TY - JOUR
T1 - Colonic Ewing Sarcoma/PNET associated with liver metastases
T2 - A systematic review and case report
AU - Parcesepe, Pietro
AU - Giordano, Guido
AU - Zanella, Caterina
AU - Giuliani, Jacopo
AU - Greco, Filippo
AU - Bonetti, Andrea
AU - Pancione, Massimo
AU - Manfrin, Erminia
AU - Molinari, Enrico
AU - Latiano, Tiziana Pia
AU - D'Andrea, Mario Rosario
AU - Fassan, Matteo
AU - Olivieri, Nunzio
AU - Remo, Andrea
PY - 2018/1/1
Y1 - 2018/1/1
N2 - 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).
AB - 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).
KW - Colon
KW - Ewing Sarcoma
KW - Extra osseous sarcoma
KW - Liver metastases
KW - PNET
KW - Rectum
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U2 - 10.1016/j.prp.2018.11.021
DO - 10.1016/j.prp.2018.11.021
M3 - Article
AN - SCOPUS:85058226725
JO - Pathology Research and Practice
JF - Pathology Research and Practice
SN - 0344-0338
ER -