Cancer Research and Treatment

Case Report

ALK Inhibition in a Patient with Inflammatory Myofibroblastic Tumor Harboring CARS1-ALK Fusion: Songji Choi, Miso Kim, Sheehyun Kim, Taekeun Park, Yoonjin Kwak, Jeong Mo Bae, Hongseok Yun, Jee Hyun Kim; Received December 10, 2024 Accepted December 14, 2024 Published online December 18, 2024; DOI: https://doi.org/10.4143/crt.2024.1184 [Accepted]

Inflammatory myofibroblastic tumor (IMT) is a rare entity, primarily affecting young individuals, often involving the abdomen, pelvis, or lung. Approximately 50% of IMTs harbor ALK gene rearrangements, making ALK inhibitors a viable treatment. We report a case of a 40-year-old female with metastatic IMT harboring a CARS1-ALK fusion. Initial chemotherapy failed, but targeted therapy with alectinib through the KOrean Precision Medicine Networking Group Study of MOlecular profiling guided therapy based on genomic alterations in advanced Solid tumors (KOSMOS)-II study led to significant tumor regression and ongoing, durable clinical improvement of 19 months. This case highlights the importance of precision medicine and raises the reappraisal of targeted agents outside of approved indications for rare cancers with actionable genomic alterations.

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Inflammatory myofibroblastic tumors of the colon in pediatrics: clinical presentation, management, and outcomes—A case report and systematic review of literature
Ismael Elhalaby, Omar Koura, Rofyda Elhalaby, Wael Zeina, Mohamed Shareef, Essam Elhalaby
International Journal of Colorectal Disease.2025;[Epub] CrossRef

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Original Article

Sarcoma

Clinicopathological Analysis and Treatment of Adult Patients with Inflammatory Myofibroblastic Tumor: A 15-Year Single-Center Study: Xin Liu, Chengcheng Gong, Jieyun Zhang, Wanjing Feng, Yanjing Guo, Youzhou Sang, Chunmeng Wang, Yong Chen, Jian Wang, Lin Yu, Xiaowei Zhang, Zhiguo Luo; Cancer Res Treat. 2023;55(3):1001-1010. Published online March 3, 2023; DOI: https://doi.org/10.4143/crt.2022.894

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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.

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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
Inflammatory myofibroblastic tumor of the adrenal gland: A case report
Jiyao Yang, Hongjin Shi, Haifeng Wang, Yidao Liu
Urology Case Reports.2024; 55: 102763. CrossRef
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
Rare giant epithelioid inflammatory myofibroblastic sarcoma of the abdominal cavity in a child: a case report and review of the literature
Jinzhou Li, Haixing Su, Sheng Zhang, Xianyun Chen, Chongzhi Hou, Tao Cheng
Frontiers in Oncology.2024;[Epub] CrossRef
Inflammatory myofibroblastic tumor from molecular diagnostics to current treatment
PAULINA CHMIEL, ALEKSANDRA SłOWIKOWSKA, ŁUKASZ BANASZEK, ANNA SZUMERA-CIEćKIEWICZ, BARTłOMIEJ SZOSTAKOWSKI, MATEUSZ J. SPAłEK, TOMASZ ŚWITAJ, PIOTR RUTKOWSKI, ANNA M. CZARNECKA
Oncology Research.2024; 32(7): 1141. CrossRef

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Case Report

A Case of Simultaneously Diagnosed Lung Adenocarcinoma and Endobronchial Inflammatory Myofibroblastic Tumor with Two Distinct Types of ALK Translocation: Shikang Zhao, Wei Liu, Shuo Li, Tao Shi, Qiusong Chen, Qi Li, Leina Sun, Dian Ren, Zuoqing Song, Chun Huang, Song Xu; Cancer Res Treat. 2021;53(2):601-606. Published online October 21, 2020; DOI: https://doi.org/10.4143/crt.2020.952

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A 61-year-old male patient was simultaneously diagnosed with lung adenocarcinoma and inflammatory myofibroblastic tumor (IMT). The lung adenocarcinoma and IMT harbored two distinct types of ALK translocation, LOC101927285-ALK, and TPM3-ALK, respectively. The ALK Ventana showed strong positivity on both lesions. The patient was therefore given an endobronchial cryotherapy and ALK inhibitor crizotinib. The tumors showed durable response however the left lung adenocarcinoma relapsed at 17th month post-crizotinib treatment. Tissue re-biopsy on the resistant tumor revealed an ALK exon 23 C1156Y missense mutation in addition to LOC101927285-ALK mutation. Further RNA-based sequence uncovered that the noncoding region rearrangement is the fusion mutation of EML4-ALK. The patient was therefore received alectinib, and the tumor exhibited partly response. Overall, it is very rare that two types of pulmonary tumors exist in one patient driven by two distinct ALK fusions, which emphasizes the necessity of gene sequencing in clinical decision-making and individualized therapy.

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TTC7A-ALK, a novel ALK fusion variant identified in a patient with metastatic lung adenocarcinoma, exhibits excellent response to crizotinib
Meijin Huang, Xiangqing Zhu, Wenmang Xu, Jun Zhu, Xin Xun, Bin Su, Hong Chen
Translational Oncology.2025; 54: 102345. CrossRef
Successful treatment of a non‐small‐cell lung cancer patient harboring HIP1‐ALK (H28:A20) and CTNNB1 p.S45del with alectinib
Vito Longo, Francesco Pesola, Rosanna Lacalamita, Annamaria Catino, Michele Montrone, Ilaria Marech, Pamela Pizzutilo, Elisabetta Sara Montagna, Stefania Tommasi, Domenico Galetta
Thoracic Cancer.2024; 15(31): 2283. CrossRef
Coexistence of a novel SV2B-ALK, EML4-ALK double-fusion in a lung poorly differentiated adenocarcinoma patient and response to alectinib: a case report and literature review
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Frontiers in Oncology.2024;[Epub] CrossRef
Novel treatment of endobronchial inflammatory myofibroblastic tumor in a child
Jessica Reyes‐Angel, Louis B. Rapkin, Jeffrey P. Simons, Hiren Muzumdar
Pediatric Pulmonology.2022; 57(1): 330. CrossRef
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Yan Xiang, Shiyu Zhang, Xiaoxu Fang, Yingying Jiang, Tingwen Fang, Jinwen Liu, Kaihua Lu
Current Oncology.2022; 29(10): 7816. CrossRef
Crizotinib in Sarcomatous Malignancies Harboring ALK Fusion With a Definitive Partner(s): Response and Efficacy
Jinchun Wu, Yongbin Hu, Omar Abdihamid, Gengwen Huang, Sheng Xiao, Bin Li
Frontiers in Oncology.2021;[Epub] CrossRef

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