Abstract
Inflammatory myofibroblastic tumors (IMTs) are extremely rare neoplasms, accounting for 0.04–0.7% of all tumors. Data on their clinical presentation, pathogenesis, and biological behavior are scarce. They can occur at any age, involving equally males and females. IMTs have been described as benign neoplasms with intermediate malignant potential, as local recurrence and distant metastasis may occur. IMTs are characterized by a solid growth type, and a multifocal type is uncommon. Definitive surgery is the treatment of choice. We describe a clinical case of a malignant IMT with multi-organ metastasis 6 years after definitive surgery on the primary tumor. There is no standard of care in such a clinical situation, especially given the absence of mutations in the primary and metastatic tumors.
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