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ABSTRACT
CIC-DUX4 sarcoma (CDS) is a recently identified subtype of small round cell sarcoma. Morphologically, CDS partially resembles Ewing sarcoma (ES) and has been classified as “ES-like sarcoma”; however, detailed clinicopathologic and molecular genetic analyses have indicated that CDS is a new independent disease. Many studies have provided light microscopic, immunohistochemical, and genetic information about CDS. However, ultrastructural findings associated with this sarcoma are lacking. The aim of this study was to investigate the ultrastructure of CDS tumors and to compare their features with those of ES. We examined two cytogenetically confirmed CDS cases. We found that, compared to typical ES, CDS presented heterogeneity: in cell density, from tightly packed to loosely unconnected areas; in cell shape, from polygonal to pleomorphic with small processes; and in nuclear shape including round, oval, polygonal, elongated, invaginated, or wrinkled formations. However, abundant glycogen in the cytoplasm and rare cell adhesion apparatus between cells are major similarities between CDS and typical ES. Neuroendocrine granules, which are seen in rare ES cases, could not be identified in these two CDS cases. Although cytogenetic differences can validate a definite diagnosis, ultrastructural features could also provide important information about the differences between CDS and ES.
Acknowledgments
The Authors Thank Ms. Tomoko Nishizawa, Ms. Shizu Fujii, and Mr. Kiyokazu Kametani for and Technical Assistance.
Disclosure of interest
The authors report no conflict of interest.
Informed consent
The parents of the patient in case 1 provided written informed consent prior to study enrollment. The patient of case 2 provided written informed consent prior to study enrollment.
Correction Statement
This article has been republished with minor changes. These changes do not impact the academic content of the article.