Parcesepe Pietro, Giordano Guido, Zanella Caterina, Giuliani Jacopo, Greco Filippo, Bonetti Andrea, Pancione Massimo, Manfrin Erminia, Molinari Enrico, Latiano Tiziana Pia, D'Andrea Mario Rosario, Fassan Matteo, Olivieri Nunzio, Remo Andrea
Department of Diagnostics and Public Health - Section of Pathology, University and Hospital Trust of Verona, Verona 37134, Italy.
Fondazione IRCCS Casa Sollievo della Sofferenza, UO di Oncologia Medica, 71013 San Giovanni Rotondo, Foggia, Italy.
Pathol Res Pract. 2019 Feb;215(2):387-391. doi: 10.1016/j.prp.2018.11.021. Epub 2018 Nov 29.
Ewing Sarcoma is a highly lethal undifferentiated tumor of bone. ES is a small round cell tumor with etiological and characteristic chromosomal translocations between TET/FET (TLS/FUS, EWSR1, and TAF15) and ETS (E26 transformation-specific) family genes. Generally, therapeutic approach for metastatic Ewing Sarcoma includes both local (surgery and radiotherapy) and systemic (chemotherapy) disease control with an overall cure rate of 20%. For extra-osseous tumors, the most common primary sites of disease are trunk, extremities, head and neck, retroperitoneum. Among other sites, Ewing Sarcoma/PNET may also rarely arise in colon and rectum. Even if colonic Ewing Sarcoma/PNET have been previously reported in 5 cases, none of those reports came from right side of the colon. In this article, we report the first case of right-sided Ewing Sarcoma with synchronous liver metastases completely responding to first line chemotherapy. Furthermore, we provide a systematic qualitative review of the current literature on adult colorectal Ewing Sarcoma using the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA).
尤因肉瘤是一种高度致命的骨未分化肿瘤。尤因肉瘤是一种小圆细胞肿瘤，具有病因学特征以及TET/FET（TLS/FUS、EWSR1和TAF15）与ETS（E26转化特异性）家族基因之间的染色体易位。一般来说，转移性尤因肉瘤的治疗方法包括局部（手术和放疗）和全身（化疗）疾病控制，总体治愈率为20%。对于骨外肿瘤，最常见的疾病原发部位是躯干、四肢、头颈部、腹膜后。在其他部位中，尤因肉瘤/原始神经外胚层肿瘤也可能很少发生在结肠和直肠。尽管之前已有5例结肠尤因肉瘤/原始神经外胚层肿瘤的报道，但这些报道均非来自结肠右侧。在本文中，我们报告了首例右侧尤因肉瘤伴同步肝转移且对一线化疗完全缓解的病例。此外，我们使用系统评价和Meta分析的首选报告项目（PRISMA）对当前关于成人结直肠尤因肉瘤的文献进行了系统的定性综述。
