• Silvia Angela Debonis, Alberto Bongiovanni, Federica Pieri, Valentina Fausti, Alessandro De Vita, Nada Riva, Lorena Gurrieri, Silvia Vanni, Danila Diano, Laura Mercatali, and Toni Ibrahim.
• Osteoncology and Rare Tumors Center, IRCCS Istituto Romagnolo per lo Studio dei Tumori (IRST) "Dino Amadori," Meldola.
• Medicine (Baltimore). 2021 May 21; 100 (20): e25972e25972.

RationaleInflammatory myofibroblastic tumor (IMT) is a rare mesenchymal tumor that is prevalent among children and adolescents. Surgery is the most important therapeutic approach for IMT and complete resection is recommended. Although 50% of IMTs show anaplastic lymphoma kinase (ALK) rearrangements, crizotinib has proven an effective therapeutic approach. However, the genetic landscape of this tumor is still not fully understood and treatment options are limited, especially in the majority of ALK-negative tumors.Patient ConcernsWe describe the clinical case of a healthy 18-year-old female in whom a pulmonary nodule was incidentally detected.DiagnosesFollowing a small increase in the size of the nodule, the patient underwent both 18FDG-PET/CT and 68Ga-PET/CT, resulting in a suspicion of bronchial hamartoma.InterventionsThe patient underwent surgery and a salivary gland-like lung tumor was diagnosed.OutcomesAfter surgery, the patient was referred to our cancer center, where a review of the histology slides gave a final diagnosis of ALK-negative lung IMT. Given the histology, it was decided not to administer adjuvant therapy and the patient was placed in a 3-monthly follow-up program. The patient is still disease-free 2 years post-surgery.LessonsAlthough there is no standard of care for the treatment of IMT, identifying genomic alterations could help to redefine the management of patients with negative-ALK disease. Our review of the literature on IMT and other kinase fusions revealed, in addition to ALK rearrangements, the potential association of ROS1, NTRK, RET, or PDGFR beta alterations with the tumor.Copyright © 2021 the Author(s). Published by Wolters Kluwer Health, Inc.

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