Colonic Ewing Sarcoma/PNET associated with liver metastases: A systematic review and case report

Pietro Parcesepe, Guido Giordano, Caterina Zanella, Jacopo Giuliani, Filippo Greco, Andrea Bonetti, Massimo Pancione, Erminia Manfrin, Enrico Molinari, Tiziana Pia Latiano, Mario Rosario D'Andrea, Matteo Fassan, Nunzio Olivieri, Andrea Remo

Research output: Contribution to journalArticlepeer-review


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).

Original languageEnglish
JournalPathology Research and Practice
Publication statusAccepted/In press - Jan 1 2018


  • Colon
  • Ewing Sarcoma
  • Extra osseous sarcoma
  • Liver metastases
  • PNET
  • Rectum

ASJC Scopus subject areas

  • Pathology and Forensic Medicine
  • Cell Biology


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