Thoracic Multidetector Computed Tomography Evaluation of Inflammatory Myofibroblastic Tumor of the Lung in Pediatric Patients in the Era of Modern Diagnosis.

Abstract

The purpose of this study was to investigate the characteristic thoracic multidetector computed tomography (MDCT) findings of pathologically proven inflammatory myofibroblastic tumor (IMT) of the lung in children in the era of modern understanding based on refined pathologic diagnosis. All pediatric patients (age 18 y and above) with a known pathologic diagnosis of IMT of the lung who underwent thoracic MDCT studies from May 2008 to December 2020 were included. Two pediatric radiologists independently evaluated thoracic MDCT studies for the presence of abnormalities in the lung (nodule, mass, cyst, ground-glass opacity, consolidation), pleura (pleural effusion, pneumothorax), and mediastinum and hilum (lymphadenopathy). When a lung abnormality was present, the number, size, composition (solid, cystic, or combination of both), location (laterality, lobar distribution, and intraparenchymal vs. pleural-based), borders (well-circumscribed vs. ill-defined), the presence and type of associated calcification (punctate, dense, curvilinear, or flocculent), the presence of associated cavitation, contrast enhancement pattern (homogeneous, heterogenous, central, or peripheral), and other associated findings (neural foramen involvement, anomalous vessels, mass effect, and invasion of adjacent thoracic structures) were also evaluated. Interobserver agreement between 2 independent reviewers was evaluated with κ statistics. In all, 12 thoracic MDCT studies from 12 individual pediatric patients (5 males [42%] and 7 females [58%]; mean age: 9.9 y; SD: 4.4 y; range: 2 to 16 y) comprised the final study population. All 12 thoracic MDCT studies (100%) were performed with intravenous contrast. The most frequent MDCT finding of IMT of the lung in children is a solitary (92%), pleural-based (83%), well-circumscribed (100%), solid (92%) mass with heterogenous contrast enhancement (100%), often with dense calcification (50%), which occurred in both lungs and all lobes with similar frequency. No pleural abnormality (pleural effusion, pneumothorax) or mediastinal abnormality (lymphadenopathy) was detected. In addition, although mass effect on adjacent thoracic structures was frequently seen (42%), no invasion, neural foramen involvement, or associated anomalous vessels was identified. There was excellent interobserver κ agreement between 2 independent reviewers for detecting abnormalities on thoracic MDCT studies (κ>0.95). IMT of the lung in children typically presents as a solitary, pleural-based, well-circumscribed, solid mass with heterogenous contrast enhancement, often with dense calcification, without significant laterality or lobar preference. In addition, pleural or mediastinal abnormalities are characteristically absent. These notable MDCT attributes of IMT of the lung are an important and novel finding, with great potential to help differentiate pediatric IMT of the lung from other thoracic masses in children.