case_images: PMC9247646_01
case_images: (A) CXR and (B) CT revealed a giant mass of 16.9 cm x 15.2 cm x 22.4 cm (AP x W x H) expanding the right thorax with significant compression on the mediastinum. (C) Post-operative CT indicates partial re-expansion of the right lung.
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case_images: PMC9247646_01_fped-10-885068-g001.jpg
case_images: F1
case_images: Subsequent X-ray and computed tomography (CT) confirmed a solid mass lesion occupying and expanding the entire right hemithorax, measuring 17 x 15 x 22 cm (Figure 1). The patient had been monitored clinically and radiographically (with CT) every 4 monthly, remaining in remission thus far 12 months from diagnosis (Figure 1C).
case_images: (A) H&E staining on the tumor biopsy specimen - abnormal spindle cells with scattered small-sized blood vessels; focal myxoid area was noted without mitosis, necrosis or rosette formation. (B) Diffuse and strong MUC4 positive shown in the spindle cells. (C) FISH study with nuclei showing isolated green FISH signal (5'end of FUS gene) compatible with presence of FUS translocation (courtesy of Prof Ka-Fai To, Chinese University of Hong Kong). (D) RNA sequencing confirming FUS-CREB3L2 chimeric transcript.
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case_images: PMC9247646_01_fped-10-885068-g002.jpg
case_images: F2
case_images: Histological analysis on fixed tissue obtained from core biopsy indicated abnormal spindle cells with focal myxoid areas, and absence of mitosis, necrosis or rosette formation (Figure 2).
case_images: Angiographic findings (A) before and (B) after embolization of tumor feeding vessels (coil indicated by red-arrow). (C-E) Intra-operative photo illustrating successful en bloc excision of tumor through a hemi-clamshell incision.
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case_images: PMC9247646_01_fped-10-885068-g003.jpg
case_images: F3
case_images: ECMO standby and embolization of feeding vessels from the right bronchial artery (Figure 3). Endotracheal intubation was achieved without complication; medial sternotomy with right anterolateral thoracotomy (hemi-clamshell approach indicated without the need for supraclavicular incision in view of the lack of blood supply from the head and neck vessels) was then performed, followed by a successful en bloc resection of the encapsulated tumor attached to the pericardium.