# Ensartinib Combined with Radiotherapy for the Treatment of Advanced Primary Pulmonary Epithelioid Inflammatory Myofibroblastic Sarcoma Harboring TPM3-ALK Fusion: A Case Report

## TL;DR

A patient with advanced lung EIMS showed significant tumor shrinkage and long-term control using ensartinib and radiotherapy.

## Contribution

This case report demonstrates the effectiveness of combining ensartinib and radiotherapy for TPM3-ALK fusion-positive EIMS.

## Key findings

- The patient achieved over 32 months of progression-free survival with no major side effects.
- Combining ensartinib with radiotherapy led to significant tumor reduction in a rare lung sarcoma case.
- TPM3-ALK fusion detection guided targeted therapy, offering a new treatment option for advanced EIMS.

## Abstract

上皮样炎性肌纤维母细胞肉瘤（epithelioid inflammatory myofibroblastic sarcoma, EIMS）是一种罕见且极具侵袭性的间叶来源肿瘤，通常与间变性淋巴瘤激酶（anaplastic lymphoma kinase, ALK）基因融合有关。手术是早中期患者主要治疗手段，但晚期患者的治疗策略目前尚无明确共识。肺原发EIMS更为罕见，仅有个案报道。虽然单药ALK-酪氨酸激酶抑制剂（tyrosine kinase inhibitors, TKIs）靶向治疗是一种可行方案，但临床疗效仍不理想。本文报告了1例晚期肺原发EIMS伴有TPM3-ALK融合的病例，接受第二代ALK-TKI恩沙替尼一线靶向治疗及残留病灶和转移灶放疗后肿瘤明显缩小并得到持续控制，无进展生存期（progression-free survival, PFS）超过32个月，且未观察到明显治疗相关不良反应。本文探讨了以基因检测为证据的靶向治疗联合局部放疗的可行性，旨在为晚期肺原发EIMS患者提供新的治疗选择。