Purpose Inflammatory myofibroblastic tumor (IMT) is a rare mesenchymal malignancy that occurs primarily in children and adolescents. The clinical and pathological features of IMT in adult patients are not well understood.
Materials and Methods We retrospectively searched for records of adult patients with IMT at Fudan University Shanghai Cancer Center from 2006 to 2021. Clinicopathological data, treatments, and outcomes were collected and analyzed.
Results Thirty adult patients with IMT, mostly women (60.0%), were included. The median age of the patients was 38 (21-77). The most common primary site was abdominopelvic region (53.3%), followed by lungs (20.0%). Seven patients had an abdominal epithelioid inflammatory myofibroblast sarcoma (EIMS). The positivity rate of anaplastic lymphoma kinase (ALK) was 81.5% (22/27). Sixteen patients with advanced ALK-positive disease received crizotinib, with an objective response rate (ORR) of 81.3% and a disease control rate of 87.5%. The median progression-free survival was 20.8 months. EIMS was associated with more aggressive behavior; however, the prognosis was similar to that of non-EIMS patients after treatment with an ALK inhibitor. At a median follow-up time of 30 months (95% confidence interval [CI], 13.6 to 46.4), the 5-year overall survival was 77% (95% CI, 66 to 88) in all patients.
Conclusion Adult IMTs appeared more aggressive, with a higher incidence of recurrence and metastases, and patients with EIMS had more aggressive cases. Treatment with ALK inhibitors resulted in a high ORR and a durable response, which suggested that ALK inhibitors could be used as a first-line treatment option in adult patients with ALK-positive advanced IMT.
Citations
Citations to this article as recorded by
Clinicopathological Characteristics of Inflammatory Myofibroblastic Tumor: A Single Center Retrospective Cohort Study Xiaoyan Si, Shafei Wu, Ruie Feng, Mengzhao Wang, Hanping Wang, Xiaotong Zhang, Li Zhang, Kaifeng Xu Thoracic Cancer.2025;[Epub] CrossRef
Case report: Intra-abdominal inflammatory myofibroblastic tumor with mucinous features: a case of rapid recurrence and dissemination post-surgery Xingchen Li, Jie Li, Chunxiao Liang, Qing Zou Frontiers in Oncology.2025;[Epub] CrossRef
Clinical diagnosis and treatment of abdominal inflammatory myofibroblastic tumors Qiang Zhang, Zhi-wei Zhang, Jing Fan, Zhuo-Ma Ji, Chun-Yan Wang, Feng Liu Discover Oncology.2025;[Epub] CrossRef
Therapeutic strategy and prognostic analysis of inflammatory myofibroblastic tumor in the head and neck: a retrospective study Feng Liu, Yanchao Qin, Zhiwei Zhang, Mengru Li, Bowei Feng, Wei Ding, Shubin Dong PeerJ.2025; 13: e19315. CrossRef
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Ibero-American Consensus for the Management of Peritoneal Sarcomatosis: Updated Review and Clinical Recommendations Francisco Cristóbal Muñoz-Casares, Javier Martín-Broto, Pedro Cascales-Campos, Juan Torres-Melero, Irene López-Rojo, José Gómez-Barbadillo, Luis González-Bayón, Ana Sebio, César Serrano, Sara Carvalhal, Joaquim Abreu de Souza, Alexandre Souza, Guillermo F Cancers.2024; 16(15): 2646. CrossRef
Thoracic epithelioid inflammatory myofibroblastic sarcoma: a rare and aggressive disease with case report and literature review Linke Yang, Pei Li, Runze Liu, Baomin Feng, Huiqing Mao, Xiaoyong Tang, Guangjian Yang Discover Oncology.2024;[Epub] CrossRef
Metastasized inflammatory myofibroblastic tumor of uterine origin Thomas Bartl, Michael Deavers, Ryan Blair Kieser, Pedro T Ramirez International Journal of Gynecological Cancer.2024; 34(10): 1643. CrossRef
Epithelioid inflammatory myofibroblastic sarcoma with exceptionally long response to lorlatinib—a case report Rafał Becht, Kajetan Kiełbowski, Justyna Żychowska, Wojciech Poncyljusz, Aleksandra Łanocha, Katarzyna Kozak, Ewa Gabrysz-Trybek, Paweł Domagała Therapeutic Advances in Medical Oncology.2024;[Epub] CrossRef
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Purpose
Drug resistance is one of the main causes of chemotherapy failure in patients with small cell lung cancer (SCLC), and extensive biological studies into chemotherapy drug resistance are required.
Materials and Methods
In this study, we performed lncRNA microarray, in vitro functional assays, in vivo models and cDNA microarray to evaluate the impact of lncRNA in SCLC chemoresistance.
Results
The results showed that KCNQ1OT1 expression was upregulated in SCLC tissues and was a poor prognostic factor for patients with SCLC. Knockdown of KCNQ1OT1 inhibited cell proliferation, migration, chemoresistance and promoted apoptosis of SCLC cells. Mechanistic investigation showed that KCNQ1OT1 can activate transforming growth factor-β1 mediated epithelial-to-mesenchymal transition in SCLC cells.
Conclusion
Taken together, our study revealed the role of KCNQ1OT1 in the progression and chemoresistance of SCLC, and suggested KCNQ1OT1 as a potential diagnostic and prognostic biomarker in SCLC clinical management.
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