #2: TPM3-NTRK1; #3: TPM3-NTRK1, DCST1-NTRK1, ZBTB7B-NTRK1, and NTRK1-DCST2; #4: LMNA-NTRK1). Our study provides new insights into the biology and management of NTRK-rearranged spindle cell tumors, contributing to the expanding evidence supporting the use of TRK inhibitors in these tumors. Further studies are needed to validate our findings and to explore the potential of second-generation TRK inhibitors in overcoming resistance.
Keywords: Larotrectinib, Trk inhibitor, NTRK-rearranged, Children, Spindle cell tumor
Received: 15 Jan 2025; Accepted: 15 Sep 2025.
Copyright: © 2025 Wu, Xie, Wang, Lu, Zhang and Huang. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
* Correspondence:
Yizhuo Zhang, zhangyzh@sysucc.org.cn
Junting Huang, huangjt@sysucc.org.cn
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