A left atrial myxomalike rhabdomyosarcoma

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A left atrial myxomalike rhabdomyosarcoma Domenico Corradi, MD, Giovanni Andrea Contini, MD, Tiziano Gherli, MD, Francesco Nicolini, MD, PhD  The Journal of Thoracic and Cardiovascular Surgery  Volume 144, Issue 1, Pages e7-e10 (July 2012) DOI: 10.1016/j.jtcvs.2012.03.073 Copyright © 2012 The American Association for Thoracic Surgery Terms and Conditions

Figure 1 A and B, Two-dimensional echocardiography in parasternal long-axis view during systole (A) and diastole (B), showing the sarcoma (asterisks) characterized by nonhomogeneous echocardiographic texture, occupying almost the entire left atrium, and projecting toward the mitral valve plan (arrows) during diastole (B). C, Gross appearance of the bilobular mass. D, Gross transverse sections of the sarcoma. The specimen on the right corresponds to the nonspecific sarcomatous component and shows grayish, yellowish gelatinous nodules and focal necrotic areas (arrowhead). The 2 grayish sections on the left coincide with the rhabdomyosarcoma component and display larger necrotic zones (arrows). LA, Left atrium; LV, left ventricle. Bars represent 4 cm in C and 5 cm in D. The Journal of Thoracic and Cardiovascular Surgery 2012 144, e7-e10DOI: (10.1016/j.jtcvs.2012.03.073) Copyright © 2012 The American Association for Thoracic Surgery Terms and Conditions

Figure 2 A, Histopathologic view of the nonspecific sarcoma, composed of spindle and stellate cells that often crowd around blood vessels (arrow). B, Low-magnification appearance of the tumor proximal portion (N), which stems from a thickened fibrous subepicardium (SE) with no infiltration of the underlying myocardium (arrow). C, Microscopic view of the high-grade rhabdomyosarcoma component, with several elongated (arrow) and rounded (arrowhead) rhabdomyoblasts. D, Immunohistochemical analysis with an antimyogenin primary antibody (a skeletal muscle–specific transcription factor) showing the boundary line between nonspecific sarcoma (S) and myogenin-positive rhabdomyosarcoma (RMS). E, Ultrastructure of a stellate cell belonging to the nonspecific sarcomatous component. The nucleus (N) is highly irregular and atypical; the cytoplasm (Cy) contains a number of mitochondria and vesicles. The stroma is myxoid with numerous bundles of collagen type I (C). There are no contractile structures. F, Electron microscopy of an exceedingly atypical rhabdomyoblast in which the sarcoplasm (Sa) is filled with disorganized and nonfunctioning myofibrillar fragments (arrow and insert). Stains: A, B, and C, hematoxylin-eosin; D, 3,3-diaminobenzidine-revealed immunohistochemistry with mild Harris hematoxylin counterstaining. Original magnifications: A and C, ×20; B and D, ×4; E, ×3500; F, ×7,100 (insert ×56,000). Bars represent 150 μm in A and C, 500 μm in B and D, 5000 nm in E; and 2000 nm in F (bar in insert represents 200 nm). The Journal of Thoracic and Cardiovascular Surgery 2012 144, e7-e10DOI: (10.1016/j.jtcvs.2012.03.073) Copyright © 2012 The American Association for Thoracic Surgery Terms and Conditions