Identification Of Actionable Genetic Targets In Primary Cardiac Sarcomas

Figures

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  Figure 1. (A) CT scan of the chest and (B) CT scan of the abdomen reveal a hypodense and heterogeneous mass in the atrium.

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  Figure 2. (A) Hematoxylin eosin staining at 10× magnification and (B) at 20× magnification showing atypical mesenchymal cell proliferation of fascicular pattern with numerous mitotic figures and necrosis areas. (C) Positive immunostaining for vimentin, (D) smooth muscle actin (SMA), (E) cytokeratins AE1/AE3 and F 90% Ki67 proliferation index.

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  Figure 3. Molecular karyotype in circos plot of the undifferentiated pleomorphic rhabdomyosarcoma (case report 1). Allele peaks (inner plots) and weighted Log2ratio (middle plots) information were obtained from Affymetrix software ChAS and further used to generate circos plot.

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  Figure 4. (A) Cardiac Magnetic Resonance Imaging of the pericardial tumor with a maximum diameter of 69.48 mm. (B)Large mass arising from the pericardium adjacent to the right atrium, which extends superiorly towards the left pulmonary artery. (C). Lesion relapsed arising from the pericardium in the right atrium which is invading the right ventricle. It is associated with extending thrombosis towards the suprahepatic veins and inferior cava vein. Bilateral pleural effusion.

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  Figure 5. (A) Hypercellular areas with focal necrosis (H-E 10×). (B) The cells showed large, irregular nuclei and prominent nucleoli, with a wide, eosinophilic cytoplasm. (C) Cells with rhabdoid features and cytoplasmatic cross striations (H-E 40×). (D) Diffuse proliferation with a storiform growth pattern, embedded in a collagenous/myxoid stroma (H-E 10×).

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  Figure 6. (A) CT scan of the abdomen and (B) CT scan of the chest reveal a large tumor in the left atrium extending to the lower pulmonary vein.

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  Figure 7. (A) Hematoxylin-eosin staining at 10× magnification and (B) at 20× magnification showing mixed inflammatory infiltrate, occasional plasma cells and mesenchymal spindle cells without atypia or mitotic figures. (C) Cells CD68+. (D) T-lymphocytes CD3+. (E) B-lymphocytes CD20+. (F) Smooth muscle actin.

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  Figure 8. Molecular karyotype in circos plot of the inflammatory myofibroblastic tumor (case report 3). Allele peaks (inner plots) and weighted Log2ratio (middle plots) information were obtained from Affymetrix software ChAS and further used to generate circos plot. Paraffin-embedded tissue with a 50–60% tumor cell content was used for molecular karyotype analysis, revealing a 25% mosaic numerical chromosomal gain in whole chromosome 8 and numerous SCAs including 25–50% mosaic losses in 1q21.1qter, 2p25.3pter, 4q12.2qter, 9p13.2pter, 16q12.2q24.3; two sub-telomeric amplifications (4+1 allele copies) in 5p15.33 containing TERT promoter in the flanking breakpoint and in 5p15.2p15.31, gains (3+1 allele copies) in 2q11.1qter, 11q14.1, 12q13.2q13.3 and in 12q13.3q14.1 containing CDK4, and amplification (9 copies) in 12q14.3q21.1 region containing MDM2.

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  Figure 9. (A) Diagnostic computed tomography scan. Pericardial effusion with heterogenic areas suggesting recent bleeding. Pericardial tumor mass depending on the right atrium wall. (B) Computed tomography scan showing progression of the pericardial mass that measures 154×93.5 mm and presents mass effect on the right cavities. Composed of vascular structures and necroticoquistic areas.

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  Figure 10. (A) Biphasic synovial sarcoma showing epithelial structures surrounded by spindle cells. (B) The epithelioid cells showed immunohistochemical expression of cytokeratin 7, (C) whereas the spindle cell component displayed a predominant vimentin expression. (D) FISH break-apart probe result was consistent with a translocation, yielded one yellow fusion, one red and one green pattern.

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  Figure 11. Surgical exposure of the heart confirmed an extra-cardiac mass on the left atrium protruding into the ventricle.

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  Figure 12. Hematoxylin and eosin staining in case report 5 revealed a myxoid variant of a high-grade undifferentiated pleomorphic sarcoma with moderate inflammatory infiltration.