RESUMO
Inflammatory myofibroblastic tumor (IMT) is a rare neoplasm of children and young adults. It consists of myofibroblastic cells of borderline malignancy admixed with inflammatory cells. According to the WHO classification, of tumors, it is a soft-tissue tumor with an intermediate malignant potential. It may arise within numerous organs. Ureteral location is exceedingly rare. We report the exceptional case of a 4-year-old boy presenting with a giant right ureteral IMT removed during a nephroureterectomy. Histology and immunohistochemistry confirmed the diagnosis of an IMT with Anaplastic Lymphoma Kinase 1 expression. A 7 months follow-up showed no recurrence or distant metastasis. We also reviewed the literature for similar cases of pediatric ureteral IMT.
