Therapeutic options in inoperable ROS1-rearranged inflammatory myofibroblastic tumor of the tongue in a child: a case report and literature review. Issue 10 (November 2021)

• Record Type: Journal Article
• Authors: Styczewska, Malgorzata; Patel, Agastya; Jaskulowska, Joanna; Godzinski, Jan; Swieton, Dominik; Wasag, Bartosz; Dass, Juliea; Bien, Ewa; Krawczyk, Malgorzata A.
• Is Part Of: Anti-cancer drugs. Volume 32:Issue 10(2021)
• Publication Date: 2021-11
• Subjects: crizotinib -- inflammatory myofibroblastic tumor -- targeted therapy -- TFG-ROS1 -- tongue; Antineoplastic agents -- Periodicals; Cancer -- Chemotherapy -- Periodicals; Antineoplastic Agents -- therapeutic use -- Periodicals; Drug Therapy -- Periodicals; 616.994061
• DOI: 10.1097/CAD.0000000000001099
• Languages: English
• ISSNs: 0959-4973

Abstract

Abstract : Inflammatory myofibroblastic tumor (IMT) is a rare borderline malignancy, usually treated with surgery only. Exceedingly rare cases of inoperable, recurrent, or metastatic IMTs pose a therapeutic challenge. We report successful treatment of a 7-year-old girl with an inoperable anaplastic lymphoma kinase (ALK)-negative IMT of the tongue. The patient underwent various anti-inflammatory (steroids, nonsteroidal anti-inflammatory drugs, clarithromycin) and antiproliferative (chemotherapy) therapies to enable tumor regression and complete resection. Ultimately, next-generation sequencing of the tumor revealed a TFG-ROS-1 translocation, allowing for an off-label targeted therapy with crizotinib. Crizotinib treatment caused slight tumor regression but evident change of its structure, allowing for complete non-mutilating resection. Two histopathology examinations revealed complete disappearance of neoplastic cells following therapy. The patient remains disease-free 22 months after the delayed surgery. In children with inoperable ALK-negative IMTs, molecular testing must be performed to identify other targetable oncogenic fusions, including TFG-ROS1.