REVIEW PAPER
Pulmonary inflammatory myofibroblastic tumor in children
1
Department of Paediatric Pneumonology and Allergology, Medical University of Warsaw, Warsaw, Poland
2
Department of Pediatric, Mazovian Centre for the Treatment of Lung Diseases and Tuberculosis in Otwock, Otwock, Poland
3
Department of Pathology, National Tuberculosis and Lung Diseases, Research Institute, Warsaw, Poland
Submission date: 2023-04-16
Final revision date: 2023-06-04
Acceptance date: 2023-06-20
Publication date: 2023-12-15
Corresponding author
Joanna Lange
Joanna Lange, PhD, Department of Paediatric Pneumonology and Allergology, Medical University of Warsaw, Zwirki i Wigury 63A, 02-091 Warsaw, Poland
Joanna Lange, PhD, Department of Paediatric Pneumonology and Allergology, Medical University of Warsaw, Zwirki i Wigury 63A, 02-091 Warsaw, Poland
Pediatr Pol 2023;98(4):320-325
KEYWORDS
TOPICS
ABSTRACT
Inflammatory myofibroblastic tumor (IMT) is a rare neoplasm characterized by mesenchymal spindle cell proliferation with a marked inflammatory infiltrate component. Although the exact etiopathology of pulmonary IMT is still not clear, gene fusions involving anaplastic lymphoma kinase (ALK) or ROS proto-oncogene 1, receptor tyrosine kinase (ROS1), neurotrophic tyrosine receptor kinase (NTRK), platelet-derived growth factor receptor (PDGFR) and rearranged during transfection (RET) have been recently detected in immunohistochemical assessment of this lesion. Due to its nonspecific clinical or radiological presentation, the diagnosis of IMT primarily depends on histopathological findings. Surgery remains the mainstay of treatment and provides the best chance to limit recurrence. In unresectable lesions or multifocal/metastatic disease, treatment with chemotherapy or ALK, ROS1, RET and NTRK inhibitors is recommended. The prognosis in children with IMT is generally perceived as favorable, although local invasion and metastasis have been reported.
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