case_images: PMC10722287_01
case_images: CT scan showing a voluminous parenchymal mass (M) of the anterior left thoracic wall, relatively well delimited, with bone destruction, which invades the lung parenchyma up to the level of the heart, with mass effect on left ventricle.
case_images: fped-11-1289256-g001.jpg
case_images: PMC10722287_01_fped-11-1289256-g001.jpg
case_images: F1
case_images: A follow-up chest CT scan performed after 14 days of treatment, revealing a parenchymal lesion measuring 61/56/39 mm with aggressive features (osteolysis and pericardial reaction) located at the base of the left hemithorax (Figure 1).
case_images: Microscopic examination of the biopsied lesion displaying small round to oval cells with dispersed chromatin [(A) 10x and (B) 40x magnification].
case_images: fped-11-1289256-g002.jpg
case_images: PMC10722287_01_fped-11-1289256-g002.jpg
case_images: F2
case_images: FISH testing revealed rearrangements of the EWSR1 gene (22q12) in 65% of tumor cells, leading to the conclusion of Ewing/PNET sarcoma, with architectural and localization characteristics of an Askin tumor (Figure 2).
case_images: (A) - CT scan revealing voluminous parenchymal mass (M) of the anterior left thoracic wall, relatively well delimited, with bone destruction, which invades the lung parenchyma up to the level of the heart, with mass effect on left ventricle; and (B) - hypodense formation in the segment VII of the liver.
case_images: fped-11-1289256-g003.jpg
case_images: PMC10722287_01_fped-11-1289256-g003.jpg
case_images: F3
case_images: An 85 x 60 x 35 mm formation in the lower half of the left hemithorax, with a tumor volume of 140.1 cm3, and a hypodense formation of approximately 15 mm in segment VII of the liver were identified (Figure 3).
case_images: A graph depicting the child's weight curve over the past 6 years reveals a notable weight loss during the oncological treatment period, and significant increase upon the initiation of EIT (elexacaftor/tezacaftor/ivacaftor) treatment.
case_images: fped-11-1289256-g004.jpg
case_images: PMC10722287_01_fped-11-1289256-g004.jpg
case_images: F4
case_images: In March 2023, CFTR modulator therapy with Elexacaftor/Tezacaftor/Ivacaftor (ETI) was initiated and has been well-tolerated, resulting in a remarkable improvement in the weight curve (Figure 4).