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<p>Inflammatory myofibroblastic tumor is an ultra-rare mesenchymal neoplasm of intermediate malignant potential, characterized by oncogenic rearrangements involving ALK (50–60%), ROS1 (5–10%), and less commonly NTRK3 or RET. We report a 32-year-old woman with an unresectable pulmonary tumor harboring a TFG–ROS1 fusion who achieved a durable partial response to brigatinib, sustained for more than 12 months. Histopathologic evaluation revealed a low-grade spindle-cell neoplasm lacking ALK expression, and genomic profiling identified the actionable fusion. This case highlights how molecular characterization can refine the management of ultra-rare sarcomas, enabling anatomically constrained disease to become targetable and underscoring the role of tumor biology in therapeutic decision-making.</p>

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Durable response to brigatinib in a ROS1 fusion–positive inflammatory myofibroblastic tumor

  • 期刊论文

  • 发表日期：

    2026年4月18日

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  • Gabriela Alamilla-García,
  • Oscar Tahuahua-Flores,
  • Alejandro Soriano-Maya,
  • Enrique Caballé-Pérez,
  • Claudia Haydee Sarai Caro-Sánchez,
  • Dorian Yarih García-Ortega

摘要

Inflammatory myofibroblastic tumor is an ultra-rare mesenchymal neoplasm of intermediate malignant potential, characterized by oncogenic rearrangements involving ALK (50–60%), ROS1 (5–10%), and less commonly NTRK3 or RET. We report a 32-year-old woman with an unresectable pulmonary tumor harboring a TFG–ROS1 fusion who achieved a durable partial response to brigatinib, sustained for more than 12 months. Histopathologic evaluation revealed a low-grade spindle-cell neoplasm lacking ALK expression, and genomic profiling identified the actionable fusion. This case highlights how molecular characterization can refine the management of ultra-rare sarcomas, enabling anatomically constrained disease to become targetable and underscoring the role of tumor biology in therapeutic decision-making.