Colonic Ewing Sarcoma/PNET associated with liver metastases: A systematic review and case report |
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| Authors: | 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 |
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| Affiliation: | 1. Department of Diagnostics and Public Health – Section of Pathology, University and Hospital Trust of Verona, Verona 37134, Italy;2. Fondazione IRCCS Casa Sollievo della Sofferenza, UO di Oncologia Medica, 71013 San Giovanni Rotondo, Foggia, Italy;3. Pathology Unit, ULSS9 "Scaligera", 37122 Verona, Italy;4. Oncology Unit, ULSS9 “Scaligera”, 37122 Verona, Italy;5. Department of Sciences and Technologies, University of Sannio, 82100 Benevento, Italy;6. Surgery Unit, ULSS9 "Scaligera", 37122 Verona, Italy;7. Medical Oncology Unit, San Filippo Neri Hospital – ASL Roma 1, 00135, Roma, Italy;8. Department of Medicine (DIMED), University of Padua, 35121, Padova, Italy;9. Department of Biology, University of Naples, Federico II, 80134 Napoli, Italy;10. Mater Salutis” Hospital, ULSS9, 37045 Legnago, Verona, Italy |
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| Abstract: | 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). |
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| Keywords: | Ewing Sarcoma Colon Rectum PNET Liver metastases Extra osseous sarcoma |
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