Here, we present the case of an adolescent with a rare metastatic Inflammatory myofibroblastic tumor (IMT) harboring a TFG-ROS1 fusion initially detected on tumor progression and retrospectively identified in the primary tumor after targeted RNA sequencing. The patient benefitted from sequential TKIs over a 5-year period with response to the third generation ALK/ROS inhibitor, lorlatinib leading to resection of the primary tumor. Detailed molecular analysis can identify targetable oncogenic kinase fusions that alters management in patients with unresectable disease and should be considered in all patients.
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http://dx.doi.org/10.1016/j.lungcan.2021.05.024 | DOI Listing |
Medicine (Baltimore)
May 2024
Rare Tumors Department, Shandong Cancer Hospital and Institute, Shandong First Medical University and Shandong Academy of Medical Sciences, Jinan, China.
Introduction: Inflammatory myofibroblastic tumor (IMT) is a rare invasive soft tissue tumor. Many IMTs are positive for anaplastic lymphoma kinase (ALK) with ALK gene fusion; other gene mutations have also been reported, which indicates a key role for genetic testing and the development of target therapy to optimize treatment strategies.
Patient Concerns: We report 2 patients who obtained clinical benefits following targeted treatment with ensartinib.
Mol Genet Genomic Med
April 2024
Division of Medical Oncology, University of Washington, Seattle, Washington, USA.
Background: Inflammatory myofibroblastic tumors (IMTs) are rare mesenchymal soft tissue sarcomas that often present diagnostic challenges due to their wide and varied morphology. A subset of IMTs have fusions involving ALK or ROS1. The role of next-generation sequencing (NGS) for classification of unselected sarcomas remains controversial.
View Article and Find Full Text PDFCancer Rep (Hoboken)
January 2024
Heinrich/Bangerter, Hämatologie-Onkologie im Zentrum MVZ, Augsburg, Germany.
Background: Inflammatory myofibroblastic tumor (IMTs) are rare mesenchymal neoplasms with slow growth. Resection is considered as therapeutic standard, with chemotherapy being insufficiently effective in advanced disease. ALK translocations are present in 50% of cases, ROS1 fusions (YWHAE::ROS1, TFG::ROS1) are extremely rare.
View Article and Find Full Text PDFZhonghua Bing Li Xue Za Zhi
June 2023
Department of Pathology, Shanghai First Maternity and Infant Hospital, Tongji University, Shanghai 201204, China.
Int J Gynecol Pathol
September 2023
The majority of inflammatory myofibroblastic tumors (IMTs) in the gynecologic tract occur in the uterine corpus and harbor anaplastic lymphoma kinase ( ALK ) rearrangement. Herein, we report 1 uterine IMT case with a novel fusion involving ALK and 1 uterine IMT case with ROS1 rearrangement. The ages of the patients were 56 and 57 yr, respectively.
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