Sarcoma Unit, Royal Marsden Hospital, London SW3 6JJ, UK; Division of Molecular Pathology, The Institute of Cancer Research, London SW3 6JB, UK.
Division of Molecular Pathology, The Institute of Cancer Research, London SW3 6JB, UK; Department of Pathology, University Hospitals Birmingham NHS Foundation Trust, Birmingham B15 2GW, UK.
Surg Pathol Clin. 2023 Dec;16(4):765-778. doi: 10.1016/j.path.2023.05.012. Epub 2023 Jul 11.
Small round cell neoplasms are diagnostically challenging owing to their clinical and pathologic overlap, necessitating use of large immunopanels and molecular analysis. Ewing sarcomas (ES) are the most common, but EWSR1 is translocated in several diverse neoplasms, some with round cell morphology. Molecular advances enable classification of many tumors previously termed 'atypical ES'. The current WHO Classification includes two new undifferentiated round cell sarcomas (with CIC or BCOR alterations), and a group of sarcomas in which EWSR1 partners with non-Ewing family transcription factor genes. This article reviews the spectrum of small round cell sarcomas within the gastrointestinal tract and abdomen.
小圆细胞肿瘤由于其临床表现和病理表现重叠，诊断具有挑战性，需要使用大型免疫组化面板和分子分析。尤文肉瘤（ES）最为常见，但 EWSR1 易位发生在多种不同的肿瘤中，其中一些具有圆形细胞形态。分子学的进步使许多以前被称为“非典型尤文肉瘤”的肿瘤得到了分类。目前的 WHO 分类包括两种新的未分化小圆细胞肉瘤（具有 CIC 或 BCOR 改变），以及一组 EWSR1 与非尤文家族转录因子基因发生伙伴关系的肉瘤。本文综述了胃肠道和腹部小圆细胞肉瘤的范围。
