CIC-DUX4 rearranged uterine cervix round-cell sarcoma exhibiting near-complete pathologic response following radiation and neoadjuvant chemotherapy: A case report

Abstract

Sarcomas harboring CIC-DUX4 fusions have recently been described as highly aggressive variants of small blue round cell tumors, affecting predominantly children and young adults. This uncommon, molecularly-defined entity can arise from various anatomical locations and often involves deep soft-tissue structures and skin. Although clinically analogous and commonly confounded with Ewing sarcoma (ES), several studies have recently shown that this unique condition maintains morphologic and pathological features distinct from ES. In this report, we present and discuss a case of CIC-rearranged sarcoma of the uterine cervix exhibiting a near-complete pathologic response following neoadjuvant chemotherapy. Due to rarity of this entity, this report is a relevant contribution to the scientific community and clinicians facing this challenging and often ominous disease.