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<p>Inflammatory myofibroblastic tumor (IMT) is a rare mesenchymal neoplasm that can occur across all age groups and presents with heterogeneous clinical behavior. Clinical manifestations vary by tumor location and may include pain, swelling, or systemic inflammatory symptoms. Diagnosis relies on imaging and histopathological confirmation. A subset of IMTs harbor ALK gene rearrangements, supporting the use of targeted therapies. This study aimed to describe the demographic, clinical, pathological, and treatment-related characteristics of adult IMT patients, with emphasis on disease course, inflammatory markers, therapeutic responses, and survival outcomes. In this retrospective cohort, 24 patients diagnosed with IMT between 2015 and 2023 were included. Clinical features, tumor characteristics, ALK status, inflammatory markers, treatment modalities, and survival outcomes were recorded. Progression-free survival (PFS) and overall survival (OS) were analyzed using Kaplan–Meier and Cox regression methods. The median age was 31 years, and 54.2% were female. The abdomen (41.7%) and extremities (29.2%) were the most common tumor locations. Surgical resection was performed in 79.2% of patients, with R0 resection achieved in 73.7% of cases. ALK positivity was detected in 20.8% of patients. Disease progression occurred in 58.3% of the cohort. Among two patients treated with crizotinib, PFS durations were 10.0 and 17.0 months, respectively. The median PFS was 20.8 months, and the median OS was 43.5 months. Hypoalbuminemia was associated with reduced OS, while CRP and ESR did not reach statistical significance. R0 surgical resection was the strongest predictor of favorable outcomes in IMT. ALK-targeted therapy provided variable but clinically meaningful disease control. Although inflammatory markers did not consistently predict survival, abnormal values trended toward poorer outcomes. Larger multicenter studies are needed to clarify prognostic indicators and improve therapeutic strategies for this rare entity.</p>

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Clinical characteristics and survival outcomes of adult inflammatory myofibroblastic tumor: a retrospective single-center study

  • 期刊论文

  • 出版模式：

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  • 发表日期：

    2026年1月19日

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  • Ayşegül Dumludağ,
  • Gizem Yıldırım,
  • Alper Topal,
  • Esmanur Kaplan Tüzün,
  • Berkan Karadurmuş,
  • İsmail Ertürk,
  • Nuri Karadurmuş

摘要

Inflammatory myofibroblastic tumor (IMT) is a rare mesenchymal neoplasm that can occur across all age groups and presents with heterogeneous clinical behavior. Clinical manifestations vary by tumor location and may include pain, swelling, or systemic inflammatory symptoms. Diagnosis relies on imaging and histopathological confirmation. A subset of IMTs harbor ALK gene rearrangements, supporting the use of targeted therapies. This study aimed to describe the demographic, clinical, pathological, and treatment-related characteristics of adult IMT patients, with emphasis on disease course, inflammatory markers, therapeutic responses, and survival outcomes. In this retrospective cohort, 24 patients diagnosed with IMT between 2015 and 2023 were included. Clinical features, tumor characteristics, ALK status, inflammatory markers, treatment modalities, and survival outcomes were recorded. Progression-free survival (PFS) and overall survival (OS) were analyzed using Kaplan–Meier and Cox regression methods. The median age was 31 years, and 54.2% were female. The abdomen (41.7%) and extremities (29.2%) were the most common tumor locations. Surgical resection was performed in 79.2% of patients, with R0 resection achieved in 73.7% of cases. ALK positivity was detected in 20.8% of patients. Disease progression occurred in 58.3% of the cohort. Among two patients treated with crizotinib, PFS durations were 10.0 and 17.0 months, respectively. The median PFS was 20.8 months, and the median OS was 43.5 months. Hypoalbuminemia was associated with reduced OS, while CRP and ESR did not reach statistical significance. R0 surgical resection was the strongest predictor of favorable outcomes in IMT. ALK-targeted therapy provided variable but clinically meaningful disease control. Although inflammatory markers did not consistently predict survival, abnormal values trended toward poorer outcomes. Larger multicenter studies are needed to clarify prognostic indicators and improve therapeutic strategies for this rare entity.