PMC8348529_01
CTA of pulmonary arteries prior to endarterectomy.. (A) Axial and (B) Coronal images show large occlusive filling defect in right main pulmonary artery (yellow arrows). . (For interpretation of the references to colour in this figure legend, the reader is referred to the Web version of this article.)
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Repeat CTA revealed near-complete occlusion of the right main pulmonary artery (Fig. 1A and B).
Histopathology of the intimal sarcoma of the pulmonary artery. (A) Low power view of the neoplastic proliferation arising from the vessel wall (H&E, 20x) (B) High power view shows the pleomorphic cells in a myxoid matrix (H&E, 200x) (C) Immunohistochemical stain, desmin shows reactivity within the cells (200x) (D) Fluorescent in-situ hybridization of probe for Ewing sarcoma breakpoint region 1 (EWSR1) locus (22q12) showed no rearrangement (the signals for the nucleus near the center of the field were on a different focal panel) (E) Genome-wide view of the copy number findings that were present in this tumor, with the data presented as the weighted log2 ratio (top); and the beta allele frequency pattern (bottom). The tumor had a complex karyotype with multiple copy number gains and losses. The red arrows highlight regions with amplification (noted on chromosomes 2, 4, and 12). The patterns noted on chromosome 12 were consistent with a chromothripsis-like pattern (multiple peaks). (F) The copy number patterns for the region that encompasses the MDM2 gene are shown (portions of band 12q15 and band 12q21.1). The dashed line (highlighted by a red arrow) denotes the location of the MDM2 gene. Note that the locus is coincident with the presence of a "peak" of high copy number gain (approximately 10 copies). . (For interpretation of the references to colour in this figure legend, the reader is referred to the Web version of this article.)
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Histopathologic examination of the specimen documented an obstructing myxoid sarcomatoid neoplasm with atypia and variable cellularity, engendering a broad differential diagnosis (Fig. 2A and B).
CTA of pulmonary arteries performed at emergency department visit three months after endarterectomy.. (A) Axial view of a 3.2 x 2.0 cm filling defect (yellow straight arrow) in the right main pulmonary artery extending into the truncus anterior and apical segmental artery. (B) Coronal view of the same filling defect (yellow straight arrow) also showing involvement of the right upper lobe (blue curved arrow). . (For interpretation of the references to colour in this figure legend, the reader is referred to the Web version of this article.)
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One month later, the patient again presented to the emergency department with worsening dyspnea, and CTA revealed interval growth of the mass to 3.2 x 2.0 cm (Fig. 3A and B).
CTA of pulmonary arteries performed one month after completion of radiotherapy.. (A) Axial and (B) Coronal sequences through right pulmonary artery shows residual tumor only located along superior margin of artery (yellow and blue arrows). . (For interpretation of the references to colour in this figure legend, the reader is referred to the Web version of this article.)
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The patient received 50 Gy in 25 fractions to the mass using a volumetric modulated arc therapy (VMAT) technique, and subsequent CTA four weeks post-radiation revealed a decrease in mass size to 2.2 x 1.2 cm (Fig. 4A and B).
CT with contrast of pulmonary arteries four months after intimal sarcoma resection, right pulmonary artery reconstruction, and right upper lobectomy.. (A) Axial view of right main pulmonary artery (yellow straight arrow) reconstructed with bovine pericardium after resection of mass. Chronic loculated hemothorax also present in right upper pleural space. (B) Coronal view of same reconstructed right main pulmonary artery (yellow straight arrow) with visible surgical clips. . (For interpretation of the references to colour in this figure legend, the reader is referred to the Web version of this article.)
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Follow-up chest CT with contrast at this time did not reveal any evidence of recurrent disease or enlarged lymph nodes (Fig. 5A and B).